Interstitial Lung Disease
Interstitial lung disease (ILD) is a heterogeneous group of disorders causing inflammation and/or fibrosis of the lung parenchyma; diagnosis depends on exposure and connective-tissue assessment, HRCT, physiology and specialist multidisciplinary review, while treatment is cause-specific and progression-focused.
Definition
Interstitial lung disease is a heterogeneous group of disorders affecting the lung interstitium and adjacent parenchyma, causing inflammation, fibrosis or both, with variable restriction, impaired gas transfer and exertional hypoxaemia.
First principles
ILD is a family of diseases, not a single diagnosis
ILD describes disorders affecting the lung interstitium and adjacent alveolar structures. Causes include connective-tissue disease, hypersensitivity or occupational exposure, drugs, infection, sarcoidosis and idiopathic interstitial pneumonias. The same symptom pattern can therefore represent inflammation that may respond to treatment, established fibrosis, or both; do not label a patient with idiopathic pulmonary fibrosis until relevant alternative causes have been assessed.
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