Pulmonary Arterial Hypertension In Systemic Sclerosis: Challenges In Diagnosis, Screening And Treatment

被引：20

作者：

Saygin, Didem ^{[1
]}

Domsic, Robyn T. ^{[2
]}

机构：

[1] Univ Pittsburgh, Med Ctr, Dept Med, Pittsburgh, PA 15213 USA

[2] Univ Pittsburgh, Sch Med, Div Rheumatol & Clin Immunol, S706 BST,200 Lothrop St, Pittsburgh, PA 15213 USA

来源：

OPEN ACCESS RHEUMATOLOGY-RESEARCH AND REVIEWS | 2019年 / 11卷

关键词：

scleroderma; pulmonary hypertension; screening; CONNECTIVE-TISSUE DISEASE; COMBINATION THERAPY; SUBGROUP ANALYSIS; RISK-FACTORS; BOSENTAN; SURVIVAL; AMBRISENTAN; PREDICTORS; ECHOCARDIOGRAPHY; MONOTHERAPY;

D O I：

10.2147/OARRR.S228234

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Systemic sclerosis (SSc) is a chronic, multisystem autoimmune disease characterized by vasculopathy, fibrosis and immune system activation. Pulmonary hypertension and interstitial lung disease account for majority of SSc-related deaths. Diagnosis of SSc-PAH can be challenging due to nonspecific clinical presentation which can lead to delayed diagnosis. Many screening algorithms have been developed to detect SSc-associated pulmonary arterial hypertension (SSc-PAH) in early stages. Currently used PAH-specific medications are largely extrapolated from IPAH studies due to smaller number of patients with SSc-PAH. In this review, we discuss the current state of knowledge in epidemiology and risk factors for development of SSc-PAH, and challenges and potential solutions in the diagnosis, screening and management of SSc-PAH.

引用

页码：323 / 333

页数：11

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