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Fibrotic lung disease

Background knowledge 🧠

Definition

Fibrotic lung diseases are a group of disorders characterised by fibrosis and/or inflammation of the pulmonary interstitium.

Pathophysiology

  1. Chronic inflammation leads to alveolar wall fibrosis and architectural distortion.
  2. Results in reduced gas exchange and lung compliance.

Aetiology

  • 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.

Clinical Features πŸŒ‘️

Clinical Features

  • Dyspnoea on exertion.
  • Non-productive cough.
  • Clubbing of fingers.
  • Bilateral inspiratory ‘velcro’ crackles on auscultation.
  • Features of specific aetiology (e.g. erythema nodosum in sarcoidosis).

Investigations πŸ§ͺ

Tests

  • 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 πŸ₯Ό

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.

Key Points

  • Fibrotic lung disease has multiple aetiologies which lead to chronic inflammation and fibrosis of the lung.
  • Investigation is based on characteristic features found on imagingΒ and pulmonary function testing.
  • Bilateral inspiratory ‘velcro’ crackles on auscultation, honeycombing and ground-glass opacities on imaging as well as a restrictive lung function pattern are characteristic of fibrotic lung disease.
  • Management varies but includes lifestyle changes, antifibrotic therapy (IPF) and immunosuppression (autoimmune).

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