Medicine Marrow Pg 251-260 (Pulmonology)
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Medicine Marrow Pg 251-260 (Pulmonology)

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Which of the following findings would be considered unlikely in the diagnosis of ABPA?

  • Bronchial wall thickening (correct)
  • Consolidation
  • Pleural involvement
  • Lymphadenopathy

    • Pleural involvement is a finding against the diagnosis of ABPA.

    True

    What is the first-line treatment for ABPA?

    12 weeks steroid and airway clearance device/hypertonic saline

    The treatment regimen for a steroid-dependent or relapsed ABPA patient includes _____ therapy for 16 weeks.

    <p>Itraconazole</p> Signup and view all the answers

    Match the following findings against the diagnosis of ABPA with their categories:

    <p>Pleural involvement = Against Lymphadenopathy = Against Small nodules = Unlikely Bronchial wall thickening = Unlikely</p> Signup and view all the answers

    Which of the following describes the primary pathology associated with hypersensitivity pneumonitis?

    <p>Non caseating granulomas and eosinophilia</p> Signup and view all the answers

    Hypersensitivity pneumonitis can occur in smokers.

    <p>False</p> Signup and view all the answers

    What is the most common antigen associated with Farmer's lung?

    <p>Thermoactinomyces vulgaris</p> Signup and view all the answers

    The primary type of hypersensitivity involved in hypersensitivity pneumonitis is _____.

    <p>Type IV</p> Signup and view all the answers

    Match the disease related to hypersensitivity pneumonitis with its respective source:

    <p>Farmer's lung = Moldy hay Bagassosis = Moldy sugarcane Bird fancier's lung = Pigeon/parrot/chicken Mushroom worker's lung = Moldy compost/mushroom</p> Signup and view all the answers

    Which of the following imaging characteristics is associated with Chronic Eosinophilic Pneumonia (CEP)?

    <p>Reverse bat-wing appearance</p> Signup and view all the answers

    Idiopathic Hypereosinophilic Syndrome is characterized by eosinophilia in peripheral blood exceeding 1500/µl for less than 6 months.

    <p>False</p> Signup and view all the answers

    What kind of imaging findings are common in Acute Eosinophilic Pneumonia?

    <p>Bilateral infiltrates</p> Signup and view all the answers

    Chronic Eosinophilic Pneumonia shows peripheral pulmonary opacities predominantly in the ______ lung.

    <p>right</p> Signup and view all the answers

    Match the following conditions with their respective peripheral eosinophils findings:

    <p>Acute Eosinophilic Pneumonia = Leukocytosis with increased eosinophils Chronic Eosinophilic Pneumonia = &gt; 240% eosinophils Idiopathic Hypereosinophilic Syndrome = &gt; 1500 /µl for &gt;6 months</p> Signup and view all the answers

    What is a key clinical feature of the acute form of pulmonary conditions?

    <p>Fever and chills</p> Signup and view all the answers

    Farmer's lung can lead to fibrosis.

    <p>False</p> Signup and view all the answers

    Which type of hypersensitivity is associated with a positive S. precipitin test?

    <p>Type III hypersensitivity</p> Signup and view all the answers

    Bird fancier's lung rarely causes _____ changes.

    <p>fibrosis</p> Signup and view all the answers

    Match the pulmonary conditions with their associated characteristics:

    <p>Farmer's lung = Emphysematous changes Cryptogenic organising pneumonia = Non-specific lung inflammation Coal worker's pneumoconiosis = Rare condition Bird fancier's lung = Rare fibrosis</p> Signup and view all the answers

    What is a common characteristic feature of bronchiectasis in allergic bronchopulmonary aspergillosis (ABPA)?

    <p>Bilateral upper lobe central bronchiectasis</p> Signup and view all the answers

    Eosinophilic inflammation in ABPA is mediated by Th₁ cells.

    <p>False</p> Signup and view all the answers

    What does HRCT thorax reveal in a patient with ABPA?

    <p>Central bronchiectasis</p> Signup and view all the answers

    The typical symptom of ABPA includes a fever and a cough with _____ expectoration.

    <p>brownish thick mucosal plug</p> Signup and view all the answers

    Match the clinical features with their corresponding percentages in ABPA:

    <p>Crackles = 20% Clubbing = 15% Recurrent acute exacerbation = Common Upper lobe fibrosis = Rare</p> Signup and view all the answers

    What is the normal amount of pleural fluid in the pleural cavity?

    <p>10-20 mL</p> Signup and view all the answers

    The visceral pleura is sensitive to pain.

    <p>False</p> Signup and view all the answers

    What is the main cell type found in pleural fluid, comprising 70% of its composition?

    <p>Macrophages</p> Signup and view all the answers

    Light's criteria is used to distinguish between exudative and ______ pleural effusions.

    <p>transudative</p> Signup and view all the answers

    Match the following pleural fluid components with their typical percentages:

    <p>Macrophages = 70% Lymphocytes = 25% Mesothelial cells = 1-5%</p> Signup and view all the answers

    Which of the following symptoms is associated with the acute component of pulmonary eosinophilia?

    <p>Dry cough</p> Signup and view all the answers

    Marked peripheral eosinophilia is defined as > 3000 cells/mm³.

    <p>True</p> Signup and view all the answers

    What is the first-line treatment for pulmonary eosinophilia?

    <p>Diethylcarbamazine (DEC)</p> Signup and view all the answers

    The presence of bilateral, indefinite mottling in lung fields is a radiological finding seen in ______.

    <p>pulmonary eosinophilia</p> Signup and view all the answers

    Match the following features with their respective condition:

    <p>Acute Eosinophilic Pneumonia = Viral fever-like symptoms, lasts 3-4 weeks Chronic Eosinophilic Pneumonia = Subacute presentation, night sweats Idiopathic Hypereosinophilic Syndrome = Multisystem involvement Myeloproliferative Disorder = JAK2/PDGFRA mutation</p> Signup and view all the answers

    What type of immune response is primarily involved in Allergic Broncho Pulmonary Aspergillosis (ABPA)?

    <p>Both Type I and III hypersensitivity</p> Signup and view all the answers

    Eosinophilia is not commonly observed in Allergic Broncho Pulmonary Aspergillosis (ABPA).

    <p>False</p> Signup and view all the answers

    What is the causative organism of Allergic Broncho Pulmonary Aspergillosis (ABPA)?

    <p>Aspergillus fumigatus</p> Signup and view all the answers

    In patients with ABPA, mucus plugs contain __________ in the bronchial wall.

    <p>septate hyphae</p> Signup and view all the answers

    Match the following manifestations of lung conditions with their descriptions:

    <p>ABPA = Eosinophilic inflammation in asthma Aspergilloma = Infection in an underlying cavity Chronic necrotizing aspergillosis = Bronchial wall invasion in COPD Angio invasive aspergillosis = Blood stream infection in neutropenic patients</p> Signup and view all the answers

    Which of the following is a key criteria for diagnosing Allergic Bronchopulmonary Aspergillosis (ABPA)?

    <p>Presence of eosinophilia greater than 500/μL</p> Signup and view all the answers

    A positive immediate skin test for Aspergillus is part of the obligatory criteria for diagnosing ABPA.

    <p>True</p> Signup and view all the answers

    What is the most useful test for evaluating ABPA?

    <p>IgE levels</p> Signup and view all the answers

    In ABPA, a predominant pattern observed in pulmonary function tests (PFT) is __________.

    <p>obstructive</p> Signup and view all the answers

    Match the imaging findings with their descriptions related to ABPA:

    <p>Fleeting infiltrates = Irregular, transient parenchymal infiltrates Finger in glove = Mucoid impaction in dilated bronchi Tram track shadows = Parallel linear shadows extending from the hilum Trees in bud = Cylindrical bronchiectasis with thickening of bronchial walls</p> Signup and view all the answers

    Which of the following is a common radiographic feature of hypersensitivity pneumonitis?

    <p>Reticulonodular opacities</p> Signup and view all the answers

    Skin test reactions to Aspergillus antigens are positive in hypersensitivity pneumonitis.

    <p>False</p> Signup and view all the answers

    What is the primary type of hypersensitivity involved in hypersensitivity pneumonitis?

    <p>Type IV hypersensitivity</p> Signup and view all the answers

    In chronic hypersensitivity pneumonitis, progressive lung damage can lead to __________.

    <p>fibrosis</p> Signup and view all the answers

    Match the following features with their respective conditions:

    <p>Non-caseating granulomas = Hypersensitivity pneumonitis Eosinophilic inflammation = Allergic Bronchopulmonary Aspergillosis CD4 &gt; 2 = Sarcoidosis Increased serum IgE levels = ABPA</p> Signup and view all the answers

    Study Notes

    X-Ray Findings against Diagnosis of ABPA

    • Bronchial wall thickening and small nodules are unlikely to indicate ABPA.
    • Pleural involvement, lymphadenopathy, consolidation, air fluid levels, and perihilar involvement are against a diagnosis of ABPA.

    Treatment of ABPA

    • Steroids are the first-line treatment for ABPA, administered for 12 weeks.
    • Itraconazole is a second-line therapy used in steroid-dependent or relapsing cases, or when there's no resolution with steroids.
    • Omalizumab is another treatment option.
    • IgE levels should be monitored periodically.

    Hypersensitivity Pneumonitis (HP)

    • HP, also known as extrinsic allergic alveolitis, is a lung disease typically affecting non-smokers.
    • Repeated inhalation of organic dust triggers a hypersensitivity reaction, primarily type IV, but also type III.
    • Inflammation affects the terminal airways, alveoli, and interstitium, predominantly involving lymphocytes.

    Pathology of HP

    • HP does not involve type I hypersensitivity.
    • Initially, diffuse pulmonary infiltrates are observed.
    • Non-caseating granulomas, eosinophilia, elevated IgE levels, migratory lung infiltrates, and a reticulo-nodular pattern are characteristic features.

    Etiology of HP

    • Farmer's lung is the most common type, caused by moldy hay containing Thermoactinomyces vulgaris.
    • Bagassosis is the second most common type, caused by moldy sugarcane.
    • Other HP types include:
      • Mushroom worker's lung, caused by moldy compost/mushroom (e.g., Aspergillus clavatus).
      • Compost lung, caused by compost containing Cladosporium or MAC (Mycobacterium avium complex).
      • Tobacco worker's lung, caused by mold on tobacco.
      • Malt worker's lung, caused by barley.
      • Cheese washer's lung.
      • Hot tub lung/Humidifier lung/Air conditioner lung, caused by avian droppings.
      • Bird fancier's lung, caused by pigeons, parrots, or chickens (e.g., Rhizopus).
      • Wood trimmer's lung, caused by wood pulp.
      • Familial HP, caused by polyurethane foams (e.g., Isocyanate) and Bacillus subtilis.
      • Chemical worker's lung.

    Acute Eosinophilic Pneumonia (AEP)

    • Peripheral blood: Leukocytosis.
    • Bronchoalveolar lavage (BAL): Increased eosinophils.
    • IgE: Increased.
    • X-ray: Bilateral infiltrates, resembling a negative of pulmonary edema.
    • CT: Diffuse parenchymal ground-glass opacity and consolidation.

    Chronic Eosinophilic Pneumonia (CEP)

    • BAL: Eosinophils greater than 240% of normal values.
    • Imaging: Reverse bat-wing appearance or photographic negative of pulmonary edema.
    • Perihilar sparing: Present.
    • Peripheral opacities: Present.
    • CT: Peripheral pulmonary opacities, predominantly on the right side.

    Idiopathic Hypereosinophilic Syndrome

    • Peripheral eosinophilia: Greater than 1500 cells per microliter for more than six months.
    • X-ray: Transient focal or diffuse pulmonary infiltrates.
    • CT: Peripheral pulmonary opacities, predominantly on the right side.

    Treatment of Eosinophilic Lung Diseases

    • AEP and CEP: Steroids.
    • Idiopathic hypereosinophilic syndrome: Steroids, but often unresponsive.

    Presentation of Hypersensitivity Pneumonitis

    • Acute: Self-resolving.
    • Chronic: Can cause fibrosis leading to interstitial lung disease (ILD).

    Clinical Features of HP

    • Acute form: Fever, chills, cough with an elevated erythrocyte sedimentation rate (ESR). The symptoms resolve within 2-4 days.
    • Subacute form: Gradual, progressive dyspnea and cough with fatigue. Systemic symptoms are absent. Upper lobe involvement is common.

    Complications of HP

    • Bird fancier's lung: Rarely causes fibrosis or ILD.
    • Farmer's lung: May cause emphysematous changes.

    Causes of Non-caseating Granulomas

    • HP, tuberculosis (30%), lymphoma, sarcoidosis, cat scratch disease, Crohn's disease, and berylliosis.

    Diseases Causing Emphysematous Changes

    • Farmer's lung, cryptogenic organizing pneumonia and (rarely) coal worker's pneumoconiosis.

    Management of HP

    • Investigations:
      • Serum precipitins: May be positive but are non-specific due to Type III hypersensitivity.
      • BAL: Shows lymphocytosis with CD8 cells exceeding CD4 cells.

    Allergic Bronchopulmonary Aspergillosis (ABPA)

    • Causative organism: Aspergillus fumigatus is the most common causative agent, followed by Aspergillus niger, Aspergillus flavus, and Aspergillus terreus.

    Identifying Features of Aspergillus

    • Conidiophore, septate hyphae, and saprophytic nature are key identifying features.

    Lung Manifestations of Aspergillus

    Condition Description
    ABPA Allergic Bronchopulmonary Aspergillosis
    Aspergilloma Occurs within pre-existing lung cavities, commonly from tuberculosis (TB)
    Chronic Necrotising Aspergillosis/Chronic Cavitating Pulmonary Aspergillosis Invasion of the bronchial wall, typically in patients with Chronic Obstructive Pulmonary Disease (COPD). Thick-walled cavities are characteristic.
    Angio Invasive Aspergillosis Found in immunocompromised individuals, including those with neutropenia or on long-term steroids, as well as those who have undergone bone marrow transplantation. Leads to bloodstream infection.

    Pathogenesis of ABPA

    • ABPA develops in patients with chronic asthma or cystic fibrosis.
    • Organism colonizes the airway, leading to a complex hypersensitivity reaction involving both type I and type III hypersensitivity.
    • Eosinophilia and elevated IgE levels are observed. This leads to eosinophilic inflammation and mucus plugs containing septate hyphae within the bronchial wall.
    • Migratory lung infiltrates are a common finding. Inflammation weakens the bronchial wall, resulting in bronchiectasis, cough with sputum, atelectasis, and bronchial mucosal plugs in sputum containing hyphae and eosinophils.

    Symptoms of ABPA

    • Acute on chronic disease:
      • Chronic component: Malaise, anorexia, weight loss, hepatosplenomegaly, and lymphadenopathy.
      • Acute component: Paroxysmal dry cough, dyspnea or wheezing, particularly at night, resembling status asthmaticus.

    Investigations for ABPA

    • Laboratory findings:

      • Significant peripheral eosinophilia (greater than 3000 cells per cubic millimeter).
      • BAL: Intense eosinophilic alveolitis (greater than 40%).
      • IgE: Greater than 1000 IU/mL.

    • Radiological findings:

      • Bilateral, indefinite mottling, uniformly throughout both lung fields, primarily in the middle and lower lobes.
      • Increased bronchovascular markings.
      • Cavitation and pleural effusion.

    Treatment of ABPA

    • The first-line treatment for ABPA is diethylcarbamazine (DEC), a medication effective against both adult filariаe and microfilariаe.
    • DEC is administered orally at a dose of 2 mg/kg three times a day (TID) for 14 to 21 days or up to four weeks.
    • Clinical improvement is typically seen within 7 days, and acute relapses occur in up to 20% of cases, requiring higher doses of DEC.

    Unknown Causes of Eosinophilic Lung Disease (ELD)

    Feature Acute Eosinophilic Pneumonia (AEP) Chronic Eosinophilic Pneumonia (CEP) Idiopathic Hypereosinophilic Syndrome Myeloproliferative Disorder (JAK2/PDGFRA mutation)
    History Restarted smoking Atopy (33–50%) - -
    Pathogenesis - - - -
    Presentation Viral fever-like symptoms (fever, cough) 2-3 days. Dyspnea, tachypnea, hypoxia (ARDS-like presentation). Lasts 3-4 weeks. Subacute presentation. Low-grade fever, wheeze, night sweats, weight loss. Cough, dyspnea, ARDS. - Multisystem involvement: End organ damage, RS + CNS + CVS + peripheral neuropathy.

    Notes on Unknown Causes of Eosinophilic Lung Disease

    • Respiratory system (RS) involvement: TB-like presentation, acute respiratory distress syndrome (ARDS), pulmonary hypertension (pulm HTN).
    • Central nervous system (CNS): Neuropsychiatric dysfunction (60%).
    • Cardiovascular system (CVS): Eosinophilic myocarditis, endomyocardial fibrosis.

    Evaluation of ABPA

    • Criteria for Diagnosis:

        1. Predisposing asthma or cystic fibrosis (CF).
        1. Obligatory Criteria:
        • IgE > 1000 IU/mL.
        • Positive immediate skin test or increased IgE antibody to Aspergillus.
        1. Supportive Criteria (at least 2):
        • Eosinophilia > 500/μL.
        • Presence of precipitins or elevated IgG antibody to Aspergillus.
        • Consistent radiographic opacities.

    • Most useful test: IgE levels.

    Pulmonary Function Testing (PFT) Pattern in ABPA

    • Obstructive pattern, predominantly due to bronchiectasis.
    • Stage 5 ABPA: Fibrosis, resulting in a restrictive pattern.

    Imaging Findings in ABPA

    • Fleeting infiltrates: Irregular, transient parenchymal infiltrates.
    • Finger in glove: Mucoid impaction in dilated bronchi.
    • Tramtrack shadows: Parallel linear shadows extending from the hilum into the bronchial distribution.
    • Trees in bud: Cylindrical and proximal bronchiectasis of the upper lobe with thickening of the bronchial walls (nodules).
    • Saccular dilatation: Visible saccular dilatation.

    Lung Conditions

    • ABPA (Allergic Bronchopulmonary Aspergillosis).
    • Langerhans cell histiocytosis.
    • Hypersensitivity pneumonitis.
    • Tuberculosis (TB).

    Features of Bronchiectasis in ABPA

    • Bilateral involvement.
    • Upper lobe involvement.
    • Central location in the upper lobes.
    • Proximal location in the upper lobes.
    • Tree in bud appearance.
    • Cystic bronchiectasis in central aspects.

    CT (Lung Window) in ABPA

    • Soft tissue upper lobe nodules represent mucoid impaction within dilated bronchi.

    Clinical Features of ABPA

    • Presentation: Recurrent acute exacerbations in patients with chronic, steroid-dependent, or treatment-refractory asthma or cystic fibrosis.
    • Symptoms: Fever, cough with expectoration (brownish, thick mucosal plug).
    • Signs: Present in only 15-20% of cases.
      • Crackles.
      • Clubbing.

    Course of ABPA

    • Rarely causes upper lobe fibrosis.

    Causes of Upper Lobe Fibrosis

    • Rheumatology:
      • Ankylosing spondylitis.
      • Sarcoidosis.

    HRCT Thorax in ABPA

    • Central bronchiectasis.

    CT Chest in ABPA

    • Hyperattenuated mucus.

    Pleural Anatomy

    • Pleural Cavity: Lined by mesothelial cells that produce surfactant.
    • Normal amount of pleural fluid (PF): 10-20 mL.
    • Negative intrapleural pressure: Maintained by pleural lymphatics and a vacuum.
    • Coupling Organ: Connects the lungs and chest wall.
    • Types of Pleura:
      • Parietal: Develops from somatopleuric mesoderm (pain-sensitive).
      • Visceral: Develops from splanchopleural mesoderm (pain-insensitive).
    • Minimum amount of Pleural Fluid for Detection:
      • Ultrasound and Lateral X-ray: 50 mL.
      • CXR Posterior-anterior (PA) View: 200 mL.
      • Clinically detectable: 300 mL.

    Composition of Pleural Fluid

    • Macrophages: 70%.
    • Lymphocytes: 25%.
    • Mesothelial cells: 1-5%.

    Pleural Fluid Chemistry

    • Glucose: Similar to blood glucose.
    • Protein: 1-1.5 g/dL.
    • Lactate dehydrogenase (LDH): Less than 50% of plasma LDH.

    Light's Criteria (Exudative versus Transudative Pleural Effusions)

    Criteria Exudative Transudative
    1. Pleural fluid protein / serum protein ≥ 0.5 < 0.5
    2. Serum protein > 3 g/dL
    3. Pleural fluid LDH / Serum LDH > 2/3rd of upper limit of normal S.LDH < 2/3rd of upper limit of normal S.LDH
    • If any one criterion is met, the effusion is classified as exudative.
    • If all three criteria are negative, the effusion is classified as transudative.

    Disadvantages of Light's Criteria

    • In patients with congestive heart failure (CCF) receiving diuretics, one-third of cases are misclassified as exudative.
    • Instead, consider using the serum pleural fluid albumin gradient (higher gradient indicates a transudative effusion).

    Occupational Exposures

    • Silicosis, coal worker's pneumoconiosis, and beryliosis.

    PET Imaging in HP

    • Chronic form (ILD/fibrosis): Diffuse opacities.

    CXR in HP

    • Diffuse opacities.
    • Reticulonodular shadows.

    CT Imaging in HP

    • Centrilobular nodules.
    • Bilateral ground glass opacities (GGO).
    • Mosaic attenuation or "head cheese" pattern (varying densities on the same CT).

    BAL Lymphocytosis in HP

    • CD4 cells greater than 2: Suggestive of sarcoidosis.
    • CD8 cells: Dominant in hypersensitivity pneumonitis.

    Treatment of HP

    • Subacute form: Responds to steroids.
    • Fibrosis: Irreversible.

    Differences Between HP and ABPA

    Feature ABPA HP
    Pathology Colonization of airway. Eosinophilic inflammation. Hypersensitivity (I + III). Migratory infiltrates. Bronchiectatic changes. Non-caseating granulomas. Lymphocytic inflammation. Hypersensitivity (IV > III).
    Radiographic Features Diffuse infiltrates. Reticulonodular opacities
    Skin test reaction to Aspergillus antigens Positive (to A. fumigatus)
    Peripheral eosinophilia Positive
    IgG Aspergillus precipitins (Type III hypersensitivity) Positive
    Serum IgE levels Significantly elevated Normal

    Note on Image Descriptions

    • The document contains radiographic images of the chest, likely CXR and CT scans.
    • These images demonstrate various patterns of lung opacities and/or nodules, suggesting potential lung conditions like hypersensitivity pneumonitis and allergic bronchopulmonary aspergillosis.
    • Written descriptions of the images and associated conditions are provided.
    • A table summarizes key differences between hypersensitivity pneumonitis and allergic bronchopulmonary aspergillosis.

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