Abstract

Clinical Case Description: A 65-year-old male presented with progressive dyspnea for six months. His medical history was notable for diabetes mellitus and hypertension. Respiratory rate was 18/min, oxygen saturation was 88% on room air, and he was classified as World Health Organization functional class (WHO-FC) III–IV. Lung auscultation revealed bilateral “velcro-like” crackles in the middle and lower zones, while cardiac examination demonstrated an accentuated pulmonary component of the second heart sound. Physical findings included bilateral +1 pretibial edema, reduced mouth opening, sclerodactyly, and Raynaud’s phenomenon. SSc was diagnosed based on positive antinuclear and anti-topoisomerase I antibodies. Pro-BNP was 1739 pg/mL, and six-minute walk distance (6MWD) under 2 L/min oxygen was 232 m. Pulmonary function tests showed FVC 62% predicted (2620 mL), FEV₁ 69% predicted (2280 mL), FEV₁/FVC 87%, and DLCO 18% predicted. Contrast-enhanced thoracic CT excluded thromboembolism but revealed traction bronchiectasis and basal honeycombing in both lungs (Image 1). Echocardiography demonstrated left ventricular ejection fraction of 66%, estimated systolic pulmonary artery pressure of 109 mmHg, peak tricuspid regurgitation velocity of 500 cm/s, right heart dilatation, and severe tricuspid regurgitation. Right heart catheterization confirmed pre-capillary PH (Image 2). Ventilation/perfusion scintigraphy did not reveal a perfusion defect suggestive of pulmonary embolism. The patient was diagnosed with progressive pulmonary fibrosis and treated with nintedanib, sildenafil and intravenous epoprostenol were started for PH. At six months, WHO-FC improved to II, Pro-BNP decreased to 138 pg/mL, and 6MWD increased to 345 m. However, he died at month eight due to infection-related deterioration.