Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody.
Successful Treatment of Pulmonary Arterial Hypertension in Systemic Sclerosis with Anticentriole Antibody.
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使用抗中心粒抗体成功治疗系统性硬化症中的肺动脉高压。
DOI：
10.1155/2020/1926908
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发表时间：
2020
期刊：
影响因子：
--
通讯作者：
Harigae H.
中科院分区：
文献类型：
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作者：
Ishii Y;Fujii H;Sugimura K;Shirai T;Hoshi Y;Fujita Y;Shirota Y;Ishii T;Shimokawa H;Harigae H.
系统性硬化症（SSc）的特征在于皮肤硬化和多器官损害，其可导致死亡，并且通常伴有几种特异性自身抗体，每种抗体都与特征性并发症相关。其中，抗中心粒抗体最近被报道与SSc相关性肺动脉高压（SSc‐PAH）高度相关。一般来说，几种血管扩张剂被用作SSc‐PAH的治疗药物，而免疫抑制疗法则不是。在此，我们报告了一例62岁女性抗中心粒抗体阳性SSc‐PAH患者接受免疫抑制剂和血管扩张剂治疗的病例。她出现了两年的劳累性呼吸困难，并因中心粒染色模式和其他症状而被诊断为肺动脉高压和SSc，但没有手指硬化。最初给予口服血管扩张剂，但对呼吸困难无效。开始使用免疫抑制剂，如泼尼松龙和环磷酰胺。两种药物均改善了平均肺动脉压和6分钟步行距离，抗中心抗体也消失了。在这种情况下，正确诊断了SSc‐PAH伴抗中心粒抗体，免疫抑制剂和血管扩张剂改善了PAH伴抗中心粒抗体的血流动力学并稳定维持，此外，还降低了抗中心粒抗体的滴度。这表明抗中心粒抗体可能代表SSc‐PAH患者亚组中对治疗的良好应答组。
Systemic sclerosis (SSc) is characterized by skin sclerosis and multiple organ damages which may cause mortality and is usually accompanied with several specific autoantibodies, each of which is associated with characteristic complications. Among them, anticentriole antibody is recently reported to be highly associated with SSc‐associated pulmonary arterial hypertension (SSc‐PAH). In general, several vasodilators are used as therapeutic drugs for SSc‐PAH, whereas immunosuppressive therapies are not. Here, we report the case of a 62‐year‐old female with anticentriole antibody‐positive SSc‐PAH treated with immunosuppressants and vasodilators. She presented with two‐year exertional dyspnea and was diagnosed with PAH and SSc owing to the centriole staining pattern and other symptoms without digital sclerosis. Oral vasodilators were initially administered but were not sufficiently effective on dyspnea. Immunosuppressants such as prednisolone and cyclophosphamide were started. Both of them improved mean pulmonary arterial pressure and 6‐minute walk distance, and the anticentriole antibody also disappeared. In this case, SSc‐PAH with anticentriole antibody was properly diagnosed and immunosuppressants and vasodilators improved the hemodynamics of PAH with anticentriole antibody and stably maintained it and, in addition, reduced the titer of anticentriole antibody. This indicates that anticentriole antibody might represent a good responsive group to therapies among subgroups of patients with SSc‐PAH.