Pulmonary arterial hypertension related to systemic sclerosis in 2008

被引：2

作者：

Agard, C. ^{[1
,2
]}

Haloun, A. ^{[3
]}

Hamidou, M. -A. ^{[1
]}

机构：

[1] CHU Hotel Dieu, Serv Med Interne, F-44035 Nantes 01, France

[2] Univ Nantes, Fac Med, UMR 915, Inst Thorax, F-44093 Nantes 01, France

[3] Hop Guillaume & Rene Laennec, Inst Thorax, Serv Pneumol, F-44035 Nantes 01, France

来源：

JOURNAL DES MALADIES VASCULAIRES | 2009年 / 34卷 / 01期

关键词：

Pulmonary arterial hypertension; Systemic sclerosis; Endothelin-1; INHALED ILOPROST; OPEN-LABEL; RISK-FACTORS; BOSENTAN; THERAPY; SITAXSENTAN; PREVALENCE; ENDOTHELIN-1; TREPROSTINIL; SILDENAFIL;

D O I：

10.1016/j.jmv.2008.10.008

中图分类号：

R6 [外科学];

学科分类号：

1002 ; 100210 ;

摘要：

Systemic sclerosis-related pulmonary arterial hypertension (PAH) is a severe disease affecting about 1000 patients in France. In 2008, all scleroderma patients are screened for PAH by a yearly cardiac Doppler ultrasonography. The pathogenesis of systemic sclerosis-related PAH is poorly known but it seems that besides common arteriolar remodeling (media hypertrophy, intimal thickening, endothelial proliferation), venular lesions suggesting obstructive venous disease and inflammatory lesions may be also be involved. Prostacyclin and analogues, phosphodiesterase-5 inhibitors (sildenafil) and endothelin-1 receptor antagonists are proposed as specific treatments for systemic sclerosis-related PAH. Unlike bosentan, which is non-selective, inhibiting both ETA and ETB receptors, sodium sitaxentan is highly selective for ETA receptors; this could favor pulmonary vasodilation. (c) 2008 Elsevier Masson SAS. All rights reserved.

引用

页码：7 / 15

页数：9

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