Limited cutaneous systemic sclerosis associated with MPO-ANCA positive renal small vessel vasculitis of the microscopic polyangiitis type.
Limited cutaneous systemic sclerosis associated with MPO-ANCA positive renal small vessel vasculitis of the microscopic polyangiitis type.
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与 MPO-ANCA 阳性的显微镜下多血管炎型肾小血管炎相关的局限性皮肤系统性硬化症。
DOI：
10.1053/ajkd.2000.16215
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发表时间：
2000
期刊：
影响因子：
--
通讯作者：
Y. Vanrenterghem
中科院分区：
文献类型：
--
作者：
B. Maes;An Van Mieghem;T. Messiaen;D. Kuypers;Boudewijn Van Damme;Y. Vanrenterghem
系统性硬化症中的肾脏疾病可表现为各种类型。1例66岁女性，长期存在局限性皮肤系统性硬化症CREST综合征变异型，临床表现为左脚突然下垂、快速进行性肾功能不全，伴轻度蛋白尿、肾炎性尿沉渣、尿量900毫升/天。无D-青霉胺吸入史，也无恶性动脉高压或微血管病理性溶血性贫血的征象。肾组织学检查显示显微镜下可见多血管炎类型的小血管炎。血清学检查显示，抗中性粒细胞胞浆抗体显著增加，呈核周型，抗髓过氧化物酶抗体效价升高。尚无Sjögren综合征的临床或实验室迹象。这份临床报告为系统性硬化症的肾脏疾病谱增加了新的信息。它讨论了系统性硬化症与显微镜下多血管炎型小血管炎的关系，以及血清标志物的可能意义。
Renal disease in systemic sclerosis may present in various patterns. A 66-year-old woman with a history of longstanding limited cutaneous systemic sclerosis of the CREST syndrome variant presented with a sudden left foot drop and rapidly progressive renal insufficiency associated with mild proteinuria, a nephritic urine sediment, and a urinary output of 900 mL/d. There was no history of intake of D-penicillamine, and there were no signs of malignant arterial hypertension or microangiopathic hemolytic anemia. Renal histology showed a small vessel vasculitis of the microscopic polyangiitis type. Serologic tests showed a marked increase of antineutrophil cytoplasmic antibodies with a perinuclear pattern and an elevated titer of antimyeloperoxidase antibodies. No clinical or laboratory signs of Sjögren's syndrome were present. This clinical report adds new information to the spectrum of renal disease in systemic sclerosis. It discusses the association between systemic sclerosis and small vessel vasculitis of the microscopic polyangiitis type as well as the possible meaning of serologic markers.