Characteristics of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis and Anticentriole Autoantibodies

被引：5

作者：

Maki, Hisataka ^{[1
]}

Kubota, Kana ^{[1
,2
]}

Hatano, Masaru ^{[1
,4
]}

Minatsuki, Shun ^{[1
]}

Amiya, Eisuke ^{[1
,4
]}

Yoshizaki, Ayumi ^{[3
]}

Asano, Yoshihide ^{[3
]}

Morita, Hiroyuki ^{[1
]}

Sato, Shinichi ^{[3
]}

Komuro, Issei ^{[1
]}

机构：

[1] Univ Tokyo, Dept Cardiovasc Med, Grad Sch Med, Tokyo, Japan

[2] Jichi Med Univ, Dept Internal Med, Div Cardiovasc Med, Shimotsuke, Tochigi, Japan

[3] Univ Tokyo, Grad Sch Med, Dept Dermatol, Tokyo, Japan

[4] Univ Tokyo, Grad Sch Med, Therapeut Strategy Heart Failure, Tokyo, Japan

来源：

INTERNATIONAL HEART JOURNAL | 2020年 / 61卷 / 02期

关键词：

Elderly; COMBINATION THERAPY; SCLERODERMA; CENTRIOLE; ANTIBODIES;

D O I：

10.1536/ihj.19-659

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Anticentriole autoantibodies-positive systemic sclerosis (SSc) has been reported to develop pulmonary arterial hypertension (PAH) at a high rate. In this report, we describe two patients with anticentriole antibodiespositive SSc-PAH who were treated with pulmonary vasodilators. Both cases were elderly women with poor physical conditions and clinical findings of SSc. Case 1 was resistant to combination therapy with pulmonary vasodilators; in Case 2, hemodynamic improvement was obtained by upfront combination therapy at an early stage. Because anticentriole antibodies-positive SSc-PAH rapidly deteriorates, careful hemodynamic observation and timely aggressive use of pulmonary vasodilators should be considered.

引用

页码：413 / 418

页数：6

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