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.

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.

Interstitial lung diseases (ILDs) represent a complex spectrum of conditions that share one  unifying truth: they are almost always serious, progressive, and life-altering. But for many patients,  the burden of disease does not fall evenly. We are increasingly learning that where you live, who  you are, and the resources available to you, can strongly influence how ILD develops and  worsens. Understanding these disparities is not about pointing fingers. Rather, it is about uncovering opportunities to deliver better, fairer care.