Abstract
Pulmonary involvement is common in patients with underlying autoimmune and connective tissue diseases (CTDs) and can encompass a broad spectrum of disorders involving the airways, pleura, parenchyma, vascular system, bronchiectasis and nodules with significant overlap in these pathologies. Interstitial lung disease (ILD) is the most common pulmonary manifestation of systemic autoimmune rheumatic diseases and CTDs. Particular diseases, such as systemic sclerosis, rheumatoid arthritis and inflammatory myopathies, frequently associate with ILD and high-risk patients should be screened. Antibody profiling and imaging characteristics should be used to prognosticate where possible, along with regular surveillance to ensure therapies are optimised. Immunosuppressive therapies can be effective for patients with CTD-associated ILD, but difficulties arise in distinguishing between treatment failures and complications of immunosuppression.