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抗中心粒自身抗体阳性的系统性硬化症患者肺动脉高压发病率高。

High incidence of pulmonary arterial hypertension in systemic sclerosis patients with anti-centriole autoantibodies.

作者信息

Hamaguchi Yasuhito, Matsushita Takashi, Hasegawa Minoru, Ueda-Hayakawa Ikuko, Sato Sinichi, Takehara Kazuhiko, Fujimoto Manabu

机构信息

a Department of Dermatology , Faculty of Medicine, Kanazawa University Institute of Medical, Pharmaceutical and Health Sciences , Kanazawa , Japan.

出版信息

Mod Rheumatol. 2015 Sep;25(5):798-801. doi: 10.3109/14397595.2013.844296. Epub 2015 Jul 29.

DOI:10.3109/14397595.2013.844296
PMID:24252018
Abstract

Systemic sclerosis (SSc)-related autoantibodies are useful tools in identifying clinically homogenous subsets of patients and predicting their prognosis. In this report, we described five SSc patients with anti-centriole antibodies. All five patients were females and had digital ulcers/gangrene. Four of five (80%) patients had pulmonary arterial hypertension (PAH). None of the five patients had active pulmonary fibrosis or developed renal crisis. Anti-centriole antibodies may be a marker for PAH and digital ulcers/gangrene.

摘要

系统性硬化症(SSc)相关自身抗体是识别临床同质患者亚组和预测其预后的有用工具。在本报告中,我们描述了5例抗中心粒抗体阳性的系统性硬化症患者。所有5例患者均为女性,并有指端溃疡/坏疽。5例患者中有4例(80%)患有肺动脉高压(PAH)。5例患者均无活动性肺纤维化或发生肾危象。抗中心粒抗体可能是肺动脉高压和指端溃疡/坏疽的一个标志物。

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Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody.
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