Connective Tissue Disease-Associated Pulmonary Arterial Hypertension

被引：15
作者：
Sung, Yon K. [1 ]
Chung, Lorinda [2 ,3 ]
机构：
[1] Stanford Univ, Sch Med, Div Pulm & Crit Care Med, Vera Moulton Wall Ctr Pulm Vasc Dis, Stanford, CA 94305 USA
[2] Stanford Univ, Sch Med, Div Rheumatol & Immunol, Stanford, CA 94305 USA
[3] VA Palo Alto Hlth Care Syst, Div Rheumatol, Palo Alto, CA 94304 USA
来源：
RHEUMATIC DISEASE CLINICS OF NORTH AMERICA | 2015年 / 41卷 / 02期
关键词：
Pulmonary hypertension; Pulmonary arterial hypertension; Connective tissue disease; Systemic sclerosis; Systemic lupus erythematosus; Mixed connective tissue disease; ANTIENDOTHELIAL CELL ANTIBODIES; SYSTEMIC-LUPUS-ERYTHEMATOSUS; IMMUNOSUPPRESSIVE THERAPY; CLINICAL CHARACTERISTICS; RISK-FACTORS; RHEUMATOID-ARTHRITIS; AUTOIMMUNE-DISEASES; ENDOTHELIAL INJURY; SCLEROSIS; PREVALENCE;
D O I：
10.1016/j.rdc.2015.01.003
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
Pulmonary arterial hypertension (PAH) is characterized by vascular remodeling of pulmonary arterioles that leads to increased pulmonary vascular resistance, right heart failure, and death. It is associated with connective tissue diseases, including systemic sclerosis, systemic lupus erythematosus, and mixed connective tissue disease. PAH is characterized by dyspnea on exertion and fatigue. Syncopal events suggest severe disease. Patients may present with signs of right heart failure. One- and 3-year survival rates are approximately 81% and 52%, respectively. Given the high prevalence and mortality, algorithms for screening are currently under investigation and will hopefully lead to earlier diagnosis and improved survival.
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收藏
页码：295 / +
页数：20
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