Connective Tissue Disease-Related Interstitial Lung Disease: Prevalence, Patterns, Predictors, Prognosis, and Treatment

Abstract

Interstitial lung disease is a common pulmonary manifestation of connective tissue diseases and results in significant morbidity and mortality. Although all connective tissue diseases are linked by underlying autoimmunity, there is significant variability in the presentation and clinical course of interstitial lung disease associated with the different types of connective tissue diseases. In this paper, we review the prevalence, patterns, predictors, and prognosis of interstitial lung disease secondary to the most common forms of connective tissue diseases. We have also highlighted the available evidence regarding treatment options for interstitial lung disease due to different connective tissue diseases.

Keywords: Connective tissue disease; Interstitial lung disease; Myositis; Rheumatoid arthritis; Sjögren’s syndrome; Systemic sclerosis.