Diffuse Parenchymal Lung Disease Quiz
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Questions and Answers

What is diffuse parenchymal lung disease?

  • A group of conditions affecting the pulmonary parenchyma and/or alveolar lumen (correct)
  • A type of heart disease
  • A specific type of lung disease
  • A type of lung infection

    • What is a typical symptom of diffuse parenchymal lung disease?

  • Coughing up blood
  • Dry, persistent and distressing cough (correct)
  • Sudden loss of breath
  • Pain in the chest

    • What is a common finding on examination in diffuse parenchymal lung disease?

  • Abnormal heart sounds
  • Wheezing
  • Sroscope sounds
  • Crackles (correct)

    • What is a radiological feature of advanced diffuse parenchymal lung disease?

    <p>Small lung volumes with reticulonodular shadowing</p> Signup and view all the answers

    What is a typical finding on pulmonary function testing in diffuse parenchymal lung disease?

    <p>Restrictive ventilatory defect</p> Signup and view all the answers

    What is a condition that can mimic diffuse parenchymal lung disease?

    <p>Viral pneumonia</p> Signup and view all the answers

    What is a type of diffuse parenchymal lung disease?

    <p>Organizing pneumonia</p> Signup and view all the answers

    What is a complication of advanced diffuse parenchymal lung disease?

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

    What is the most common laboratory investigation for sarcoidosis?

    <p>Serum angiotensin-converting enzyme</p> Signup and view all the answers

    Which of the following is NOT a cause of diffuse parenchymal lung disease?

    <p>Pulmonary oedema</p> Signup and view all the answers

    What is the primary purpose of bronchoalveolar lavage?

    <p>To exclude infection</p> Signup and view all the answers

    What is the definition of idiopathic pulmonary fibrosis?

    <p>A progressive fibrosing interstitial pneumonia of unknown cause</p> Signup and view all the answers

    What can be found in occupational fibrosing lung disease through a biopsy?

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

    What is the purpose of a liver biopsy in sarcoidosis?

    <p>To rule out other causes of liver disease</p> Signup and view all the answers

    What is the association of elevated Ca2+ levels?

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

    Which of the following is a connective tissue disease associated with diffuse parenchymal lung disease?

    <p>Systemic sclerosis</p> Signup and view all the answers

    When is bronchoscopy usually indicated?

    <p>When there is consideration of differential diagnoses of infection or malignant process</p> Signup and view all the answers

    What is the primary concern in cases where lung biopsy is being considered?

    <p>Diagnostic uncertainty or atypical features</p> Signup and view all the answers

    What is the primary goal of pirfenidone and nintedanib treatments?

    <p>To reduce the rate of decline in lung function</p> Signup and view all the answers

    What is the definition of pneumoconiosis?

    <p>A permanent alteration of lung structure due to mineral dust inhalation</p> Signup and view all the answers

    What is the most important type of pneumoconiosis?

    <p>All of the above are equally important</p> Signup and view all the answers

    What is the result of prolonged inhalation of coal dust?

    <p>Fibrotic reaction resulting in scattered discrete fibrotic lesions</p> Signup and view all the answers

    What is the classification of coal worker’s pneumoconiosis based on?

    <p>Size and extent of radiographic nodularity</p> Signup and view all the answers

    What is simple coal worker’s pneumoconiosis (SCWP) characterized by?

    <p>Small radiographic nodules in an otherwise asymptomatic individual</p> Signup and view all the answers

    What is an important step in excluding fibrosing diseases caused by occupational exposure, medication, or connective tissue diseases?

    <p>Careful history, examination, and investigation</p> Signup and view all the answers

    What is the typical age of onset for IPF?

    <p>After 50 years</p> Signup and view all the answers

    What is a common symptom of IPF?

    <p>Progressive breathlessness</p> Signup and view all the answers

    What is a characteristic clinical finding in IPF?

    <p>Finger clubbing</p> Signup and view all the answers

    What is the typical pattern of reticular shadowing on chest X-ray in IPF?

    <p>Bilateral lower lobe and subpleural</p> Signup and view all the answers

    What is the high positive predictive value of HRCT for the diagnosis of IPF based on?

    <p>Patchy, peripheral, and basal reticular pattern</p> Signup and view all the answers

    What is a characteristic feature of advanced IPF on HRCT?

    <p>Honeycombing cysts and traction bronchiectasis</p> Signup and view all the answers

    What is the significance of pleural plaques on HRCT?

    <p>Suggestive of asbestosis</p> Signup and view all the answers

    What is the characteristic of conglomerate masses in Progressive Massive Fibrosis (PMF)?

    <p>More than 1 cm in diameter</p> Signup and view all the answers

    What is the common symptom associated with the development of PMF?

    <p>Cough, sputum that may be black, and breathlessness</p> Signup and view all the answers

    What is the treatment goal for PMF?

    <p>To remove or reduce coal dust exposure</p> Signup and view all the answers

    What is the condition characterized by the coexistence of rheumatoid arthritis and rounded fibrotic nodules?

    <p>Caplan syndrome</p> Signup and view all the answers

    What is the usual form of silica inhaled by workers that leads to silicosis?

    <p>Crystalline silica, usually in the form of quartz</p> Signup and view all the answers

    What is the typical duration of silica exposure associated with classic or sub-acute silicosis?

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

    What is the increased risk associated with silicosis?

    <p>Tuberculosis, non-tuberculous mycobacterial infection, lung cancer, and COPD</p> Signup and view all the answers

    What is the primary goal of treatment for silicosis?

    <p>To cure the disease with medication</p> Signup and view all the answers

    Study Notes

    Diffuse Parenchymal Lung Disease

    • Heterogeneous group of conditions affecting the pulmonary parenchyma (interstitium) and/or alveolar lumen
    • Shares clinical, physiological, and radiographic similarities

    Clinical Presentation

    • Cough: usually dry, persistent, and distressing
    • Breathlessness: usually slowly progressive, insidious onset, acute in some cases
    • Examination findings:
      • Crackles: typically bilateral and basal
      • Clubbing: common in idiopathic pulmonary fibrosis, also seen in other types (e.g., asbestosis)
      • Central cyanosis and signs of right heart failure in advanced disease (corpulmonale)

    Radiology

    • Chest X-ray: typically small lung volumes with reticulonodular shadowing, but may be normal in early or limited disease
    • High-resolution computed tomography: combinations of ground glass changes, reticulonodular shadowing, honeycomb cysts, and traction bronchiectasis, depending on stage of disease

    PFT

    • Typically restrictive ventilatory defect with reduced lung volumes and impaired gas transfer
    • Exercise tests assess exercise tolerance and exercise-related fall in SaO2

    Acute Interstitial Pneumonia

    • Organizing pneumonia
    • Bronchopneumonia
    • Pulmonary oedema

    Conditions that Mimic Diffuse Parenchymal Lung Disease

    • Infection:
      • Viral pneumonia
      • Tuberculosis
      • Pneumocystis jirovecii
      • Parasite (e.g., lariasis)
      • Fungal infection
    • Malignancy:
      • Leukemia and lymphoma
      • Multiple metastases
      • Lymphangitic carcinomatosis
      • Bronchoalveolar carcinoma
    • Pulmonary oedema
    • Aspiration pneumonia

    Laboratory Investigations

    • Full blood count: lymphopenia in sarcoidosis, eosinophilia in pulmonary eosinophilias and drug reactions, neutrophilia in hypersensitivity pneumonitis
    • Erythrocyte sedimentation rate and C-reactive protein: non-specifically raised
    • Ca2+: may be elevated in sarcoidosis
    • Serum angiotensin-converting enzyme: non-specific indicator of disease activity in sarcoidosis
    • Lactate dehydrogenase: may be elevated in active alveolitis
    • Autoimmune screen: anti-cyclic citrullinated peptide (anti-CCP) and other autoantibodies may suggest connective tissue disease

    Radiology and PFT

    • Chest X-ray and high-resolution computed tomography
    • PFT: typically restrictive ventilatory defect with reduced lung volumes and impaired gas transfer

    Bronchoscopy and Biopsy

    • Bronchoscope: differential cell counts may point to sarcoidosis, drug-induced pneumonitis, pulmonary eosinophilias, hypersensitivity pneumonitis, or cryptogenic organizing pneumonia
    • Transbronchial biopsy: useful in sarcoidosis and differential of malignancy or infection
    • Video-assisted thoracoscopic lung biopsy (VATS): allows pathological classification, presence of asbestos bodies may suggest asbestosis, silica in occupational fibrosing lung disease

    Idiopathic Pulmonary Fibrosis

    • Defined as a progressive fibrosing interstitial pneumonia of unknown cause, occurring in adults and associated with the histological or radiological pattern of UIP
    • Important differentials include fibrosing diseases caused by occupational exposure, medication, or connective tissue diseases
    • Histological features suggest repeated episodes of focal damage to the alveolar epithelium
    • Familial cases are rare, but genetic factors that control the inflammatory and fibrotic response are likely to be important
    • Strong association with cigarette smoking

    Clinical Features of IPF

    • Presents in older adults, uncommon before the age of 50 years
    • May present as an incidental finding on CT scan or with progressive breathlessness and a non-productive cough
    • Constitutional symptoms are unusual
    • Clinical findings include finger clubbing and bi-basal fine late inspiratory crackles

    Investigations for IPF

    • Full blood count, erythrocyte sedimentation rate, and C-reactive protein
    • Chest X-ray and high-resolution computed tomography
    • Bronchoscope: lymphocytosis may suggest chronic hypersensitivity pneumonitis
    • Surgical lung biopsy: should be sought if necessary

    Treatment of IPF

    • Pirfenidone (an antifibrotic agent) or nintedanib (a tyrosine kinase inhibitor) may be offered
    • Both agents have been shown to reduce the rate of decline in lung function
    • Neither drug improves cough or breathlessness, and treatment should be discontinued if lung function declines by more than 10% over the first year of treatment
    • Medication to control gastro-oesophageal reflux may improve the cough
    • Stop smoking, oxygen therapy, and lung transplantation may be considered

    Prognosis of IPF

    • Median survival of 3 years is widely quoted
    • Rate of disease progression varies considerably

    Pneumoconiosis

    • Permanent alteration of lung structure due to the inhalation of mineral dust and the tissue reactions of the lung to its presence
    • Important pneumoconiosis include coal worker's pneumoconiosis, silicosis, and asbestosis

    Coal Worker's Pneumoconiosis (CWP)

    • Follows prolonged inhalation of coal dust, resulting in a fibrotic reaction and the appearance of scattered discrete fibrotic lesions
    • Classification is based on the size and extent of radiographic nodularity
    • Simple coal worker's pneumoconiosis (SCWP) refers to the appearance of small radiographic nodules in an otherwise asymptomatic individual
    • Progressive massive fibrosis (PMF) refers to the formation of one or more conglomerate masses more than 1 cm in diameter
    • Treatment involves removing or reducing coal dust exposure, and is otherwise supportive treatment
    • PMF may progress, even after coal dust exposure ceases, and in extreme cases leads to respiratory failure and right ventricular failure
    • CWP is not associated with increased risk of lung cancer
    • Caplan syndrome describes the coexistence of rheumatoid arthritis and rounded fibrotic nodules 0.5-5 cm in diameter

    Silicosis

    • Results from the inhalation of crystalline silica, usually in the form of quartz, by workers cutting, grinding, and polishing stone
    • Patients experience dry cough and breathlessness with the sensation of chest restriction
    • Classic or sub-acute silicosis is most common and usually manifests after 10-20 years of continuous silica exposure
    • Accelerated silicosis is associated with a much shorter duration of dust exposure (typically 5-10 years), may present as early as after 1 year of exposure, and follows a more aggressive course
    • Intense exposure to very fine crystalline silica dust can cause a more acute disease: silicoproteinosis
    • Chronic silicosis can develop after 10-30 years of continuous exposure to lower concentrations of silica dust
    • Treatment, aside from stopping exposure, is mainly supportive
    • Increased risk of TB (silicotuberculosis), non-tuberculous mycobacterial infection, lung cancer, and COPD

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    Test your knowledge on diffuse parenchymal lung disease, a heterogeneous group of conditions affecting the pulmonary parenchyma and/or alveolar lumen. Learn about its clinical presentation, symptoms and examination findings.

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