Adults
Interstitial lung disease in autoimmune disease
New or worsening cough or dyspnea in someone with SSc, myositis, RA, Sjögren's or MCTD, or a falling FVC or DLCO.
What to look for
- Exertional dyspnea, dry cough or inspiratory crackles in a patient with a systemic autoimmune rheumatic disease
- Highest risk: systemic sclerosis, myositis (especially antisynthetase and anti-MDA5), MCTD; also RA and Sjögren's with risk factors
- A falling FVC (about 10% or more, or 5–10% with worsening symptoms or imaging) or DLCO (about 15% or more)
What to do
- Get PFTs with spirometry, lung volumes and DLCO, plus HRCT chest. ACR/CHEST 2023 conditionally advises against chest X-ray or 6-minute walk alone for screening.
- Refer to rheumatology and pulmonology. Anti-MDA5 with ILD, or rapid decline, is emergent.
- ERS/EULAR 2025 strongly recommends HRCT screening for everyone with SSc or MCTD, and for myositis with risk factors such as antisynthetase or anti-MDA5 antibodies or amyopathic dermatomyositis.
About this urgency level
Early specialist assessment changes outcomes. Send the usual workup with the referral, but don't hold the referral while waiting for results.
Timeframes are typical targets. Local pathways, capacity and clinical judgment come first.