Connective Tissue Related Interstitial Lung Disease

Purpose of ReviewInterstitial lung disease (ILD) is a common complication of the connective tissue diseases (CTD) and results in significant morbidity and mortality. This review will focus on recent literature pertaining to the epidemiology, clinical presentation, diagnosis, and treatment of CTD-ILD.Recent FindingsSubclinical ILD can be found in the majority of patients with CTD. Clinically significant ILD is most commonly seen in scleroderma followed by polymyositis/dermatomyositis and rheumatoid arthritis, although it can occur in all of the CTDs. Nonspecific interstitial pneumonia is the most common radiographic and histologic pattern, although usual interstitial pneumonia occurs more frequently in rheumatoid arthritis. Pathogenesis is likely related to a combination of autoimmunity/inflammation, disordered fibrogenesis, and vascular injury. Treatment strategies are evolving to target all three of these pathways.SummaryAlthough further research into treatment strategies is needed, the clinician should be aware of the risk factors and clinical presentation of ILD in the various CTDs in order to identify patients who should be screened and/or have modifications in treatment strategies in order to mitigate the morbidity and mortality associated with CTD-ILD.