Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects

被引：31

作者：

Kato, Masaru

Atsumi, Tatsuya ^{[1
,2
]}

机构：

[1] Hokkaido Univ, Fac Med, Dept Rheumatol Endocrinol & Nephrol, Sapporo, Hokkaido, Japan

[2] Hokkaido Univ, Grad Sch Med, Sapporo, Hokkaido, Japan

来源：

EUROPEAN JOURNAL OF CLINICAL INVESTIGATION | 2018年 / 48卷 / 02期

关键词：

connective tissue diseases; pulmonary arterial hypertension; pulmonary hypertension; systemic lupus erythematosus; systemic sclerosis; SYSTEMIC-LUPUS-ERYTHEMATOSUS; BRAIN NATRIURETIC PEPTIDE; IMMUNOSUPPRESSIVE THERAPY; VENOOCCLUSIVE DISEASE; PROGNOSTIC-FACTORS; SCLEROSIS; SURVIVAL; REGISTRY; AMBRISENTAN; PREVALENCE;

D O I：

10.1111/eci.12876

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Recent studies have clarified that pulmonary arterial hypertension associated with connective tissue diseases (CTD-PAH) has some distinctive clinical aspects from other PAH, such as high prevalence, venous and cardiac involvement, less favourable outcome, helpfulness of detection algorithm, response to immunosuppression, pre-PAH conditions in borderline pulmonary arterial pressure and coexistence of interstitial lung disease. In this review, by focusing on these distinctive aspects, we discuss how to provide an efficacious and safe management of CTD-PAH and garner attention to areas where further evidence is desired.

引用

页数：9

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