Connective tissue diseases (CTDs)

Intertitial lung diseases (ILDs) are present in approximately 40 % of patients with connective tissue diseases (CTD) such as Systemic Sclerosis (SSc-ILD), rheumatoid arthritis (RA-ILD), Sjögren’s syndrome, idiopathic inflammatory myositis (IIM), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD-ILD)). The occurrence of ILD is an increasing cause of death in CTD, and is now the leading cause of death in SSc. Their pathogenesis is still not well characterized because of the heterogeneities of the diseases. However, some common risk factors such as genetic abnormalities and environmental risk factors have been associated with many ILDs. Although patterns of ILD-CTD are similar to those seen in idiopathic interstitial pneumonias, a multidisciplinary evaluation is always needed to optimize diagnosis and management strategies. In the management of CTD associated ILD, early treatment is very important to stabilise lung function and improve outcomes, patients are typically given immunosuppressants to address underlying inflammation.