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摘要:
Pulmonary arterial hypertension(PAH)represents a devastating vascular complication of systemic sclerosis(SSc)and is found in 10%-15%of cases carrying a severe prognosis.PAH has a dramatic impact on the clinical course and overall survival,being the single most common cause of death in patients with this entity.The clinical course and aggressive progression of PAH has led clinicians to perform annual screening for it,since early detection and diagnosis are the cornerstone of a prompt therapeutic intervention.The diagnosis of PAH can be challenging to clinicians,particularly in its early stages,since in the context of SSc,the multiple causes of dyspnea need to be assessed.Doppler echocardiography represents the best initial screening tool,however,right heart catheterization remains the gold standard and definitive diagnostic means.Remarkable advances have been achieved in elucidating the pathogenesis of PAH in the past two decades,leading to the development of disease-specific targeted therapies:prostacyclin analogues,endothelin receptor antagonists and inhibitors of five phosphodiesterase pathways.However,the clinical response to these therapies in SSc-associated PAH has not been as great as the one seen with idiopathic PAH.This review also focuses on the diagnosis and novel therapies that are currently available for PAH,as well as potential future therapeutic developments based on newly acquired knowledge of diverse pathogenic mechanisms.
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篇名 Pulmonary arterial hypertension associated with systemic sclerosis:Current diagnostic approach and therapeutic strategies
来源期刊 世界风湿病学杂志 学科 医学
关键词 PULMONARY ARTERIAL HYPERTENSION SYSTEMIC SCLEROSIS Diagnosis Therapy PROGNOSIS
年,卷(期) 2012,(2) 所属期刊栏目
研究方向 页码范围 12-20
页数 9页 分类号 R
字数 语种
DOI
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研究主题发展历程
节点文献
PULMONARY
ARTERIAL
HYPERTENSION
SYSTEMIC
SCLEROSIS
Diagnosis
Therapy
PROGNOSIS
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研究来源
研究分支
研究去脉
引文网络交叉学科
相关学者/机构
期刊影响力
世界风湿病学杂志
不定期
2220-3214
北京市朝阳区东四环中路62号楼远洋国际中
出版文献量(篇)
32
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0
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0
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