Respiratory Finals PDF

Question 1 of 106 A 27-year-old woman is reviewed in the asthma clinic. She currently uses salbutamol inhaler 100mcg prn combined with beclometasone dipropionate inhaler 400mcg bd. Despite this she is having frequent exacerbations of her asthma and recently required a course of prednisolone. What is...

Question 1 of 106 A 27-year-old woman is reviewed in the asthma clinic. She currently uses salbutamol inhaler 100mcg prn combined with beclometasone dipropionate inhaler 400mcg bd. Despite this she is having frequent exacerbations of her asthma and recently required a course of prednisolone. What is the most appropriate next step in management? A. Add a leukotriene receptor antagonist B. Add tiopropium C. Add salmeterol D. Start to take the salbutamol regularly, 2 puffs qds E. Switch beclometasone to fluticasone ----------------------------------------------------- C. Add salmeterol Asthma: stepwise management in adults............................................ The management of stable asthma is now well established with a step-wise approach: Step 1 Inhaled short-acting B2 agonist as required Step 2 Add inhaled steroid at 200-800 mcg/day* 400 mcg is an appropriate starting dose for many patients. Start at dose of inhaled steroid appropriate to severity of disease Step 3 1. Add inhaled long-acting B2 agonist (LABA) 2. Assess control of asthma: good response to LABA - continue LABA benefit from LABA but control still inadequate: continue LABA and increase inhaled steroid dose to 800 mcg/day* (if not already on this dose) no response to LABA: stop LABA and increase inhaled steroid to 800 mcg/ day.* If control still inadequate, institute trial of other therapies, leukotriene receptor antagonist or SR theophylline Step 4 Consider trials of: increasing inhaled steroid up to 2000 mcg/day* addition of a fourth drug e.g. Leukotriene receptor antagonist, SR theophylline, B2 agonist tablet 1 Step 5 Use daily steroid tablet in lowest dose providing adequate control. Consider other treatments to minimise the use of steroid tablets Maintain high dose inhaled steroid at 2000 mcg/day* Refer patient for specialist care *beclometasone dipropionate or equivalent Additional notes Leukotriene receptor antagonists e.g. Montelukast, zafirlukast have both anti-inflammatory and bronchodilatory properties should be used when patients are poorly controlled on high-dose inhaled corticosteroids and a long-acting b2-agonist particularly useful in aspirin-induced asthma associated with the development of Churg-Strauss syndrome Fluticasone is more lipophilic and has a longer duration of action than beclometasone Hydrofluoroalkane is now replacing chlorofluorocarbon as the propellant of choice. Only half the usually dose is needed with hydrofluoroalkane due to the smaller size of the particles Long acting B2-agonists acts as bronchodilators but also inhibit mediator release from mast cells. Recent meta-analysis showed adding salmeterol improved symptoms compared to doubling the inhaled steroid dose 2 Question 2 of 106 Which one of the following is a contraindication to surgical resection in lung cancer? A. Haemoptysis B. FEV 1.9 litres C. Histology shows squamous cell cancer D. Vocal cord paralysis E. Calcium = 2.84 mmol/L ----------------------------------------------------- D. Vocal cord paralysis Contraindications to lung cancer surgery include SVC obstruction, FEV < 1.5, MALIGNANT pleural effusion, and vocal cord paralysis Paralysis of a vocal cord implies extracapsular spread to mediastinal nodes and is an indication of inoperability. Lung cancer: non-small cell management............................................ Management only 20% suitable for surgery mediastinoscopy performed prior to surgery as CT does not always show mediastinal lymph node involvement curative or palliative radiotherapy poor response to chemotherapy Surgery contraindications assess general health stage IIIb or IV (i.e. metastases present) FEV1 < 1.5 litres is considered a general cut-off point* malignant pleural effusion tumour near hilum vocal cord paralysis SVC obstruction * However if FEV1 < 1.5 for lobectomy or < 2.0 for pneumonectomy then some authorities advocate further lung function tests as operations may still go ahead based on the results 3 Question 3 of 106 A 24-year-old female presents with episodic wheezing and shortness of breath for the past 4 months. She has smoked for the past 8 years and has a history of eczema. Examination of her chest is unremarkable. Spirometry is arranged and is reported as normal. What is the most appropriate management of her symptoms? A. Peak flow diary B. Trial of lansoprazole C. Baseline FEV1 repeated following inhaled corticosteroids D. Arrange a chest x-ray E. Trial of salbutamol inhaler ----------------------------------------------------- E. Trial of salbutamol inhaler Asthma diagnosis - if high probability of asthma - start treatment The new British Thoracic Society guidelines take a more practical approach to diagnosing asthma. If a patient has typical symptoms of asthma a trial of treatment is recommended. Normal spirometry when the patient is well does not exclude a diagnosis of asthma. The smoking history is unlikely to be relevant at her age. What is not necessarily clear is whether all patients should have spirometry prior to starting treatment - how do you interpret the guidelines? Asthma: diagnosis in adults............................................ The 2008 British Thoracic Society guidelines marked a subtle change in the approach to diagnosing asthma. It suggests dividing patients into a high, intermediate and low probability of having asthma based on the presence or absence of typical symptoms. A list can be found in the external link but include typical symptoms such as wheeze, nocturnal cough etc Example of features used to assess asthma (not complete, please see link) Increase possibility of asthma Wheeze, breathlessness, chest tightness and cough, worse at night/early morning History of atopic disorder 4  Wheeze heard on auscultation Unexplained peripheral blood eosinophilia Decrease possibility of asthma Prominent dizziness, lightheadedness, peripheral tingling Chronic productive cough in the absence of wheeze or breathlessness Repeatedly normal physical examination Significant smoking history (i.e. > 20 pack-years) Normal PEF or spirometry when Symptomatic Management is based on this assessment: high probability: trial of treatment intermediate probability: see below low probability: investigate/treat other condition For patients with an intermediate probability of asthma further investigations are suggested. The guidelines state that spirometry is the preferred initial test: FEV1/FVC < 0.7: trial of treatment FEV1/FVC > 0.7: further investigation/consider referral Recent studies have shown the limited value of other 'objective' tests. It is now recognised that in patients with normal or near-normal pre-treatment lung function there is little room for measurable improvement in FEV1 or peak flow. A > 400 ml improvement in FEV1 is considered significant before and after 400 mcg inhaled salbutamol in patients with diagnostic uncertainty and airflow obstruction present at the time of assessment if there is an incomplete response to inhaled salbutamol, after either inhaled corticosteroids (200 mcg twice daily beclometasone equivalent for 6-8 weeks) or oral prednisolone (30 mg once daily for 14 days) It is now advised to interpret peak flow variability with caution due to the poor sensitivity of the test diurnal variation % = [(Highest – Lowest PEFR) / Highest PEFR] x 100 assessment should be made over 2 weeks greater than 20% diurnal variation is considered significant 5 Question 4 of 106 Which of the following factors is least useful in assessing patients with a poor prognosis in community-acquired pneumonia? A. Mini-mental score of 6/10 B. Urea of 11.4 mmol/l C. C-reactive protein of 154 D. Respiratory rate of 30 E. Aged 75 years old ----------------------------------------------------- C. C-reactive protein of 154 The C-reactive protein is the least useful of the above in predicting mortality in patients with community-acquired pneumonia. The rest of the answers are part of the CURB-65 criteria Pneumonia: prognostic factors............................................ CURB-65 criteria of severe pneumonia Confusion (abbreviated mental test score < 8/10) Urea > 7 mmol/L Respiratory rate = 30 / min BP: systolic < 90 or diastolic < 60 mmHg age > 65 years Patients with 3 or more (out of 5) of the above criteria are regarded as having a severe pneumonia Other factors associated with a poor prognosis include: presence of coexisting disease hypoxaemia (pO2 < 8 kPa) independent of FiO2 6 Question 5 of 106 Which one of the following is responsible for farmer's lung? A. Aspergillus clavatus B. Micropolyspora faeni C. ThermoActinomyces candidus D. Mycobacterium avium E. Avian proteins ----------------------------------------------------- B. Micropolyspora faeni Micropolyspora faeni causes farmer's lung, a type of EAA Extrinsic allergic alveolitis............................................ Extrinsic allergic alveolitis (EAA) is a condition caused by hypersensitivity induced lung damage due to a variety of inhaled organic particles. It is thought to be largely caused by immune-complex mediated tissue damage (type III hypersensitivity) although delayed hypersensitivity (type IV) is also thought to play a role in EAA, especially in the chronic phase Examples bird fanciers' lung (avian proteins) farmers lung (spores of Micropolyspora faeni) malt workers' lung (Aspergillus clavatus) mushroom workers' lung (thermophilic actinomycetes*) Presentation acute: occur 4-8 hrs after exposure, SOB, dry cough, fever chronic Investigation CXR: upper lobe fibrosis BAL: lymphocytosis blood: NO eosinophilia *here the terminology is slightly confusing as thermophilic actinomycetes is an umbrella term covering strains such as Micropolyspora faeni 7 Question 6 of 106 A 24-year-old male with no past medical history presents to the Emergency Department with pleuritic chest pain. There is no history of a productive cough and he is not short of breath. Chest x-ray shows a right-sided pneumothorax with a 1 cm rim of air and no mediastinal shift. What is the most appropriate management? A. Immediate 14G cannula into 2nd intercostal space, mid-clavicular line B. Discharge with outpatient chest x-ray C. Aspiration D. Intercostal drain insertion E. Admit for 48 hours observation ----------------------------------------------------- B. Discharge with outpatient chest x-ray It was of course be prudent to give advice about what he should do if his symptoms worsen and also suggest routine follow-up with his GP Pneumothorax............................................ The British Thoracic Society (BTS) published guidelines for the management of spontaneous pneumothorax in 2003. A pneumothorax is termed primary if there is no underlying lung disease and secondary if there is Primary pneumothorax Recommendations include: if the rim of air is < 2cm and the patient is not short of breath then discharge should be considered otherwise aspiration should be attempted if this fails then repeat aspiration should be considered if this fails then a chest drain should be inserted Secondary pneumothorax Recommendations include: if the patient is > 50 years old and the rim of air is > 2cm and the patient is short of breath then a chest drain should be inserted. otherwise aspiration should be attempted. If aspiration fails a chest drain should be inserted. All patients should be admitted for at least 24 hours Iatrogenic pneumothorax Recommendations include: less likelihood of recurrence than spontaneous pneumothorax majority will resolve with observation, if treatment is required then aspiration should be used ventilated patients need chest drains, as may some patients with COPD 8 Question 7 of 106 A 65-year-old female with a history of chronic obstructive pulmonary disease (COPD) is reviewed in the Emergency Department. She has presented with a sudden worsening of her dyspnoea associated with haemoptysis. What is the most suitable initial imaging investigation to exclude a pulmonary embolism? A. Ventilation-perfusion scan B. Echocardiogram C. Pulmonary angiography D. Computed tomographic pulmonary angiography E. MRI thorax ----------------------------------------------------- D. Computed tomographic pulmonary angiography It is still common in UK hospitals, despite guidelines, for a ventilation-perfusion scan to be done first-line Pulmonary embolism: investigation............................................ The British Thoracic Society (BTS) published guidelines in 2003 on the management of patients with suspected pulmonary embolism (PE) Key points from the guidelines include: computed tomographic pulmonary angiography (CTPA) is now the recommended initial lung- imaging modality for non-massive PE. Advantages compared to V/Q scans include speed, easier to perform out-of-hours, a reduced need for further imaging and the possibility of providing an alternative diagnosis if PE is excluded if the CTPA is negative then patients do not need further investigations or treatment for PE ventilation-perfusion scanning may be used initially if appropriate facilities exist, the chest x-ray is normal, and there is no significant symptomatic concurrent cardiopulmonary disease Some other points Clinicalprobability scores based on risk factors and history and now widely used to help decide on further investigation/management D-dimers sensitivity = 95-98%, but poor specificity V/Q scan sensitivity = 98%; specificity = 40% - high negative predictive value, i.e. if normal virtually excludes PE other causes of mismatch in V/Q include old pulmonary embolisms, AV malformations, vasculitis, previous radiotherapy COPD gives matched defects CTPA peripheral emboli affecting subsegmental arteries may be missed Pulmonary angiography the gold standard significant complication rate compared to other investigations 9 Question 8 of 106 You are reviewing the results from investigations requested at the previous respiratory clinic. A 40-year-old man is being investigated for increasing shortness of breath. The notes show he has smoked for the past 25 years. Pulmonary function tests reveal the following: FEV1 1.4 L FVC 1.7 L FEV1/FVC 82% Which one of the following is the most likely explanation? A. Asthma B. Bronchiectasis C. Kyphoscoliosis D. Chronic obstructive pulmonary disease E. Laryngeal malignancy ----------------------------------------------------- C. Kyphoscoliosis These results show a restrictive picture, which may result from a number of conditions including kyphoscoliosis. The other answers cause an obstructive picture. Pulmonary function tests............................................ Pulmonary function tests can be used to determine whether a respiratory disease is obstructive or restrictive. The table below summarises the main findings and gives some example conditions: Obstructive lung disease Restrictive lung disease FEV1 - significantly reduced FEV1 - reduced FVC - reduced or normal FVC - significantly reduced FEV1% (FEV1/FVC) - reduced FEV1% (FEV1/FVC) - normal or increased Asthma Pulmonary fibrosis COPD Asbestosis Bronchiectasis Sarcoidosis Bronchiolitis obliterans Acute respiratory distress syndrome Infant respiratory distress syndrome Kyphoscoliosis Neuromuscular disorders 10 Question 9 of 106 A 45-year-old female develops pleuritic chest pain following a hysterectomy 10 days ago. Low-molecular weight heparin is given initially and CTPA confirms a pulmonary embolism. There is no previous history of venous thromboembolism. How long should the patient be warfarinised for? A. Not suitable for anticoagulation B. 6 weeks C. 3 months D. 6 months E. 12 months ----------------------------------------------------- C. 3 months As this patient has a temporary risk factor for a thromboembolic event the recommended period of anticoagulation is 3 months. Pulmonary embolism: management............................................ Unfortunately there is a lack of clear guidelines on the optimal length of anticoagulation following a pulmonary embolism. The 2003 British Thoracic Society guidelines which advocate a shorter duration of treatment are not widely followed. The following is based on the 2005 British Committee for Standards in Haematology (BCSH) guidelines and Clinical Knowledge Summaries. Initial anticoagulation with heparin low molecular weight heparin (LMWH), rather than unfractionated heparin (UFH), should be used routinely in patients with suspected pulmonary embolism. This reflects the equal efficacy and safety of LMWHs as well as their ease of use exceptions include patients with a massive PE or in situations where rapid reversal of anticoagulation may be necessary Ongoing anticoagulation with warfarin target INR 2.0 - 3.0, length of treatment: calf DVT: at least 6 weeks proximal DVT or PE where there is transient risk factors: at least 3 months idiopathic venous thromboembolism or permanent risk factors: at least 6 months Thrombolysis thrombolysis is now recommended as the first-line treatment for massive PE where there is circulatory failure (e.g. Hypotension). Other invasive approaches should be considered where appropriate facilities exist 11 Question 10 of 106 Which one of the following causes of lung fibrosis predominately affect the upper zones? A. Bleomycin B. Rheumatoid arthritis C. Cryptogenic fibrosis alveolitis D. Methotrexate E. Extrinsic allergic alveolitis ----------------------------------------------------- E. Extrinsic allergic alveolitis Lung fibrosis............................................ It is important in the exam to be able to differentiate between conditions causing predominately upper or lower zone fibrosis. It should be noted that the more common causes (cryptogenic fibrosing alveolitis, drugs) tend to affect the lower zones Fibrosis predominately affecting the upper zones extrinsic allergic alveolitis coal worker's pneumoconiosis/progressive massive fibrosis silicosis sarcoidosis ankylosing spondylitis (rare) histiocytosis tuberculosis Fibrosis predominately affecting the lower zones cryptogenic fibrosing alveolitis most connective tissue disorders (except ankylosing spondylitis) drug-induced: amiodarone, bleomycin, methotrexate asbestosis 12 Question 11 of 106 A 74-year-old woman with thyroid cancer is admitted due to shortness of breath. What is the best investigation to assess for possible compression of the upper airways? A. Arterial blood gases B. Forced vital capacity C. Transfer factor D. Peak expiratory flow rate E. Flow volume loop ----------------------------------------------------- E. Flow volume loop Flow volume loop is the investigation of choice for upper airway compression Flow volume loop............................................ A normal flow volume loop is often described as a 'triangle on top of a semi circle' Flow volume loops are the most suitable way of assessing compression of the upper airway 13 Question 12 of 106 Which one of the following types of lung cancer is most associated with cavitating lesions? A. Carcinoid B. Large cell C. Small cell D. Squamous cell E. Adenocarcinoma ----------------------------------------------------- D. Squamous cell Whilst the other types of lung cancer may cause cavitating lesions, it is most commonly seen in squamous cell cancer CXR: cavitating lung lesion............................................ Differential tuberculosis lung cancer (especially squamous cell) abscess (Staph aureus, Klebsiella and Pseudomonas) Wegener's granulomatosis pulmonary embolism rheumatoid arthritis aspergillosis, histoplasmosis, coccidioidomycosis 14 Question 13 of 106 A 46-year-old female with a history of rheumatoid arthritis is investigated due to progressive shortness of breath. She is currently treated with methotrexate and ibuprofen. The following results are obtained from spirometry: FEV1/FVC 45% What is the most likely cause of the dyspnoea? A. Bronchiolitis obliterans B. Methotrexate pneumonitis C. Pulmonary fibrosis D. Caplan's syndrome E. Lung cancer ----------------------------------------------------- A. Bronchiolitis obliterans The spirometry reveals an obstructive picture which would be in keeping with bronchiolitis obliterans Rheumatoid arthritis: respiratory manifestations............................................ A variety of respiratory problems may be seen in patients with rheumatoid arthritis: pulmonary fibrosis pleural effusion pulmonary nodules bronchiolitis obliterans complications of drug therapy e.g. methotrexate pneumonitis pleurisy Caplan's syndrome - massive fibrotic nodules with occupational coal dust exposure infection (possibly atypical) secondary to immunosuppression 15 Question 14 of 106 A 41-year-old female presents with 3 day history of a dry cough and shortness of breath. This was preceded by flu-like symptoms. On examination there is a symmetrical, erythematous rash with 'target' lesions over the whole body. What is the likely organism causing the symptoms? A. Pseudomonas B. Staphylococcus aureus C. Mycoplasma pneumoniae D. Chlamydia pneumoniae E. Legionella pneumophilia ----------------------------------------------------- C. Mycoplasma pneumoniae Pneumococcus may also cause erythema multiforme Mycoplasma pneumoniae............................................ Mycoplasma pneumoniae is a cause of atypical pneumonia which often affects younger patients. It is associated with a number of characteristic complications such as erythema multiforme and cold autoimmune haemolytic anaemia. Epidemics of Mycoplasma pneumoniae classically occur every 4 years. It is important to recognise atypical pneumonias as they may not respond to penicillins or cephalosporins Features flu-like symptoms classically precede a dry cough bilateral consolidation on x-ray complications may occur as below Complications cold agglutins (IgM) may cause an haemolytic anaemia, thrombocytopenia erythema multiforme, erythema nodosum meningoencephalitis, Guillain-Barre syndrome bullous myringitis: painful vesicles on the tympanic membrane pericarditis/myocarditis gastrointestinal: hepatitis, pancreatitis renal: acute glomerulonephritis Diagnosis Mycoplasma serology Management erythromycin/clarithromycin tetracyclines such as doxycycline are an alternative 16 Question 15 of 106 A 45-year-old man is noted to have bilateral hilar lymphadenopathy on chest x-ray. Which one of the following is the least likely cause? A. Amyloidosis B. Sarcoidosis C. Histoplasmosis D. Tuberculosis E. Berylliosis ----------------------------------------------------- A. Amyloidosis Amyloidosis is not commonly associated with bilateral hilar lymphadenopathy............................................ The most common causes of bilateral hilar lymphadenopathy are sarcoidosis and tuberculosis Other causes include: lymphoma/other malignancy pneumoconiosis e.g. berylliosis fungi e.g. histoplasmosis, coccidioidomycosis 17 Question 16 of 106 A 65-year-old woman with a history of chronic obstructive pulmonary disease (COPD) is admitted to the Emergency Department with breathlessness. This is her first admission with an exacerbation of COPD. Blood gases taken on room air shortly after admission are as follows: pH 7.38 pCO2 4.9 kPa pO2 8.8 kPa What should her target oxygen saturations be? A. 94-98% B. 88-92% C. 92-94% D. >98% E. > 95% first 48 hours, > 90% rest of admission ----------------------------------------------------- A. 94-98% Oxygen therapy............................................ The British Thoracic Society published guidelines on emergency oxygen therapy in 2008. The following selected points are taken from the guidelines. Please see the link provided for the full guideline. Oxygen saturation targets acutely ill patients: 94-98% patients at risk of hypercapnia (e.g. COPD patients): 88-92% (see below) oxygen should be reduced in stable patients with satisfactory oxygen saturation Management of COPD patients prior to the availability of blood gases, use a 28% Venturi mask at 4 l/min and aim for an oxygen saturation of 88-92% for patients with risk factors for hypercapnia but no prior history of respiratory acidosis adjust target range to 94-98% if the pCO2 is normal Situations where oxygen therapy should not be used routinely if there is no evidence of hypoxia: myocardial infarction and acute coronary syndromes stroke obstetric emergencies anxiety-related hyperventilation 18 Question 17 of 106 A 62-year-old female with a 40 pack year history of smoking is investigated for a chronic cough associated with haemoptysis. Bronchoscopy reveals a small 1 cm tumour confined to the right main bronchus. A biopsy taken shows small cell lung cancer. What is the most appropriate management? A. Laser therapy B. Chemotherapy C. Surgery D. Radiotherapy E. Interferon-alpha ----------------------------------------------------- B. Chemotherapy Surgery plays little role in the management of small cell lung cancer, with chemotherapy being the mainstay of treatment cancer: small cell............................................ Features usually central arise from APUD* cells associated with ectopic ADH, ACTH secretion ADH --> hyponatraemia ACTH --> Cushing's syndrome ACTH secretion can cause bilateral adrenal hyperplasia, the high levels of cortisol can lead to hypokalaemic alkalosis Lambert-Eaton syndrome: antibodies to voltage gated calcium channels causing myasthenic like syndrome Management usually metastatic disease by time of diagnosis surgery: only used for debulking radiotherapy: only used for debulking chemotherapy: good response to combination chemotherapy, may extend life by approximately 4 months *an acronym for Amine - high amine content Precursor Uptake - high uptake of amine precursors Decarboxylase - high content of the enzyme decarboxylase 19 Question 18 of 106 A chest x-ray of a patient with sarcoidosis shows bilateral hilar lymphadenopathy but is otherwise normal. What chest x-ray stage does this correspond to? A. Stage 0 B. Stage 1 C. Stage 2 D. Stage 3 E. Stage 4 ----------------------------------------------------- B. Stage 1 Sarcoidosis CXR 1 = BHL 2 = BHL + infiltrates 3 = infiltrates 4 = fibrosis Sarcoidosis: investigation............................................ There is no one diagnostic test for sarcoidosis and hence diagnosis is still largely clinical. ACE levels have a sensitivity of 60% and specificity of 70% and are therefore not reliable in the diagnosis of sarcoidosis although they may have a role in monitoring disease activity. Routine bloods may show hypercalcaemia (seen in 10% if patients) and a raised ESR A chest x-ray may show the following changes: stage 0 = normal stage 1 = bilateral hilar lymphadenopathy (BHL) stage 2 = BHL + interstitial infiltrates stage 3 = diffuse interstitial infiltrates only stage 4 = diffuse fibrosis Other investigations* spirometry: may show a restrictive defect tissue biopsy: non-caseating granulomas gallium-67 scan - not used routinely *the Kveim test (where part of the spleen from a patient with known sarcoidosis is injected under the skin) is no longer performed due to concerns about crossinfection 20 Question 19 of 106 A 20-year-old man who has a family history of alpha-1 antitrypsin deficiency has genetic testing. The following results are received: A1AT genotype PiMZ What is the most likely outcome? A. Weekly intravenous alpha1-antitrypsin protein concentrates in later life B. Mild emphysema controlled with bronchodilator therapy C. Death within 5-10 years D. Lung transplantation in later life E. No evidence of lung disease ----------------------------------------------------- E. No evidence of lung disease Heterozygote patients such as those with the PiMZ genotype have alpha-1 antitrypsin levels approximately 35% of normal. They therefore have a low risk of developing clinically evident lung disease. Alpha-1 antitrypsin deficiency............................................ Alpha-1 antitrypsin (A1AT) deficiency is a common inherited condition caused by a lack of a protease inhibitor (Pi) normally produced by the liver Genetics located on chromosome 14 inherited in an autosomal recessive / co-dominant fashion* alleles classified by their electrophoretic mobility - M for normal, S for slow, and Z for very slow normal = PiMM homozygous PiSS (50% normal A1AT levels) homozygous PiZZ (10% normal A1AT levels) Features patients who manifest disease usually have PiZZ genotype lungs: panacinar emphysema, most marked in lower lobes liver: cirrhosis and hepatocellular carcinoma in adults, cholestasis in children Investigations A1AT concentrations Management no smoking supportive: bronchodilators, physiotherapy intravenous alpha1-antitrypsin protein concentrates surgery: volume reduction surgery, lung transplantation *trusted sources are split on which is a more accurate description 21 Question 20 of 106 A 52-year-old woman with a history of breast cancer is admitted with acute dyspnoea. Her respiratory rate on admission is 42 / min and her oxygen saturations are 87% on room air. A pulmonary embolism is suspected and she is transferred to the high dependency unit after being treated with oxygen and enoxaparin. Which one of the following would be strongest indication for thrombolysis? A. Extensive deep venous thrombosis B. Hypotension C. Patient choice following informed consent D. Hypoxaemia despite high flow oxygen E. ECG showing right ventricular strain ----------------------------------------------------- B. Hypotension Massive PE + hypotension – thrombolyse Pulmonary embolism: management............................................ Unfortunately there is a lack of clear guidelines on the optimal length of anticoagulation following a pulmonary embolism. The 2003 British Thoracic Society guidelines which advocate a shorter duration of treatment are not widely followed. The following is based on the 2005 British Committee for Standards in Haematology (BCSH) guidelines and Clinical Knowledge Summaries. Initial anticoagulation with heparin low molecular weight heparin (LMWH), rather than unfractionated heparin (UFH), should be used routinely in patients with suspected pulmonary embolism. This reflects the equal efficacy and safety of LMWHs as well as their ease of use exceptions include patients with a massive PE or in situations where rapid reversal of anticoagulation may be necessary Ongoing anticoagulation with warfarin target INR 2.0 - 3.0, length of treatment: calf DVT: at least 6 weeks proximal DVT or PE where there is transient risk factors: at least 3 months idiopathic venous thromboembolism or permanent risk factors: at least 6 months Thrombolysis thrombolysis is now recommended as the first-line treatment for massive PE where there is circulatory failure (e.g. Hypotension). Other invasive approaches should be considered where appropriate facilities exist 22 Question 21 of 106 A 35-year-old female with sarcoidosis is started on a course of prednisolone. Which one of the following is a suitable indication for commencing steroid therapy in such patients? A. Bilateral hilar lymphadenopathy B. Arthralgia C. Hypercalcaemia D. Serum ACE > 120 u/l E. Erythema nodosum ----------------------------------------------------- C. Hypercalcaemia Sarcoidosis: management............................................ Sarcoidosis is a multisystem disorder of unknown aetiology characterised by noncaseating granulomas. It is more common in young adults and in people of African descent Indications for steroids hypercalcaemia worsening lung function eye, heart or neuro involvement 23 Question 22 of 106 Which one of the following is least associated with Kartagener's syndrome? A. Male subfertility B. Recurrent sinusitis C. Malabsorption D. Dextrocardia E. Bronchiectasis ----------------------------------------------------- C. Malabsorption Kartagener's syndrome............................................ Kartagener's syndrome (also known as primary ciliary dyskinesia) was first described in 1933 and most frequently occurs in examinations due to its association with dextrocardia (e.g. 'quiet heart sounds', 'small volume complexes in lateral leads') Features dextrocardia or complete situs inversus bronchiectasis recurrent sinusitis subfertility (secondary to diminished sperm motility and defective ciliary action in the fallopian tubes) 24 Question 23 of 106 A 24-year-old female with a history of anxiety is taken to the Emergency Department following an acute onset of shortness of breath. On examination the chest is clear to auscultation but the respiratory rate is raised at 40 breaths per minute. A diagnosis of hyperventilation secondary to anxiety is suspected. Which of the following arterial blood gas results (taken on room air) are consistent with this? A. pH = 7.56; pCO2 = 2.9 kPa; pO2 = 10.1 kPa B. pH = 7.24; pCO2 = 8.4 kPa; pO2 = 12.7 kPa C. pH = 7.34; pCO2 = 2.7 kPa; pO2 = 15.4 kPa D. pH = 7.54; pCO2 = 2.4 kPa; pO2 = 14.1 kPa E. pH = 7.54; pCO2 = 4.9 kPa; pO2 = 13.3 kPa ----------------------------------------------------- D. pH = 7.54; pCO2 = 2.4 kPa; pO2 = 14.1 kPa Hyperventilation will result in carbon dioxide being 'blown off', causing an alkalosis. Whilst the gases in answer A show a respiratory alkalosis the hypoxia could not be explained by hyperventilation Respiratory alkalosis............................................ Common causes anxiety leading to hyperventilation pulmonary embolism salicylate poisoning* CNS disorders: stroke, subarachnoid haemorrhage, encephalitis altitude pregnancy *salicylate overdose leads to a mixed respiratory alkalosis and metabolic acidosis. Early stimulation of the respiratory centre leads to a respiratory alkalosis whilst later the direct acid effects of salicylates (combined with acute renal failure) may lead to an acidosis 25 Question 24 of 106 Which one of the following markers is most useful for monitoring the progression of patients with chronic obstructive pulmonary disease? A. FEV1/FVC ratio B. Lifestyle questionnaire C. Oxygen saturations D. FEV1 E. Number of exacerbations per year ----------------------------------------------------- D. FEV1 COPD: investigation and diagnosis............................................ The following investigations are recommended in patients with suspected COPD: spirometry to demonstrate airflow obstruction: forced expiratory volume in 1 second (FEV1) less than 80% of the predicted value and FEV1/FVC ratio less than 70% chest x-ray: hyperinflation, bullae, flat hemidiaphragm. Also important to exclude lung cancer full blood count: exclude secondary polycythaemia The severity of COPD is categorised using the FEV1: Severity FEV1 (of predicted) Mild 50–80% Moderate 30–49% Severe < 30% Measuring peak expiratory flow is of limited value in COPD, as it may underestimate the degree of airflow obstruction 26 Question 25 of 106 A 19-year-old man presents as he is concerned he may be asthmatic. Which one of the following points in the history would make this diagnosis less likely? A. Smoking since age of 16 years B. Peripheral tingling during episodes C. Peripheral blood eosinophilia D. Chest tightness whilst exercising E. History of eczema ----------------------------------------------------- B. Peripheral tingling during episodes The British Thoracic Society suggest peripheral tingling is one of the factors which makes a diagnosis of asthma less likely. His smoking history does not preclude a diagnosis of asthma Asthma: diagnosis in adults............................................ The 2008 British Thoracic Society guidelines marked a subtle change in the approach to diagnosing asthma. It suggests dividing patients into a high, intermediate and low probability of having asthma based on the presence or absence of typical symptoms. A list can be found in the external link but include typical symptoms such as wheeze, nocturnal cough etc Example of features used to assess asthma (not complete, please see link) Increase possibility of asthma Wheeze, breathlessness, chest tightness and cough, worse at night/early morning History of atopic disorder Wheeze heard on auscultation Unexplained peripheral blood eosinophilia Decrease possibility of asthma Prominent dizziness, lightheadedness, peripheral tingling Chronic productive cough in the absence of wheeze or breathlessness Repeatedly normal physical examination Significant smoking history (i.e. > 20 pack-years) Normal PEF or spirometry when Symptomatic 27 Management is based on this assessment: high probability: trial of treatment intermediate probability: see below low probability: investigate/treat other condition For patients with an intermediate probability of asthma further investigations are suggested. The guidelines state that spirometry is the preferred initial test: FEV1/FVC < 0.7: trial of treatment FEV1/FVC > 0.7: further investigation/consider referral Recent studies have shown the limited value of other 'objective' tests. It is now recognised that in patients with normal or near-normal pre-treatment lung function there is little room for measurable improvement in FEV1 or peak flow. A > 400 ml improvement in FEV1 is considered significant before and after 400 mcg inhaled salbutamol in patients with diagnostic uncertainty and airflow obstruction present at the time of assessment if there is an incomplete response to inhaled salbutamol, after either inhaled corticosteroids (200 mcg twice daily beclometasone equivalent for 6-8 weeks) or oral prednisolone (30 mg once daily for 14 days) It is now advised to interpret peak flow variability with caution due to the poor sensitivity of the test diurnal variation % = [(Highest – Lowest PEFR) / Highest PEFR] x 100 assessment should be made over 2 weeks greater than 20% diurnal variation is considered significant 28 Question 26 of 106 A 44-year-old man who is known to be HIV positive presents with shortness- ofbreath. Which one of the following features is most characteristic of Pneumocystis carinii pneumonia? A. Usually occurs when the CD4 count is 200-300/mm³ B. Absence of fever C. Productive cough D. Oxygen saturations usually improve after short period of exertion E. Normal chest auscultation ----------------------------------------------------- E. Normal chest auscultation HIV: Pneumocystis jiroveci pneumonia............................................ Whilst the organism Pneumocystis carinii is now referred to as Pneumocystis jiroveci, the term Pneumocystis carinii pneumonia (PCP) is still in common use Pneumocystis jiroveci is an unicellular eukaryote, generally classified as a fungus but some authorities consider it a protozoa PCP is the most common opportunistic infection in AIDS all patients with a CD4 count < 200/mm³ should receive PCP prophylaxis Features dyspnoea dry cough fever very few chest signs Extrapulmonary manifestations are rare (1-2% of cases), may cause hepatosplenomegaly lymphadenopathy choroid lesions Investigation CXR: typically shows bilateral interstitial pulmonary infiltrates but can present with other x-ray findings e.g. lobar consolidation. May be normal exercise-induced desaturation sputum often fails to show PCP, bronchoalveolar lavage (BAL) often needed to demonstrate PCP (silver stain) Management co-trimoxazole IV pentamidine in severe cases steroids if hypoxic (if pO2 < 9.3kPa then steroids reduce risk of respiratory failure by 50% and death by a third) 29 Question 27 of 106 A 31-year-old woman is referred to the acute medical unit with a 4 day history of polyarthritis and a low-grade pyrexia. Examination reveals shin lesions which the patient states are painful. Chest x-ray shows a bulky mediastinum. What is the most likely diagnosis? A. Loffler's syndrome B. Lofgren's syndrome C. Systemic lupus erythematous D. Gonococcal arthritis E. Reiter's syndrome ----------------------------------------------------- B. Lofgren's syndrome Loffler's syndrome is a cause of pulmonary eosinophilia thought to be caused by parasites such as Ascaris lumbricoides Lofgren's syndrome............................................ Lofgren's syndrome is an acute form sarcoidosis characterised by bilateral hilar lymphadenopathy (BHL), erythema nodosum, fever and polyarthralgia. It typically occurs in young females and carries an excellent prognosis 30 Question 28 of 106 A 24-year-old woman who is 34 weeks pregnant presents with pleuritic chest pain and shortness of breath. She has noticed some pain in her left calf for the past 3 days and on examination she has clinical signs consistent with a left calf deep vein thrombosis. What is the most appropriate investigation? A. D-dimer B. Compression duplex Doppler C. Computed tomographic pulmonary angiography D. Venogram E. Ventilation-perfusion scan ----------------------------------------------------- B. Compression duplex Doppler Confirming a DVT is the first step as this may provide indirect evidence of a pulmonary embolism. As both conditions require anticoagulation this may negate the need for further radiation exposure. Pregnancy: DVT/PE investigation............................................ Guidelines were published in 2007 by the Royal College of Obstetricians. Key points include: chest x-ray should be performed in all patients - compression duplex Doppler should be performed if the chest x-ray is normal – this may provide indirect evidence of a pulmonary embolism and negate the need for further radiation exposure the decision to perform a V/Q or CTPA should be taken at a local level after discussion with the patient and radiologist CTPA exposes the fetus to about 10-30% of the radiation dose of a V/Q scan V/Q scanning exposes the maternal breast tissue to less radiation than a CTPA D-dimer is of no use in the investigation of thromboembolism as it raised in Pregnancy 31 Question 29 of 106 A 62-year-old female is admitted with a suspected infective exacerbation of COPD. A chest x-ray shows no evidence of consolidation. What is the most likely causative organism? A. Pseudomonas aeruginosa B. Haemophilus influenzae C. Staphylococcus aureus D. Streptococcus pneumoniae E. Moraxella catarrhalis ----------------------------------------------------- B. Haemophilus influenzae COPD: acute management............................................ The most common bacterial organisms that cause infective exacerbations of COPD are: Haemophilus influenzae (most common cause) Streptococcus pneumoniae Moraxella catarrhalis Respiratory viruses account for around 30% of exacerbations, with the human rhinovirus being the most important pathogen. 32 Question 30 of 106 A 49-year-old male with a past history of alcohol excess presents to the Emergency Department due to fever and shortness of breath. Chest x-ray reveals a cavitating lesion in the right middle zone. What is the most likely causative organism? A. Klebsiella B. Bartonella C. Pneumococcus D. Coxiella burnetii E. Haemophilus influenzae ----------------------------------------------------- A. Klebsiella Klebsiella pneumonia (Friedlander's pneumonia) typically occurs in middle- aged alcoholic men. Chest x-ray features may include abscess formation in the middle/upper lobes and empyema. The mortality approaches 30-50% CXR: cavitating lung lesion............................................ Differential tuberculosis lung cancer (especially squamous cell) abscess (Staph aureus, Klebsiella and Pseudomonas) Wegener's granulomatosis pulmonary embolism rheumatoid arthritis aspergillosis, histoplasmosis, coccidioidomycosis 33 Question 31 of 106 Which one of the following paraneoplastic features is least commonly seen in patients with squamous cell lung cancer?ia A. Lambert-Eaton syndrome B. Hyperthyroidism C. Hypertrophic pulmonary osteoarthropathy D. Hypercalcaemia E. Clubbing ----------------------------------------------------- A. Lambert-Eaton syndrome Paraneoplastic features of lung cancer squamous cell: PTHrp, clubbing, HPOA small cell: ADH, ACTH, Lambert-Eaton syndrome Lambert-Eaton syndrome occurs almost exclusively in small cell lung cancer Lung cancer: paraneoplastic features............................................ Small cell ADH ACTH - not typical, hypertension, hyperglycaemia, hypokalaemia, alkalosis and muscle weakness are more common than buffalo hump etc Lambert-Eaton syndrome Squamous cell PTH-rp clubbing hypertrophic pulmonary osteoarthropathy (HPOA) hyperthyroidism due to ectopic TSH Adenocarcinoma gynaecomastia 34 Question 32 of 106 A 24-year-old female comes for review. She was diagnosed with asthma two years ago and is currently using a salbutamol inhaler 100mcg prn combined with beclometasone dipropionate inhaler 200mcg bd. Despite this her asthma is not well controlled. On examination her chest is clear and she has a good inhaler technique. What is the most appropriate next step in management? A. Increase beclometasone dipropionate to 400mcg bd B. Switch steroid to fluticasone propionate C. Trial of leukotriene receptor antagonist D. Add salmeterol E. Add tiotropium ----------------------------------------------------- D. Add salmeterol The British Thoracic Society recommend adding a long-acting B2 agonist if there is an inadequate response to the addition of inhaled steroid. The inhaled steroid dose should be increased if there is an inadequate response to the long-acting B2 agonist. Asthma: stepwise management in adults............................................ The management of stable asthma is now well established with a step-wise approach: Step 1 Inhaled short-acting B2 agonist as required Step 2 Add inhaled steroid at 200-800 mcg/day* 400 mcg is an appropriate starting dose for many patients. Start at dose of inhaled steroid appropriate to severity of disease Step 3 1. Add inhaled long-acting B2 agonist (LABA) 2. Assess control of asthma: good response to LABA - continue LABA benefit from LABA but control still inadequate: continue LABA and increase inhaled steroid dose to 800 mcg/day* (if not already on this dose) no response to LABA: stop LABA and increase inhaled steroid to 800 mcg/ day.* If control still inadequate, institute trial of other therapies, leukotriene receptor antagonist or SR theophylline 35 Step 4 Consider trials of: increasing inhaled steroid up to 2000 mcg/day* addition of a fourth drug e.g. Leukotriene receptor antagonist, SR theophylline, B2 agonist tablet Step 5 Use daily steroid tablet in lowest dose providing adequate control. Consider other treatments to minimise the use of steroid tablets Maintain high dose inhaled steroid at 2000 mcg/day* Refer patient for specialist care *beclometasone dipropionate or equivalent Additional notes Leukotriene receptor antagonists e.g. Montelukast, zafirlukast have both anti-inflammatory and bronchodilatory properties should be used when patients are poorly controlled on high-dose inhaled corticosteroids and a long-acting b2-agonist particularly useful in aspirin-induced asthma associated with the development of Churg-Strauss syndrome Fluticasone is more lipophilic and has a longer duration of action than beclometasone Hydrofluoroalkane is now replacing chlorofluorocarbon as the propellant of choice. Only half the usually dose is needed with hydrofluoroalkane due to the smaller size of the particles Long acting B2-agonists acts as bronchodilators but also inhibit mediator release from mast cells. Recent meta-analysis showed adding salmeterol improved symptoms compared to doubling the inhaled steroid dose 36 Question 33 of 106 A 24-year-old heroin addict is admitted following an overdose. He is drowsy and has a respiratory rate of 6 / min. Which of the following arterial blood gas results (taken on room air) are most consistent with this? A. pH = 7.49; pCO2 = 4.9 kPa; pO2 = 10.1 kPa B. pH = 7.52; pCO2 = 2.9 kPa; pO2 = 13.1 kPa C. pH = 7.31; pCO2 = 7.4 kPa; pO2 = 8.1 kPa D. pH = 7.55; pCO2 = 3.4 kPa; pO2 = 14.3 kPa E. pH = 7.32; pCO2 = 3.4 kPa; pO2 = 8.3 kPa ----------------------------------------------------- C. pH = 7.31; pCO2 = 7.4 kPa; pO2 = 8.1 kPa This patient is likely to have developed a respiratory acidosis secondary to hypoventilation. Respiratory acidosis............................................ Respiratory acidosis may be caused by a number of conditions COPD decompensation in other respiratory conditions e.g. Life-threatening asthma / pulmonary oedema sedative drugs: benzodiazepines, opiate overdose 37 Question 34 of 106 Which one of the following interventions is most likely to increase survival in patients with COPD? A. Home nebulisers B. Prophylactic antibiotic therapy C. Pulmonary rehabilitation D. Long-term steroid therapy E. Long-term oxygen therapy ----------------------------------------------------- E. Long-term oxygen therapy After smoking cessation, long-term oxygen therapy is one of the few interventions that has been shown to improve survival in COPD COPD: stable management............................................ General management smoking cessation advice annual influenza vaccination one-off pneumococcal vaccination Bronchodilator therapy short acting beta2-agoinst or anticholinergic is first-line treatment if still symptomatic add a long-acting anti-cholinergic (e.g. Tiotropium) or a long-acting beta2-agonist (e.g. Salmeterol) Inhaled steroids NICE recommends that an inhaled corticosteroid should for patients with an FEV1 < 50% of predicted (i.e. Moderate or severe COPD), who are having two or more exacerbations requiring treatment with antibiotics or oral corticosteroids in a 12 month period reduce frequency of exacerbations If the patient is symptomatic despite the above measures oral theophylline The role of mucolytics is still being evaluated but they appear to reduce exacerbation frequency and duration Factors which may improve survival in patients with stable COPD smoking cessation - the single most important intervention in patients who are still smoking long term oxygen therapy in patients who fit criteria lung volume reduction surgery in selected patients 38 Question 35 of 106 A 67-year-old female is referred to the acute medical unit with an infective exacerbation of COPD. Despite maximal medical therapy the arterial blood gases continue to show type II respiratory failure. You are asked to consider non-invasive ventilation. At what pH is the patient most likely to receive benefit from non-invasive ventilation? A. pH 7.13 B. pH 7.18 C. pH 7.23 D. pH 7.29 E. pH 7.37 ----------------------------------------------------- D. pH 7.29 The evidence surrounding the use of NIV in COPD shows that patients with a pH in the range of 7.25-7.35 achieve the most benefit. If the pH is < 7.25 then invasive ventilation should be considered if appropriate Non-invasive ventilation............................................ The British Thoracic Society (BTS) published guidelines in 2002 on the use of noninvasive ventilation in acute respiratory failure Non-invasive ventilation - key indications COPD with respiratory acidosis pH 7.25-7.35 type II respiratory failure secondary to chest wall deformity, neuromuscular disease or obstructive sleep apnoea cardiogenic pulmonary oedema unresponsive to CPAP weaning from tracheal intubation Recommended initial settings for bi-level pressure support in COPD Expiratory Positive Airway Pressure (EPAP): 4-5 cm H2O Inspiratory Positive Airway Pressure (IPAP): RCP advocate 10 cm H20 whilst BTS suggest 12-15 cm H2O back up rate: 15 breaths/min back up inspiration:expiration ratio: 1:3 39 Question 36 of 106 Each one of the following may result in bronchiectasis, except: A. Kartagener's syndrome B. Amyloidosis C. Selective IgA deficiency D. Lung cancer E. Allergic bronchopulmonary aspergillosis ----------------------------------------------------- B. Amyloidosis Amyloidosis does not cause bronchiectasis per se, but may be seen in bronchiectasis as a consequence of chronic inflammation and infection Bronchiectasis: causes............................................ Bronchiectasis describes a permanent dilatation of the airways secondary to chronic infection or inflammation. There are a wide variety of causes are listed below: Causes post-infective: tuberculosis, measles, pertussis, pneumonia cystic fibrosis bronchial obstruction e.g. lung cancer/foreign body immune deficiency: selective IgA, hypogammaglobulinaemia allergic bronchopulmonary aspergillosis (ABPA) ciliary dyskinetic syndromes: Kartagener's syndrome, Young's syndrome yellow nail syndrome 40 Question 37 of 106 A 54-year-old man is investigated for a chronic cough. A chest x-ray arranged by his GP shows a suspicious lesion in the right lung. He has no past history of note and is a life-long non-smoker. An urgent bronchoscopy is arranged which is normal. What is the most likely diagnosis? A. Lung sarcoma B. Squamous cell lung cancer C. Lung adenocarcinoma D. Small cell lung cancer E. Lung carcinoid ----------------------------------------------------- C. Lung adenocarcinoma Lung adenocarcinoma most common type in non-smokers peripheral lesion The clues are the absence of a smoking history and normal bronchoscopy, which suggests a peripherally located lesion. Lung cancer: non-small cell............................................ There are three main subtypes of non-small cell lung cancer: Squamous cell cancer typically central associated with ectopic PTH secretion --> hypercalcaemia strongly associated with finger clubbing hypertrophic pulmonary osteoarthropathy (HPOA) Adenocarcinoma most common type of lung cancer in non-smokers, although the majority of patients who develop lung adenocarcinoma are smokers typically located on the lung periphery Large cell lung carcinoma 41 Question 38 of 106 A 52-year-old man is admitted to hospital with breathlessness and fever. He has no significant past medical history of note and has not seen a doctor for over 5 years. On examination the temperature is 38.4TC, respiratory rate is 24 / min, pulse is 84 / min and the blood pressure is 118/72 mmHg. A chest x-ray reveals right lower lobe consolidation. Arterial blood gases on air are as follows: pH 7.39 pCO2 4.6 kPa pO2 9.8 kPa What is the most suitable antibiotic therapy? A. Oral co-amoxiclav B. Oral amoxicillin + erythromycin C. Oral amoxicillin D. IV ceftriaxone + clarithromycin E. Oral co-amoxiclav + metronidazole ----------------------------------------------------- C. Oral amoxicillin Uncomplicated - give only amoxicillin if: treated in the community, or admitted to hospital for non-clinical reasons, or not previously treated in the community The 2004 British Thoracic Society guidelines recommend oral amoxicillin as the first-line antibiotic for hospitalised patients with non-severe community acquired pneumonia if they have not previously been treated in the community or have been admitted for non-clinical reasons Pneumonia: community-acquired............................................ Community acquired pneumonia (CAP) may be caused by the following infectious agents: Streptococcus pneumoniae (accounts for around 80% of cases) Haemophilus influenzae Staphylococcal aureus atypical pneumonias (e.g. Due to Mycoplasma pneumoniae) viruses Klebsiella pneumoniae is classically in alcoholics Streptococcus pneumoniae (pneumococcus) is the most common cause of community-acquired pneumonia Characteristic features of pneumococcal pneumonia rapid onset high fever pleuritic chest pain herpes labialis Antibiotic choices home-treated uncomplicated CAP: first line - oral amoxicillin hospitalized uncomplicated CAP: if admitted for non-clinical reasons or not previously treated in the community for this episode then oral amoxicillin, otherwise amoxicillin + macrolide 42 Question 39 of 106 A 24-year-old male is admitted with acute severe asthma. Treatment is initiated with 100% oxygen, nebulised salbutamol and ipratropium bromide nebulisers and IV hydrocortisone. Despite initial treatment there is no improvement. What is the next step in management? A. IV aminophylline B. IV magnesium sulphate C. IV salbutamol D. Non-invasive ventilation E. IV adrenaline ----------------------------------------------------- B. IV magnesium sulphate Current guidelines do not support the routine use of non-invasive ventilation in asthmatics. Asthma: acute severe............................................ Patients with acute severe asthma are stratified into moderate, severe or lifethreatening Moderate Severe Life-threatening PEF > 50% best or PEF 33 - 50% best or PEF < 33% best or predicted predicted predicted Speech normal Can't complete Oxygen sats < 92% RR < 25 / min sentences Silent chest, cyanosis or Pulse < 110 bpm RR > 25/min feeble respiratory effort Pulse > 110 bpm Bradycardia, dysrhythmia or hypotension Exhaustion, confusion or coma British Thoracic Society guidelines 2003 (updated 2004) magnesium sulphate recommended as next step for patients who are not responding (e.g. 1.2 - 2g IV over 20 mins) little evidence to support use of IV aminophylline (although still mentioned in management plans) if no response consider IV salbutamol 43 Question 40 of 106 A 62-year-old man who is investigated for haemoptysis is found to have squamous cell lung cancer. Which one of the following is a contraindication to surgery? A. Pleural effusion B. Superior vena caval obstruction C. Haemoptysis D. Hypercalcaemia E. Enlarged mediastinal lymph nodes ----------------------------------------------------- B. Superior vena caval obstruction Contraindications to lung cancer surgery include SVC obstruction, FEV < 1.5, MALIGNANT pleural effusion, and vocal cord paralysis If one of the options was 'malignant pleural effusion', rather than just 'pleural effusion' (which may be reactive), then this would also be a contraindication Lung cancer: non-small cell management............................................ Management only 20% suitable for surgery mediastinoscopy performed prior to surgery as CT does not always show mediastinal lymph node involvement curative or palliative radiotherapy poor response to chemotherapy Surgery contraindications assess general health stage IIIb or IV (i.e. metastases present) FEV1 < 1.5 litres is considered a general cut-off point* malignant pleural effusion tumour near hilum vocal cord paralysis SVC obstruction * However if FEV1 < 1.5 for lobectomy or < 2.0 for pneumonectomy then some authorities advocate further lung function tests as operations may still go ahead based on the results 44 Question 41 of 106 A 55-year-old man is referred to the medical admissions unit. He recently returned from a holiday in Italy and has failed to respond to a course of co- amoxiclav for a suspected lower respiratory tract infection. Chest x-ray shows bilateral infiltrates. Bloods are as follows: Na+ 122 mmol/l K+ 4.3 mmol/l Urea 8.4 mmol/l Creatinine 130 µmol/l What is the likely diagnosis? A. Goodpasture's syndrome B. Legionella pneumonia C. Pneumocystis carinii pneumonia D. Pulmonary eosinophilia E. Mycoplasma pneumonia ----------------------------------------------------- B. Legionella pneumonia Legionella............................................ Legionnaire's disease is caused by the intracellular bacterium Legionella pneumophilia. It is typically colonizes water tanks and hence questions may hint at air-conditioning systems or foreign holidays. Person-to-person transmission is not seen Features flu-like symptoms dry cough lymphopenia hyponatraemia deranged LFTs Diagnosis urinary antigen Management treat with erythromycin 45 Question 42 of 106 A 58-year-old man is investigated for a chronic cough and is found to have lung cancer. He enquires whether it may be work related. Which one of the following is most likely to increase his risk of developing lung cancer?ia A. Isocyanates B. Soldering flux resin C. Passive smoking D. Coal dust E. Polyvinyl chloride ----------------------------------------------------- C. Passive smoking Whilst many chemicals have been implicated in the development of lung cancer passive smoking is the most likely cause. Up to 15% of lung cancers in patients who do not smoke are thought to be caused by passive smoking Lung cancer: risk factors............................................ Smoking increases risk of lung ca by a factor of 10 Other factors asbestos - increases risk of lung ca by a factor of 5 arsenic radon nickel chromate aromatic hydrocarbon cryptogenic fibrosing alveolitis Factors that are NOT related coal dust Smoking and asbestos are synergistic, i.e. a smoker with asbestos exposure has a 10 * 5 = 50 times increased risk 46 Question 43 of 106 Which one of the following is least associated with the development of COPD? A. Cadmium exposure B. Smoking C. Coal dust D. Isocyanates E. Alpha-1 antitrypsin deficiency ----------------------------------------------------- D. Isocyanates Isocyanates are more associated with occupational asthma COPD: causes............................................ Smoking! Alpha-1 antitrypsin deficiency Other causes cadmium (used in smelting) coal cotton cement grain 47 Question 44 of 106 Each one of the following predisposes to the development of obstructive sleep apnoea, except: A. Acromegaly B. Chronic obstructive pulmonary disease C. Amyloidosis D. Obesity E. Hypothyroidism ----------------------------------------------------- B. Chronic obstructive pulmonary disease Sleep apnoea causes include obesity and macroglossia The Sleep Heart Health Study showed that when these two conditions do coexist, this is the result of chance alone Obstructive sleep apnoea/hypopnoea syndrome............................................ Predisposing factors obesity macroglossia: acromegaly, hypothyroidism, amyloidosis large tonsils Marfan's syndrome Consequence daytime somnolence hypertension SIGN guidelines for the diagnosis and management of patients with OSAHS were published in 2003 Assessment of sleepiness Epworth Sleepiness Scale - questionnaire completed by patient +/- partner Multiple Sleep Latency Test (MSLT) - measures the time to fall asleep in a dark room (using EEG criteria) Diagnostic tests sleep studies - ranging from monitoring of pulse oximetry at night to full polysomnography where a wide variety of physiological factors are measured including EEG, respiratory airflow, thoraco-abdominal movement, snoring and pulse oximetry Management weight loss CPAP is first line for moderate or severe OSAHS intra-oral devices (e.g. mandibular advancement) may be used if CPAP is not tolerated or for patients with mild OSAHS where there is no daytime sleepiness limited evidence to support use of pharmacological agents 48 Question 45 of 106 A 30-year-old female with a past history of asthma presents to the Emergency Department with shortness of breath. Chest x-ray shows a right-sided pneumothorax with a 1.5cm rim of air and no mediastinal shift. What is the most appropriate management? A. Admit for 48 hours observation B. Intercostal drain insertion C. Aspiration D. Discharge E. Immediate 14G cannula into 2nd intercostal space, mid-clavicular Line ----------------------------------------------------- C. Aspiration This should be treated as a secondary pneumothorax as the patient has a history of Asthma Pneumothorax............................................ The British Thoracic Society (BTS) published guidelines for the management of spontaneous pneumothorax in 2003. A pneumothorax is termed primary if there is no underlying lung disease and secondary if there is Primary pneumothorax Recommendations include: if the rim of air is < 2cm and the patient is not short of breath then discharge should be considered otherwise aspiration should be attempted if this fails then repeat aspiration should be considered if this fails then a chest drain should be inserted Secondary pneumothorax Recommendations include: if the patient is > 50 years old and the rim of air is > 2cm and the patient is short of breath then a chest drain should be inserted. otherwise aspiration should be attempted. If aspiration fails a chest drain should be inserted. All patients should be admitted for at least 24 hours Iatrogenic pneumothorax Recommendations include: less likelihood of recurrence than spontaneous pneumothorax majority will resolve with observation, if treatment is required then aspiration should be used ventilated patients need chest drains, as may some patients with COPD 49 Question 46 of 106 A 54-year-old man is admitted with suspected pulmonary embolism. He has no past medical history of note. Blood pressure is 120/80 mmHg with a pulse of 90/min. The chest x-ray is normal. Following treatment with low-molecular weight heparin, what is the most appropriate initial lung imaging investigation to perform? A. Pulmonary angiography B. Echocardiogram C. MRI thorax D. Ventilation-perfusion scan E. Computed tomographic pulmonary angiography ----------------------------------------------------- E. Computed tomographic pulmonary angiography CTPA is the first line investigation for PE according to current BTS guidelines This is a difficult question to answer as both computed tomographic pulmonary angiography (CTPA) and ventilation-perfusion scanning are commonly used in UK clinical practice. The 2003 British Thoracic Society (BTS) guidelines, however, recommended that CTPA is now used as the initial lung imaging modality of choice. Pulmonary angiography is of course the 'gold standard' but this is not what the question asks for Pulmonary embolism: investigation............................................ The British Thoracic Society (BTS) published guidelines in 2003 on the management of patients with suspected pulmonary embolism (PE) Key points from the guidelines include: computed tomographic pulmonary angiography (CTPA) is now the recommended initial lung- imaging modality for non-massive PE. Advantages compared to V/Q scans include speed, easier to perform out-of-hours, a reduced need for further imaging and the possibility of providing an alternative diagnosis if PE is excluded if the CTPA is negative then patients do not need further investigations or treatment for PE ventilation-perfusion scanning may be used initially if appropriate facilities exist, the chest x-ray is normal, and there is no significant symptomatic concurrent cardiopulmonary disease Some other points Clinical probability scores based on risk factors and history and now widely used to help decide on further investigation/management D-dimers sensitivity = 95-98%, but poor specificity V/Q scan sensitivity = 98%; specificity = 40% - high negative predictive value, i.e. if normal virtually excludes PE other causes of mismatch in V/Q include old pulmonary embolisms, AV malformations, vasculitis, previous radiotherapy COPD gives matched defects CTPA peripheral emboli affecting subsegmental arteries may be missed Pulmonary angiography the gold standard significant complication rate compared to other investigations 50 Question 47 of 106 A 60-year-old woman who has recently been diagnosed with chronic obstructive pulmonary disease (COPD) presents for review. She is still occasionally breathless despite bronchodilator therapy. Which one of the following criteria is most relevant when deciding who would benefit from inhaled corticosteroids? A. Failure of long-acting beta2-agonist to improve breathlessness B. FEV1/FVC < 50% predicted C. FEV1 < 50% predicted and 2 or more exacerbations per year D. FEV1/FVC < 50% predicted and 3 or more exacerbations per year E. Four or more than exacerbations per year ----------------------------------------------------- C. FEV1 < 50% predicted and 2 or more exacerbations per year COPD: stable management............................................ General management smoking cessation advice annual influenza vaccination one-off pneumococcal vaccination Bronchodilator therapy short acting beta2-agoinst or anticholinergic is first-line treatment if still symptomatic add a long-acting anti-cholinergic (e.g. Tiotropium) or a long-acting beta2-agonist (e.g. Salmeterol) Inhaled steroids NICE recommends that an inhaled corticosteroid should for patients with an FEV1 < 50% of predicted (i.e. Moderate or severe COPD), who are having two or more exacerbations requiring treatment with antibiotics or oral corticosteroids in a 12 month period reduce frequency of exacerbations If the patient is symptomatic despite the above measures oral theophylline The role of mucolytics is still being evaluated but they appear to reduce exacerbation frequency and duration Factors which may improve survival in patients with stable COPD smoking cessation - the single most important intervention in patients who are still smoking long term oxygen therapy in patients who fit criteria lung volume reduction surgery in selected patients 51 Question 48 of 106 Which type of hypersensitivity reaction predominates in the acute phase of extrinsic allergic alveolitis? A. Type I B. Type II C. Type III D. Type IV E. Type V ----------------------------------------------------- C. Type III Although it is known that the pathogenesis of extrinsic allergic alveolitis involves a type IV (delayed) hypersensitivity reaction, a type III hypersensitivity reaction is thought to predominate, especially in the acute phase Extrinsic allergic alveolitis............................................ Extrinsic allergic alveolitis (EAA) is a condition caused by hypersensitivity induced lung damage due to a variety of inhaled organic particles. It is thought to be largely caused by immune-complex mediated tissue damage (type III hypersensitivity) although delayed hypersensitivity (type IV) is also thought to play a role in EAA, especially in the chronic phase Examples bird fanciers' lung (avian proteins) farmers lung (spores of Micropolyspora faeni) malt workers' lung (Aspergillus clavatus) mushroom workers' lung (thermophilic actinomycetes*) Presentation acute: occur 4-8 hrs after exposure, SOB, dry cough, fever chronic Investigation CXR: upper lobe fibrosis BAL: lymphocytosis blood: NO eosinophilia *here the terminology is slightly confusing as thermophilic actinomycetes is an umbrella term covering strains such as Micropolyspora faeni 52 Question 49 of 106 A 31-year-old female with no past medical history of note is admitted to hospital with dyspnoea and fever. She has recently returned from holiday in Turkey. A clinical diagnosis of pneumonia is made. On examination she is noted to have an ulcerated lesion on her upper lip consistent with reactivation of herpes simplex. Which organism is most associated with this examination finding? A. Legionella pneumophilia B. Staphylococcal aureus C. Streptococcus pneumoniae D. Pneumocystis carinii E. Mycoplasma pneumoniae ----------------------------------------------------- C. Streptococcus pneumoniae Streptococcus pneumoniae commonly causes reactivation of the herpes simplex virus resulting in 'cold sores' Pneumonia: community-acquired............................................ Community acquired pneumonia (CAP) may be caused by the following infectious agents: Streptococcus pneumoniae (accounts for around 80% of cases) Haemophilus influenzae Staphylococcal aureus atypical pneumonias (e.g. Due to Mycoplasma pneumoniae) viruses Klebsiella pneumoniae is classically in alcoholics Streptococcus pneumoniae (pneumococcus) is the most common cause of community-acquired pneumonia Characteristic features of pneumococcal pneumonia rapid onset high fever pleuritic chest pain herpes labialis Antibiotic choices home-treated uncomplicated CAP: first line - oral amoxicillin hospitalized uncomplicated CAP: if admitted for non-clinical reasons or not previously treated in the community for this episode then oral amoxicillin, otherwise amoxicillin + macrolide 53 Question 50 of 106 A 63-year-old man presents to the respiratory out-patients department with shortness of breath. Investigations reveal a fibrosing lung disease. A sputum sample however is positive for acid-fast bacilli. Which of the following may have predisposed him to developing tuberculosis? A. Cadmium B. Coal dust C. White asbestos fibres D. Blue asbestos fibres E. Silica ----------------------------------------------------- E. Silica Silicosis............................................ Silicosis is a risk factor for developing TB (silica is toxic to macrophages) Features fibrosing lung disease 'egg-shell' calcification of hilar lymph nodes 54 Question 51 of 106 A preliminary diagnosis of extrinsic allergic alveolitis in a 55-year-old man. Which one of the following features would most support this diagnosis? A. Clubbing B. Eosinophilia C. Cyanosis D. Fibrosis in the upper zones E. History of working in the steel industry ----------------------------------------------------- D. Fibrosis in the upper zones A history of working in the steel industry and eosinophilia are not features of extrinsic allergic alveolitis. Clubbing and cyanosis are non-specific Extrinsic allergic alveolitis............................................ Extrinsic allergic alveolitis (EAA) is a condition caused by hypersensitivity induced lung damage due to a variety of inhaled organic particles. It is thought to be largely caused by immune-complex mediated tissue damage (type III hypersensitivity) although delayed hypersensitivity (type IV) is also thought to play a role in EAA, especially in the chronic phase Examples bird fanciers' lung (avian proteins) farmers lung (spores of Micropolyspora faeni) malt workers' lung (Aspergillus clavatus) mushroom workers' lung (thermophilic actinomycetes*) Presentation acute: occur 4-8 hrs after exposure, SOB, dry cough, fever chronic Investigation CXR: upper lobe fibrosis BAL: lymphocytosis blood: NO eosinophilia *here the terminology is slightly confusing as thermophilic actinomycetes is an umbrella term covering strains such as Micropolyspora faeni 55 Question 52 of 106 You review a 27-year-old woman in the Emergency Department who has been admitted with an acute exacerbation of her asthma. Which one of the following features is most likely to indicate a life-threatening attack? A. Failure to improve after nebulised salbutamol 5mg B. Cannot complete sentences C. Oxygen saturations of 94% on room air D. Peak flow of 30% best or predicted E. Respiratory rate of 42 / min ----------------------------------------------------- D. Peak flow of 30% best or predicted Asthma: acute severe............................................ Patients with acute severe asthma are stratified into moderate, severe or lifethreatening Moderate Severe Life-threatening PEF > 50% best or PEF 33 - 50% best or PEF < 33% best or predicted predicted predicted Speech normal Can't complete Oxygen sats < 92% RR < 25 / min sentences Silent chest, cyanosis or Pulse < 110 bpm RR > 25/min feeble respiratory effort Pulse > 110 bpm Bradycardia, dysrhythmia or hypotension Exhaustion, confusion or coma British Thoracic Society guidelines 2003 (updated 2004) magnesium sulphate recommended as next step for patients who are not responding (e.g. 1.2 - 2g IV over 20 mins) little evidence to support use of IV aminophylline (although still mentioned in management plans) if no response consider IV salbutamol 56 Question 53 of 106 Which one of the following is not part of the diagnostic criteria of acute respiratory distress syndrome (ARDS)? A. Bilateral infiltrates on CXR B. Non-cardiogenic C. pO2/FiO2 < 200 mmHg D. Respiratory rate > 24/min E. Acute onset ----------------------------------------------------- D. Respiratory rate > 24/min ARDS............................................ Basics acute respiratory distress syndrome caused by increased permeability of alveolar capillaries leading to fluid accumulation in alveoli i.e. non-cardiogenic pulmonary oedema Criteria (American-European Consensus Conference) acute onset bilateral infiltrates on CXR non-cardiogenic (pulmonary artery wedge pressure needed if doubt) pO2/FiO2 < 200 mmHg Causes infection: sepsis, pneumonia massive blood transfusion trauma smoke inhalation pancreatitis cardio-pulmonary bypass 57 Question 54 of 106 Which one of the following is least associated with bronchiectasis? A. Hypogammaglobulinaemia B. Allergic bronchopulmonary aspergillosis C. Measles D. Cystic fibrosis E. Sarcoidosis ----------------------------------------------------- E. Sarcoidosis Traction bronchiectasis may be seen in some rare cases of stage IV pulmonary sarcoidosis but this is the least strong association of the five options Bronchiectasis: causes............................................ Bronchiectasis describes a permanent dilatation of the airways secondary to chronic infection or inflammation. There are a wide variety of causes are listed below: Causes post-infective: tuberculosis, measles, pertussis, pneumonia cystic fibrosis bronchial obstruction e.g. lung cancer/foreign body immune deficiency: selective IgA, hypogammaglobulinaemia allergic bronchopulmonary aspergillosis (ABPA) ciliary dyskinetic syndromes: Kartagener's syndrome, Young's syndrome yellow nail syndrome 58 Question 55 of 106 A 62-year-old man with a history of recurrent lower respiratory tract infections is diagnosed as having bilateral bronchiectasis following a high resolution CT scan. Which one of the following is most important in controlling his symptoms? A. Inhaled corticosteroids B. Prophylactic antibiotics C. Surgery D. Postural drainage E. Mucolytic therapy ----------------------------------------------------- D. Postural drainage Symptom control in non-CF bronchiectasis - inspiratory muscle training + postural drainage Bronchiectasis: management............................................ Bronchiectasis describes a permanent dilatation of the airways secondary to chronic infection or inflammation. After assessing for treatable causes (e.g. immune deficiency) management is as follows: physical training (e.g. inspiratory muscle training) - has a good evidence base for patients with non-cystic fibrosis bronchiectasis postural drainage antibiotics for exacerbations + long-term rotating antibiotics in severe cases bronchodilators in selected cases immunisations surgery in selected cases (e.g. Localised disease) Most common organisms isolated from patients with bronchiectasis: Haemophilus influenzae (most common) Pseudomonas aeruginosa Klebsiella spp. Streptococcus pneumoniae 59 Question 56 of 106 Which of the following features is associated with a good prognosis in sarcoidosis? A. Insidious onset B. Splenomegaly C. Disease in black people D. Stage III features on CXR E. Erythema nodosum ----------------------------------------------------- E. Erythema nodosum Erythema nodosum is associated with a good prognosis in sarcoidosis Sarcoidosis: prognostic features............................................ Sarcoidosis is a multisystem disorder of unknown aetiology characterised by noncaseating granulomas. It is more common in young adults and in people of African descent. Sarcoidosis remits without treatment in approximately two-thirds of people Factors associated with poor prognosis insidious onset, symptoms > 6 months absence of erythema nodosum extrapulmonary manifestations: e.g. lupus pernio, splenomegaly CXR: stage III-IV features black people 60 Question 57 of 106 A 57-year-old female presents to the Emergency Department with shortness of breath and pleuritic chest pain. She has no past medical history of note and enjoys good health. Investigations reveal a non-massive pulmonary embolism. What is the recommended length of warfarinisation for this patient? A. 6 weeks B. 3 months C. 6 months D. 12 months E. Life-long ----------------------------------------------------- C. 6 months There are no transient risk factors for venous thromboembolism therefore the patient should be anticoagulated for 6 months. Pulmonary embolism: management............................................ Unfortunately there is a lack of clear guidelines on the optimal length of anticoagulation following a pulmonary embolism. The 2003 British Thoracic Society guidelines which advocate a shorter duration of treatment are not widely followed. The following is based on the 2005 British Committee for Standards in Haematology (BCSH) guidelines and Clinical Knowledge Summaries. Initial anticoagulation with heparin low molecular weight heparin (LMWH), rather than unfractionated heparin (UFH), should be used routinely in patients with suspected pulmonary embolism. This reflects the equal efficacy and safety of LMWHs as well as their ease of use exceptions include patients with a massive PE or in situations where rapid reversal of anticoagulation may be necessary Ongoing anticoagulation with warfarin target INR 2.0 - 3.0, length of treatment: calf DVT: at least 6 weeks proximal DVT or PE where there is transient risk factors: at least 3 months idiopathic venous thromboembolism or permanent risk factors: at least 6 months Thrombolysis thrombolysis is now recommended as the first-line treatment for massive PE where there is circulatory failure (e.g. Hypotension). Other invasive approaches should be considered where appropriate facilities exist 61 Question 58 of 106 A 43-year-old man is admitted due to shortness of breath and is noted to have a cavitating lesion on his chest x-ray. Which one of the following conditions is not part of the differential diagnosis? A. Lung cancer B. Pulmonary embolism C. Wegener's granulomatosis D. Churg-Strauss syndrome E. Tuberculosis ----------------------------------------------------- D. Churg-Strauss syndrome CXR: cavitating lung lesion............................................ Differential tuberculosis lung cancer (especially squamous cell) abscess (Staph aureus, Klebsiella and Pseudomonas) Wegener's granulomatosis pulmonary embolism rheumatoid arthritis aspergillosis, histoplasmosis, coccidioidomycosis 62 Question 59 of 106 Which one the following statements regarding asbestos is not correct? A. Pleural plaques are premalignant B. Asbestosis typically affects the lower zones C. Crocidolite (blue) asbestos is the most dangerous form D. Severity of asbestosis is related to the length of exposure E. Mesothelioma may develop following minimal exposure ----------------------------------------------------- A. Pleural plaques are premalignant Asbestos and the lung............................................ Asbestos can cause a variety of lung disease from benign pleural plaques to mesothelioma. Pleural plaques Pleural plaques are benign and do not undergo malignant change. They are the most common form of asbestos related lung disease and generally occur after a latent period of 20-40 years. Pleural thickening Asbestos exposure may cause diffuse pleural thickening in a similar pattern to that seen following an empyema or haemothorax. The underlying pathophysiology is not fully understood. Asbestosis The severity of asbestosis is related to the length of exposure. This is in contrast to mesothelioma where even very limited exposure can cause disease. The latent period is typically 15-30 years. Asbestosis typically causes lower lobe fibrosis. As with other forms of lung fibrosis the most common symptoms are shortness-ofbreath and reduced exercise tolerance. Mesothelioma Mesothelioma is a malignant disease of the pleura. Crocidolite (blue) asbestos is the most dangerous form. Possible features progressive shortness-of-breath chest pain pleural effusion Patients are usually offered palliative chemotherapy and there is also a limited role for surgery and radiotherapy. Unfortunately the prognosis is very poor, with a median survival from diagnosis of 8-14 months. Lung cancer Asbestos exposure is a risk factor for lung cancer and also has a synergistic effect with cigarette smoke. 63 Question 60 of 106 Each one of the following is a known cause of occupational asthma, except: A. Isocyanates B. Cadmium C. Soldering flux resin D. Flour E. Platinum salts ----------------------------------------------------- B. Cadmium Asthma: occupational............................................ Causes isocyanates platinum salts soldering flux resin glutaraldehyde flour epoxy resins proteolytic enzymes Diagnosis specific recommendations are made in the 2007 joint British Thoracic Society and SIGN guidelines serial measurements of peak expiratory flow are recommended at work and away from work 64 Question 61 of 106 Which one of the following causes of lung fibrosis predominately affect the lower zones? A. Methotrexate B. Sarcoidosis C. Coal worker's pneumoconiosis D. Ankylosing spondylitis E. Extrinsic allergic alveolitis ----------------------------------------------------- A. Methotrexate Lung fibrosis............................................ It is important in the exam to be able to differentiate between conditions causing predominately upper or lower zone fibrosis. It should be noted that the more common causes (cryptogenic fibrosing alveolitis, drugs) tend to affect the lower zones Fibrosis predominately affecting the upper zones extrinsic allergic alveolitis coal worker's pneumoconiosis/progressive massive fibrosis silicosis sarcoidosis ankylosing spondylitis (rare) histiocytosis tuberculosis Fibrosis predominately affecting the lower zones cryptogenic fibrosing alveolitis most connective tissue disorders (except ankylosing spondylitis) drug-induced: amiodarone, bleomycin, methotrexate asbestosis 65 Question 62 of 106 A 39-year-old man presents with shortness of breath following one week of flu-like symptoms. He also has a non-productive cough but no chest pain. A chest x-ray shows bilateral consolidation and examination reveals erythematous lesions on his limbs and trunk. Which one of the following investigations is most likely to be diagnostic? A. Cold agglutins B. Sputum culture C. Urinary antigen for Legionella D. Serology for Mycoplasma E. Blood culture ----------------------------------------------------- D. Serology for Mycoplasma Mycoplasma? - serology is diagnostic The flu-like symptoms, bilateral consolidation and erythema multiforme point to a diagnosis of Mycoplasma. The most appropriate diagnostic test is Mycoplasma serology Mycoplasma pneumoniae............................................ Mycoplasma pneumoniae is a cause of atypical pneumonia which often affects younger patients. It is associated with a number of characteristic complications such as erythema multiforme and cold autoimmune haemolytic anaemia. Epidemics of Mycoplasma pneumoniae classically occur every 4 years. It is important to recognise atypical pneumonias as they may not respond to penicillins or cephalosporins Features flu-like symptoms classically precede a dry cough bilateral consolidation on x-ray complications may occur as below Complications cold agglutins (IgM) may cause an haemolytic anaemia, thrombocytopenia erythema multiforme, erythema nodosum meningoencephalitis, Guillain-Barre syndrome bullous myringitis: painful vesicles on the tympanic membrane pericarditis/myocarditis gastrointestinal: hepatitis, pancreatitis renal: acute glomerulonephritis Diagnosis Mycoplasma serology Management erythromycin/clarithromycin tetracyclines such as doxycycline are an alternative 66 Question 63 of 106 Which of the following is not a recognised cause of pulmonary eosinophilia? A. Churg-Strauss syndrome B. Sulphonamides C. Extrinsic allergic alveolitis D. Loffler's syndrome E. Allergic bronchopulmonary aspergillosis ----------------------------------------------------- C. Extrinsic allergic alveolitis Pulmonary eosinophilia............................................ Causes of pulmonary eosinophilia Churg-Strauss syndrome allergic bronchopulmonary aspergillosis (ABPA) Loffler's syndrome eosinophilic pneumonia hypereosinophilic syndrome tropical pulmonary eosinophilia drugs: nitrofurantoin, sulphonamides less common: Wegener's granulomatosis Loffler's syndrome transient CXR shadowing and blood eosinophilia thought to be due to parasites such as Ascaris lumbricoides causing an alveolar reaction presents with a fever, cough and night sweats which often last for less than 2 weeks. generally a self-limiting disease Tropical pulmonary eosinophilia associated with Wuchereria bancrofti infection 67 Question 64 of 106 A 65-year-old man with a history of Parkinson's disease is referred to the respiratory clinic with shortness of breath. He has never smoked. Spirometry is performed: Percentage Predicted FEV1 71% FVC 74% Which one of the following drugs is most likely to be responsible? A. Levodopa B. Entacapone C. Ropinirole D. Selegiline E. Pergolide ----------------------------------------------------- E. Pergolide This patient has developed pulmonary fibrosis (explaining the restrictive picture on spirometry) secondary to pergolide therapy Parkinson's disease: management............................................ Currently accepted practice in the management of patients with Parkinson's disease (PD) is to delay treatment until the onset of disabling symptoms and then to introduce a dopamine receptor agonist. If the patient is elderly, levodopa is sometimes used as an initial treatment. Dopamine receptor agonists e.g. Bromocriptine, ropinirole, cabergoline, apomorphine ergot-derived dopamine receptor agonists (bromocriptine, cabergoline, pergolide*) have been associated with pulmonary, retroperitoneal and cardiac fibrosis. The Committee on Safety of Medicines advice that an echocardiogram, ESR, creatinine and chest x-ray should be obtained prior to treatment and patients should be closely monitored patients should be warned about the potential for dopamine receptor agonists to cause impulse control disorders and excessive daytime somnolence 68 Levodopa usually combined with a decarboxylase inhibitor (e.g. Carbidopa or benserazide) to prevent peripheral metabolism of levodopa to dopamine reduced effectiveness with time (usually by 2 years) unwanted effects: dyskinesia, 'on-off' effect no use in neuroleptic induced parkinsonism MAO-B (Monoamine Oxidase-B) inhibitors e.g. Selegiline inhibits the breakdown of dopamine secreted by the dopaminergic neurons Amantadine mechanism is not fully understood, probably increases dopamine release and inhibits its uptake at dopaminergic synapses COMT (Catechol-O-Methyl Transferase) inhibitors e.g. Entacapone COMT is an enzyme involved in the breakdown of dopamine, and hence may be used as an adjunct to levodopa therapy used in established PD Antimuscarinics block cholinergic receptors now used more to treat drug-induced parkinsonism rather than idiopathic Parkinson's disease help tremor and rigidity e.g. Procyclidine, benzotropine, trihexyphenidyl (benzhexol *pergolide was withdrawn from the US market in March 2007 due to concern regarding increased incidence of valvular dysfunction 69 Question 65 of 106 A 45-year-old female with a 30 pack-year history of smoking is admitted to the Emergency Department with shortness of breath. Arterial blood gases taken on room air are as follows: pH 7.49 pCO2 2.9 kPa pO2 8.8 kPa Which one of the following is the most likely diagnosis? A. Salicylate overdose B. Chronic obstructive pulmonary disease C. Pulmonary embolism D. Vomiting E. Anxiety ----------------------------------------------------- C. Pulmonary embolism Pulmonary embolism needs to be excluded. Even with a significant smoking history a reduced pO2 should not be attributed to anxiety. A salicylate overdose would not account for a reduced pO2, unless it is severe enough to have caused pulmonary oedema. This option is much less likely than a pulmonary embolism Respiratory alkalosis............................................ Common causes anxiety leading to hyperventilation pulmonary embolism salicylate poisoning* CNS disorders: stroke, subarachnoid haemorrhage, encephalitis altitude pregnancy *salicylate overdose leads to a mixed respiratory alkalosis and metabolic acidosis. Early stimulation of the respiratory centre leads to a respiratory alkalosis whilst later the direct acid effects of salicylates (combined with acute renal failure) may lead to an acidosis 70 Question 66 of 106 A 56-year-old man is admitted with type II respiratory failure secondary to COPD but fails to respond to maximal medical therapy. It is decided that a trial of noninvasive ventilation in the form of bi-level pressure support should be given. What are the most appropriate initial settings for the ventilator? A. IPAP = 10 cm H2O; EPAP = 5 cm H2O B. IPAP = 15 cm H2O; EPAP = 15 cm H2 C. IPAP = 50 cm H2O; EPAP = 20 cm H2O D. IPAP = 20 cm H2O; EPAP = 50 cm H2O E. IPAP = 5 cm H2O; EPAP = 12 cm H2O ----------------------------------------------------- A. IPAP = 10 cm H2O; EPAP = 5 cm H2O The 2008 Royal College of Physicians guidelines recommend an initial IPAP of 10 cm H20. The 2002 British Thoracic Society guidelines had previously advocated starting at 12-15 cm H20 Non-invasive ventilation............................................ The British Thoracic Society (BTS) published guidelines in 2002 on the use of noninvasive ventilation in acute respiratory failure Non-invasive ventilation - key indications COPD with respiratory acidosis pH 7.25-7.35 type II respiratory failure secondary to chest wall deformity, neuromuscular disease or obstructive sleep apnoea cardiogenic pulmonary oedema unresponsive to CPAP weaning from tracheal intub