Connective tissue disease–associated interstitial lung disease in adults

ILD occurs in most connective tissue diseases, generally as a consequence of abnormal immune system overactivity and immune-mediated lung damage. ILD is most often seen in systemic sclerosis, rheumatoid arthritis and idiopathic inflammatory myopathy (including polymyositis and dermatomyositis).

The most common HRCT patterns in connective tissue disease–associated interstitial lung disease (CTD–ILD) are nonspecific interstitial pneumonia pattern and organising pneumonia, although a usual interstitial pneumonia pattern predominates in rheumatoid arthritis. Overlap patterns (particularly nonspecific interstitial pneumonia/organising pneumonia overlap) are not uncommon.

If CTD–ILD is suspected, refer to a specialist to identify patients who require treatment (generally corticosteroids, with or without steroid-sparing drugs). Treatment of CTD–ILD depends on several factors including severity, disease behaviour and patient factors (eg age, comorbidities, frailty, other drugs). Smoking cessation is recommended; see Smoking cessation. For more information on diagnosis and management of CTD–ILD, see the Thoracic Society of Australia and New Zealand position statement.