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Fibrotic lung disease

Definition
  • Group of disorders characterised by fibrosis and/or inflammation of the pulmonary interstitium.
Etiology
  • Idiopathic: e.g., Idiopathic Pulmonary Fibrosis (IPF).
  • Occupational: e.g., Asbestosis, Silicosis.
  • Drug-induced: e.g., Amiodarone, Methotrexate.
  • Autoimmune: e.g., Rheumatoid arthritis, Systemic sclerosis, Sarcoidosis.
  • Other: Radiation pneumonitis, hypersensitivity pneumonitis.
Pathophysiology
  • Chronic inflammation leads to alveolar wall fibrosis and architectural distortion.
  • Results in reduced gas exchange and lung compliance.
Clinical Presentation
  • Dyspnoea on exertion.
  • Non-productive cough.
  • Clubbing of fingers.
  • Bilateral inspiratory ‘velcro’ crackles on auscultation.
Investigations
  • Chest X-ray: Reticulonodular shadowing, often basilar predominant.
  • HRCT: Ground-glass opacities, honeycombing, and reticulation.
  • Pulmonary function tests: Restrictive pattern with reduced diffusing capacity for carbon monoxide (DLCO).
  • Bronchoalveolar lavage or lung biopsy in selected cases.
Management
  • General: Smoking cessation, vaccination (influenza, pneumococcal), and pulmonary rehabilitation.
  • Idiopathic Pulmonary Fibrosis: Antifibrotic agents like pirfenidone and nintedanib.
  • Autoimmune: Immunosuppression e.g., corticosteroids, cyclophosphamide.
  • Oxygen therapy for those with hypoxemia.
  • Lung transplantation: Considered in severe, progressive disease.
Complications
  • Respiratory failure.
  • Cor pulmonale.
  • Increased risk of lung cancer.
  • Exacerbations leading to rapid progression.
Prognosis
  • Variable depending on the underlying cause; IPF has a poor prognosis with median survival of 2-5 years post-diagnosis.

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