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Interstitial Lung Disease (ILD)

Intersitial lung disease is an umbrella term for conditions that diffusely affect the lung interstitium/parenchyma, and is characterised by chronic inflammation and/or progressive pulmonary fibrosis. The most common cause of it is Idiopathic Pulmonary Fibrosis, which typically affects men > 70 yrs, and smokers.

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Idiopathic pulmonary fibrosis - National Heart Lung and Blood Institute

Complications of it include:

  • Cor pulmonale

  • Type 2 respiratory failure

  • Lung cancer


N.B. Fibrosis of lung tissue causes damage to pulmonary arterioles and capillaries, resulting in the gradual development of pulmonary HTN, therefore leading to RHF and Cor Pulmonale.


Presentation

  • Dry cough

  • Progressive SOB

  • Fatigue


O/E - Fine end-inspiratory crackles, Clubbing


N.B. Fine crackles are heard during inspiration as the thickened and stiffened walls of the alveoli are moved apart by the air entering.


N.B. Clubbing is a sign of chronic hypoxia.


Investigations

  • High-resolution CT Chest - ground glass appearance or honeycombing

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“High-resolution computed tomography scan of the chest of a patient with Idiopathic Pulmonary Fibrosis (IPF)” © IPFeditor (Licensed under CC BY-SA 3.0) https://creativecommons.org/licenses/by-sa/3.0/


  • Spirometry - restrictive pattern

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Management

Conservative - Smoking cessation, Pulmonary rehabilitation


Medical - LTOT, Antifibrotic drugs (e.g. Pirfenidine), Diuretics if signs of cor pulmonale


Surgical - Lung transplant (curative)




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