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.
van Leeuwen, N. M., Ramiro, S., Ninaber, M. K., Nossent, E., & de Vries-Bouwstra, J. K. (2019). Pulmonary veno-occlusive disease in a patient with recently diagnosed systemic sclerosis. Journal of Scleroderma and Related Disorders. https://doi.org/10.1177/2397198319852194