Synopsis
Detect interstitial lung disease linked to systemic autoimmunity, recognise rapidly progressive phenotypes and coordinate respiratory and rheumatology treatment without obscuring infection or medicine toxicity.
- Connective-tissue disease may precede ILD, appear concurrently or emerge after lung disease, so absence of a previous rheumatology diagnosis is not exclusion.
- Systemic sclerosis, rheumatoid arthritis, inflammatory myopathy, Sjögren disease, mixed connective-tissue disease and lupus produce different patterns and risks.
- Ask about Raynaud phenomenon, digital ulcers, skin tightening, inflammatory joints, sicca, dysphagia, reflux, photosensitive rash, proximal weakness and mechanic's hands.
Key red flags
Escalating cough, dyspnoea and oxygen requirement over days or weeks with new ground glass or consolidation can occur in inflammatory myopathy and requires urgent specialist assessment.
Investigation priorities
Define the systemic phenotype that gives lung findings meaning.
Management branches
ILD is found in known connective-tissue disease or autoimmune clues appear in unexplained ILD.
- Assess oxygenation and tempo, obtain complete physiology and HRCT, and document medication, infection, smoking and exposure histories that could provide alternative explanations.
- Perform a directed rheumatological examination and phenotype-led serology rather than treating a laboratory panel as a diagnosis.
Key medicines
Mycophenolate mofetilA specialist often titrates from a low oral dose towards a locally agreed divided maintenance regimen; exact dosing, formulation and off-label indication must follow rheumatology or ILD protocol and current BNF advice.
NintedanibFor licensed adult SSc-ILD or eligible progressive fibrosing ILD, specialist initiation is generally 150 mg orally twice daily with food; use interruption or 100 mg twice daily for adverse effects under the SmPC.