Scleroderma and pulmonary hypertension

Revista Brasileira de Reumatologia

Karen A. Fagan²  David H. Collier¹  David B. Badesch¹ 

[1] ,University of Colorado Health Sciences Center

关键词: scleroderma;    pulmonary hypertension;    dyspnea;    esclerodermia;    hipertensão pulmonar;    dispnéia;   

DOI  :  10.1590/S0482-50042003000500006

来源: SciELO

【 摘 要 】

Patients with scleroderma are at increased risk for the development of pulmonary hypertension, and the development of unexplained dyspnea or an isolated decrease in diffusing capacity should prompt evaluation. Echocardiography is often helpful in this situation, with further testing being performed as indicated. Because the prognosis of untreated pulmonary hypertension occurring in the setting of scleroderma is generally quite poor, vigilance is required on the part of physicians following this "at risk" group of patients. The past decade has seen important advances in the treatment of pulmonary arterial hypertension, including intravenous epoprostenol, oral bosentan and subcutaneously infused treprostinil. As new therapies are developed for the treatment of pulmonary arterial hypertension, it is essential that patients with scleroderma-related disease are included in clinical trials.

【 授权许可】

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