Curiosity CRESTed: A PAH's Etiology

Abstract

Pulmonary arterial hypertension (PAH) is a progressive condition that may occur idiopathically or in association with connective tissue diseases such as limited cutaneous systemic sclerosis (lcSSc). We present a case of late-onset lcSSc identified during evaluation of presumed idiopathic PAH in a 75-year-old patient with longstanding Raynaud’s phenomenon. This case highlights missed opportunities for earlier diagnosis and screening. Patients with lcSSc, particularly those with declining DLCO, abnormal FVC/DLCO ratios, elevated NT-proBNP, and positive autoantibodies, are at increased risk for PAH. Screening for lcSSc in older patients with idiopathic AH, and for PAH in lcSSc, may enable earlier intervention and improve outcomes.