Bridging knowledge gaps: A case report of pulmonary Veno-occlusive disease in systemic sclerosis

Pulmonary Veno-Occlusive Disease (PVOD) is a rare cause of pulmonary hypertension (PH) in patients with systemic sclerosis (SSc), posing significant diagnostic and therapeutic challenges. Here, we present a case of a patient with systemic sclerosis who presented with symptoms of progressive dyspnea for 1 year. Her chest radiogram was normal, and echocardiography was suggestive of severe PH. She was started on a combination of Ambrisentan and Tadalafil. However, she developed pulmonary edema after the initiation of pulmonary vasodilators. Diagnostic workup, including imaging studies, with clinical and radiological worsening with pulmonary vasodilators, confirmed PVOD in the setting of SSc. The patient gradually improved with diuretic therapy. This case emphasizes the importance of early clinical suspicion and multidisciplinary discussion in managing rare pulmonary complications associated with systemic sclerosis. Additionally, it highlights the urgent need for further research to delineate the underlying pathophysiological mechanisms and optimize therapeutic strategies for this challenging clinical entity.