Early use of an endothelin receptor antagonist in a patient with scleroderma-spectrum connective tissue disease–associated PAH
19 Sep 2024
byDr. Iris Yan-Ki Tang, Specialist in Rheumatology, Department of Medicine, The University of Hong Kong, Hong Kong
A 39-year-old female hospital porter presented with dyspnoea and Raynaud’s phenomenon in 2010, with no evidence of sclerodactyly. She tested positive for anti-ribonucleoprotein (anti-RNP) and anti–Scl-70 autoantibodies. High-resolution CT (HRCT) of the chest revealed bilateral ground-glass opacities, suggesting interstitial thickening or fibrosis. Echocardiography showed an elevated estimated right ventricular systolic pressure (RVSP) of 74 mm Hg. These findings, along with other defining features, supported a diagnosis of mixed connective tissue disease (MCTD), complicated by interstitial lung disease (ILD) and possibly pulmonary arterial hypertension (PAH).1
