High incidence of pulmonary arterial hypertension in systemic sclerosis patients with anti-centriole autoantibodies
High incidence of pulmonary arterial hypertension in systemic sclerosis patients with anti-centriole autoantibodies
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DOI：
10.3109/14397595.2013.844296
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发表时间：
2015-01-01
影响因子：
2.2
通讯作者：
Fujimoto, Manabu
中科院分区：
文献类型：
--
作者：
Hamaguchi, Yasuhito;Matsushita, Takashi;Fujimoto, Manabu
系统性硬化症(SSC)相关自身抗体是识别临床同源亚群患者并预测其预后的有用工具。在这篇报道中，我们描述了5例具有抗中心粒抗体的SSc患者。所有五名患者都是女性，都有手指溃疡/坏疽。5例患者中有4例(80%)有肺动脉高压。五名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PAH和指端溃疡/坏疽的标志物。
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.