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Pulmonary hypertension: screening and evaluation in scleroderma

York, Michaela; Farber, Harrison W.b

Current Opinion in Rheumatology: November 2011 - Volume 23 - Issue 6 - p 536–544
doi: 10.1097/BOR.0b013e32834ba6a7
Raynaud phenomenon, scleroderma, overlap syndromes and other fi brosing syndromes: Edited by John Varga

Purpose of review The review provides an update of the epidemiology, pathogenesis, risk factors, screening and treatment of pulmonary arterial hypertension in systemic sclerosis.

Recent findings Several recent studies have investigated the utility of several noninvasive screening methods and the propagation of new treatments promise the clinician better outcomes than the current median survival time of 1 year for patients with scleroderma-related pulmonary arterial hypertension.

Summary Pulmonary hypertension is a frequent cause of morbidity and mortality in patients with systemic sclerosis. This review discusses the recent changes in the classification of pulmonary hypertension, especially the significance for the rheumatologist. A high clinical suspicion should be maintained, even in early scleroderma. Despite progress in echocardiography and biomarkers, right heart catheterization remains the only test that can diagnose pulmonary hypertension and differentiate pulmonary veno-occlusive disease from pulmonary arterial hypertension. The differentiation of these causes of pulmonary hypertension in the scleroderma patient is essential because the initiation of pulmonary vasodilators in veno-occlusive disease often leads to increased mortality. The role of screening with serum biomarkers and noninvasive testing remains controversial, and in this review we discuss the controversies and new recommendations in detail.

aArthritis Center

bPulmonary Center, Boston University School of Medicine, Boston, Massachusetts, USA

Correspondence to Harrison W. Farber, Pulmonary Center, Boston University School of Medicine, 72 East Concord Street, R-304 Boston, MA 02118, USA E-mail:

© 2011 Lippincott Williams & Wilkins, Inc.