[Systemic sclerosis associated with microscopic polyangitis presenting with high myeloperoxidase (MPO) titer and necrotizing angitis: a case report].
[Systemic sclerosis associated with microscopic polyangitis presenting with high myeloperoxidase (MPO) titer and necrotizing angitis: a case report].
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[与显微多血管炎相关的系统性硬化症，表现为高髓过氧化物酶（MPO）滴度和坏死性血管炎：病例报告]。
DOI：
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发表时间：
2002
期刊：
影响因子：
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通讯作者：
E. Suematsu
中科院分区：
文献类型：
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作者：
T. Miyamura;Masahiro Yamamoto;H. Shimada;E. Suematsu
我们在此报告一例系统性硬化症合并显微多血管炎。患者是一名54岁的女性，1992年在一家医院被诊断为系统性硬化症，但她没有接受任何治疗。患者自2001年初开始出现发热、感觉异常、双下肢肌无力等症状，被介绍到我院就诊。她的皮肤从手指一直延伸到上臂，皮肤硬化，双下肢无力，并伴有网状肌。实验室检查WBC 11600 /微升，CRP 6.63 mg/dl, ch50 24 U/ml，抗Scl-70抗体90.1指数，MPO-ANCA 281 EU，未发现肾功能受损。胸部计算机断层扫描显示间质性肺炎，活检标本中发现右腓肠神经坏死性血管炎。基于这些发现，我们诊断她患有系统性硬化症并显微镜下多血管炎（MPA）。在确诊后，她接受了类固醇治疗，此后她的症状和实验室检查结果立即得到改善。在合并mpo -ANCA相关血管炎的系统性硬化症患者中，有许多病例报道为ANCA阳性系统性硬化症。然而，由于我们的患者在腓肠神经活检中表现为坏死性血管炎，并且没有肾功能受损的证据，我们诊断她患有系统性硬化症合并MPA，而不是ANCA阳性系统性硬化症。因此，该患者的病理状态似乎不同于anca阳性的系统性硬化症。我们得出结论，该患者同时患有系统性硬化症和MPA。因此，值得注意的是，一些报告为anca阳性系统性硬化症的患者也可能患有系统性硬化症合并MPA。
We herein report a case of systemic sclerosis associated with microscopic polyangitis. The patient was a 54-year-old woman, who was diagnosed to have systemic sclerosis at a hospital in 1992, but she did not receive any medical treatment. She had been suffering from pyrexia, paresthesia and muscle weakness of both lower limbs since the beginning of 2001, and was introduced to our hospital. She showed hardened skin extending from her fingers to upper arms, weakness in both lower limbs and livedo reticularis. Her laboratory test showed WBC 11, 600/microliter, CRP 6.63 mg/dl, CH 50 24 U/ml, anti Scl-70 antibody 90.1 index, and MPO-ANCA 281 EU, but no impaired renal function was recognized. Chest computed tomography showed interstitial pneumonia while necrotising vasculitis of the right sural nerve was found in a biopsy specimen. Based on these findings, we diagnosed her to have systemic sclerosis accompanied with microscopic polyangitis (MPA). She received steroid treatment after the diagnosis was made, and her symptoms and the laboratory findings thereafter immediately improved. Many cases have been reported to have ANCA positive systemic sclerosis among patients with systemic sclerosis that are complicated MPO-ANCA-related vasculitis. However, since our patient demonstrated necrotising vasculitis in a sural nerve biopsy and no evidence of an impaired renal function, we diagnosed her to have systemic sclerosis complicated with MPA instead of ANCA positive systemic sclerosis. The pathological state of this patient thus seemed to be different from that of ANCA-positive systemic sclerosis. We concluded that this patient had both systemic sclerosis and MPA. It is therefore important to note that some patients who have been reported to have ANCA-positive systemic sclerosis may also have systemic sclerosis complicated with MPA.