Pulmonary arterial hypertension (PAH) is a serious complication of connective tissue disorders. Approximately 25% of patients with PAH have an underlying CTD, making CTD-PAH the second most common cause of PAH. During the session on Management of Lung Complications in CTD at APLAR 2025, Prof. Dinesh Khanna and Masataka Kuwana provided an excellent overview of PAH diagnosis and management strategies the context of CTD-ILD.

Rheumatologists typically manage systemic autoimmune rheumatic diseases (SARD) and associated interstitial lung disease (ILD) with immunosuppressants. However, evidence increasingly supports also the use of antifibrotic agents in this setting. Pulmonologists have long used antifibrotics for ILD caused by idiopathic pulmonary fibrosis (IPF) and other conditions, but rheumatologists have limited experience with these drugs.

The European Respiratory Society (ERS) and European Alliance of Associations for Rheumatology (EULAR) have published clinical practice guidelines for the evaluation and management of connective tissue diseases (CTD) associated interstitial lung disease (ILD) in two simultaneous publications in the European Respiratory Journal and Annals of Rheumatic Diseases.