Interstitial lung disease and connective tissue diseases

Lung involvement in rheumatologic diseases has a broad spectrum of clinical and radiological presentations, from acute inflammatory to chronic, fibrosing predominance. For prognostic and therapeutic considerations a detailled work-up and optimally multidisciplinary evalution is needed, also to rule out other reasons for pulmonary deterioration such as infection (under immunosuppressive treatment) or pulmonary hypertension. For treatment guidance, several aspects need to be taken into consideration, such as disease severity, disease activity, clinical and radiological presentation, prognostic markers and comorbidities. Immunosuppressive treatment differs based on the type of rheumatologic diagnosis, although evidence for optimal therapy is rare in ILD associated with rheumatologic diseases.