Pulmonary veno-occlusive disease in a patient with recently diagnosed systemic sclerosis

Nina M. van Leeuwen, Sofia Ramiro, Maarten K. Ninaber, Esther Nossent, Jeska K. de Vries-Bouwstra

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Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension in patients with systemic sclerosis that can be misclassified as pulmonary arterial hypertension. Differentiation between pulmonary veno-occlusive disease and pulmonary arterial hypertension is challenging because of the similar clinical picture. Nevertheless, discrimination is important because pulmonary veno-occlusive disease has a worse prognosis. Vasodilators including phosphodiesterase type 5 inhibitors and endothelin receptor antagonists should be started with caution and often in combination with diuretics to prevent pulmonary edema.
Original languageEnglish
Pages (from-to)NP1-NP4
JournalJournal of Scleroderma and Related Disorders
Issue number2
Early online date7 Jun 2019
Publication statusPublished - 2020

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