Abstract
The major significance of developing pulmonary complications in the setting of connective tissue disease (CTD) is now well recognized. This chapter describes the general approach toward diagnosing and treating interstitial lung disease (ILD) associated with CTD before focusing on each specific CTD in turn. The pulmonary complications, including ILD and pulmonary hypertension, are discussed for systemic sclerosis, rheumatoid arthritis, systemic lupus erythematosus, Sjögren syndrome, polymyositis/dermatomyositis, and other less common CTDs. We also discuss the relevance of acknowledging those patients with some features of CTD who do not meet the specific criteria for diagnosing any CTD. This group is now termed “idiopathic pneumonia with autoimmune features” and is the focus of future research to understand its prognostic and treatment implications. The approach to treatment for each CTD and its pulmonary complications are discussed.
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