Interstitial Lung Disease

• What does interstitial lung disease include?

  Conditions that cause inflammation and fibrosis of the lung parenchyma.
• What is fibrosis in the context of interstitial lung disease?

  Replacement of elastic and functional lung tissue with non-functional scar tissue.
• What are the conditions covered under interstitial lung disease?

  • Idiopathic pulmonary fibrosis
  • Secondary pulmonary fibrosis
  • Hypersensitivity pneumonitis
  • Cryptogenic organising pneumonia
  • Asbestosis
• What are the key presenting features of interstitial lung disease?

  Shortness of breath on exertion, dry cough, and fatigue.
• What typical findings are associated with idiopathic pulmonary fibrosis during examination?

  Bibasal fine end-inspiratory crackles and finger clubbing.
• Why are patients with idiopathic pulmonary fibrosis considered ideal for OSCEs?

  They are stable and have good signs.
• What is involved in the diagnosis of interstitial lung disease?

  • Clinical features
  • High-resolution CT scan (HRCT) showing a "ground glass" appearance
  • Spirometry
  • May be normal or show a restrictive pattern
  • FEV1 and FVC are equally reduced
  • FEV1:FVC ratio greater than 70%
  • Other investigations: lung biopsy, bronchoalveolar lavage
• What does spirometry show in interstitial lung disease?

  It may be normal or show a restrictive pattern with equally reduced FEV1 and FVC.
• What is the prognosis for interstitial lung disease?

  Generally poor with limited management options.
• What are the general management options for interstitial lung disease?

  • Remove or treat the underlying cause
  • Home oxygen for hypoxia
  • Stop smoking
  • Physiotherapy and pulmonary rehabilitation
  • Pneumococcal and flu vaccine
  • Advanced care planning and palliative care
  • Lung transplant (with careful consideration of risks and benefits)
• What characterizes idiopathic pulmonary fibrosis?

  Progressive pulmonary fibrosis with no apparent cause.
• How does idiopathic pulmonary fibrosis typically present?

  With insidious onset of shortness of breath and dry cough over more than 3 months.
• What is the typical age group affected by idiopathic pulmonary fibrosis?

  Adults over 50 years old.
• What is the life expectancy from diagnosis for idiopathic pulmonary fibrosis?

  2-5 years.
• What medications are licensed to slow the progression of idiopathic pulmonary fibrosis?

  • Pirfenidone: reduces fibrosis and inflammation through various mechanisms
  • Nintedanib: reduces fibrosis and inflammation by inhibiting tyrosine kinase
• Which drug is known to cause grey/blue skin as a side effect?

  Amiodarone.
• What conditions can lead to secondary pulmonary fibrosis?

  Alpha-1 antitrypsin deficiency, rheumatoid arthritis, SLE, systemic sclerosis, and sarcoidosis.
• What is hypersensitivity pneumonitis also known as?

  Extrinsic allergic alveolitis.
• What type of hypersensitivity reaction is involved in hypersensitivity pneumonitis?

  Type III and type IV hypersensitivity reaction.
• What is the role of bronchoalveolar lavage in diagnosing hypersensitivity pneumonitis?

  It gathers cells for analysis to suggest the presence of the condition.
• What does lymphocytosis indicate in the context of hypersensitivity pneumonitis?

  It is suggestive of hypersensitivity pneumonitis.
• What is the management for hypersensitivity pneumonitis?

  • Remove the allergen
  • Oxygen where necessary
  • Steroids
• What is an example of a specific cause of hypersensitivity pneumonitis related to bird droppings?

  Bird-fancier’s lung.
• What is farmer’s lung associated with?

  Reaction to mouldy spores in hay.
• What is mushroom worker’s lung associated with?

  Reaction to specific mushroom antigens.
• What is malt worker’s lung associated with?

  Reaction to mould on barley.
• What is cryptogenic organising pneumonia previously known as?

  Bronchiolitis obliterans organising pneumonia.
• What does cryptogenic organising pneumonia involve?

  A focal area of inflammation of the lung tissue.
• What can trigger cryptogenic organising pneumonia?

  Infection, inflammatory disorders, medications, radiation, environmental toxins, or allergens.
• What are the common symptoms of cryptogenic organising pneumonia?

  Shortness of breath, cough, fever, and lethargy.
• What are the chest x-ray findings in cryptogenic organising pneumonia?

  Focal consolidation similar to pneumonia.
• What is the definitive investigation for cryptogenic organising pneumonia?

  Lung biopsy.
• Why is the diagnosis of cryptogenic organising pneumonia often delayed?

  Due to similarities to infectious pneumonia.
• What is the treatment for cryptogenic organising pneumonia?

  • Systemic corticosteroids
• What does asbestosis refer to?

  Lung fibrosis related to asbestos exposure.
• What are the effects of asbestos exposure?

  Lung fibrosis, pleural thickening, and cancer.
• How long does it typically take for the effects of asbestos exposure to develop?

  Several decades.
• What are the potential health problems caused by asbestos inhalation?

  Lung fibrosis, pleural thickening, pleural plaques, adenocarcinoma, and mesothelioma.
• What must be done for deceased patients with occupational asbestos exposure?

  They must be referred to the coroner.
• What is the significance of compensation for patients with asbestos-related health conditions?

  Patients may be eligible for compensation if they develop asbestos-related health conditions.