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🌟 Interstitial lung disease

 🎯 Pathology

  • ILD is a name given to a group of lung conditions that fall under restrictive lung diseases

  • restrictive lung diseases limit how much the lungs can expand —> impaired lung compliance and gas exchange

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⚡️ Types of restrictive lung diseases

  • pleural diseases (e.g. pleural effusion, asbestosis)

  • neuromuscular disorders (e.g. myasthenia gravis)

  • thoracic wall disorders (e.g. kyphoscoliosis)

  • interstitial lung diseases (ILD)

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💡 Causes

  • Exposure-related

    • due to long-term exposure to occupational or environmental agents e.g. silica, asbestos, beryllium and coal mine dust

  • Systemic conditions

    • can be associated with autoimmune and connective tissue diseases such as rheumatoid arthritis, sarcoidosis, systemic lupus erythematosus (SLE) and systemic sclerosis

  • Drugs

    • more than 350 have been identified

    • typical ones = amiodarone, bleomycin, busulfan, sulfasalazine

  • Idiopathic pulmonary fibrosis

    • most common type of ILD

    • most cases are sporadic but there may be a genetic component

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🩺 Clinical features

  • history

    • triad = exertional dyspnoea, dry cough, clubbing

  • examination

    • clubbing

    • bilateral fine-end inspiratory crackles

    • features of systemic conditions

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🧐 Which part of the lung is affected?

  • causes of upper zone fibrosis ("CHARTS")

    • Coal worker’s pneumoconiosis

    • Histiocytosis / Hypersensitivity pneumonitis

    • Ankylosing spondylitis

    • Radiation (e.g. for breast cancer)

    • Tuberculosis

    • Sarcoidosis / silicosis

  • causes of lower zone fibrosis

    • IPF

    • Drugs

    • Connective tissue diseases

    • Asbestosis

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🕵️‍♂️ Investigations

  • bloods

    • full blood count —> anaemia of chronic disease in inflammation

    • CRP/ESR —> high in inflammation

    • autoimmune screen to look for autoantibodies

  • lung function tests

    • spirometry = restrictive pattern (increased FEV1/FVC ratio)

    • transfer factor = reduced

  • imaging

    • initial = chest x-ray —> fine reticular opacities

    • best = high-resolution CT scan —> honeycombing and traction bronchiectasis

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💆‍♂️ Management

  • conservative

    • avoid underlying causes e.g. drugs

    • pulmonary rehabilitation

    • vaccinations —> annual influenza vaccine or the one-off pneumococcal vaccine

  • medical

    • treat the underlying cause

    • supplementary oxygen

    • idiopathic pulmonary fibrosis —> pirfenidone or nintedanib (to help slow the progression of the disease)

    • sarcoidosis —> corticosteroids

  • surgical

    • lung transplantation

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