Pulmonary arterial hypertension: the most devastating vascular complication of systemic sclerosis

被引:58
作者
McLaughlin, V. [1 ]
Humbert, M. [2 ]
Coghlan, G. [3 ]
Nash, P. [4 ]
Steen, V. [5 ]
机构
[1] Univ Michigan, Dept Internal Med, Ann Arbor, MI 48109 USA
[2] Hop Antoine Beclere, Dept Resp Med, Clamart, France
[3] Royal Free & Univ Coll Med Sch, Cardiol Unit, London WC1E 6BT, England
[4] Univ Queensland, Sunshine Coast Queensland Dept Med, Rheumatol Res Unit, Brisbane, Qld 4072, Australia
[5] Georgetown Univ, Dept Med, Washington, DC USA
关键词
Pulmonary arterial hypertension; Systemic sclerosis; Vasculopathy; CONTINUOUS INTRAVENOUS EPOPROSTENOL; RECEPTOR ANTAGONIST BOSENTAN; BRAIN NATRIURETIC PEPTIDE; CONNECTIVE-TISSUE DISEASE; PROSTACYCLIN ANALOG; INHALED ILOPROST; THERAPY; HEMODYNAMICS; TREPROSTINIL; SILDENAFIL;
D O I
10.1093/rheumatology/kep107
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Pulmonary arterial hypertension (PAH) is a devastating vascular complication of a number of CTDs. In patients with SSc, PAH has a dramatic impact on prognosis and survival and is the single most common cause of disease-related death. Yearly echocardiographic screening for PAH is recommended in patients with SSc. It suspected, confirmation of PAH diagnosis by right heart catheterization is necessary. Treatment goals for patients with PAH associated with SSc (PAH-SSc) aim to slow disease progression and improve quality of life. Some measures used to gauge the effect of treatment in patients with PAH-SSc remain to be fully validated; the 6-min walk distance, for example, is a simple and reproducible means of assessing exercise capacity, but there exists a need to understand what constitutes a clinically relevant change in this specific patient population. Currently, pharmacological intervention in PAH-SSc may target one or more of three pathophysiological pathways in PAR The prostacyclin analogue epoprostenol has been shown to improve exercise capacity and haemodynamics in PAH-SSc patients and similar data are available from smaller studies on trepostinil and iloprost. The dual endothelin receptor antagonist bosentan has been shown to improve exercise capacity and haemodynamics in PAH-SSc, and similar data have been obtained in small numbers of patients treated with the endothelin receptor A antagonists sitaxsentan and ambrisentan. Impaired production of nitric oxide may be addressed by inhibiting phosphodiesterase type-5 with sildenafil or possibly tadalafil. Combinations of multiple targeted therapies may be beneficial to this patient population.
引用
收藏
页码:25 / 31
页数:7
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