C63-03 Successful Long-Term Pharmacotherapy in Pulmonary Capillary Hemangiomatosis With Resolution of Ground-Glass Opacities
Abstract
Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis (PCH) are rare subtypes of pulmonary arterial hypertension characterized by venous remodeling and capillary proliferation. Prognosis is typically poor, effective pharmacotherapies are not established, and vasodilators may precipitate pulmonary edema. Histological confirmation is often avoided because of procedural risk, so diagnosis relies on clinical and radiologic features. We report a case of systemic-sclerosis-associated PCH in which medical therapy resulted in disappearance of characteristic bat-wing ground-glass opacities (GGO) and long-term hemodynamic stability.
A 48-year-old woman with intermittent follow-up for inflammatory arthritis developed progressive exertional dyspnea. Evaluation revealed systemic sclerosis complicated by pulmonary hypertension. Chest computed tomography demonstrated patchy bat-wing GGO, interlobular septal thickening, and pleural and pericardial effusions (Panels A and B). Echocardiography estimated a pulmonary artery systolic pressure of 75 mmHg. Laboratory tests showed anemia (Hb 10.3 g/dL), mild hepatic and renal dysfunction, markedly increased D-dimer (10.78 μg/mL) and NT-proBNP (1957 pg/mL), and a high-titer antinuclear antibody (1:1280) with negative anti-Scl-70, anticentromere, and anti-RNA polymerase III antibodies. Ventilation-perfusion scanning showed no mismatched defects. Pulmonary function testing revealed a restrictive pattern (VC 1.73 L, %VC 56.3%) and…
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