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肉芽肿性多血管炎与嗜酸性肉芽肿性多血管炎的重叠。

An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis.

作者信息

Surendran Sujit, Gundappa Chandramohan, Gandhi Arun, Kurien Anila Abraham, Fernando Edwin

机构信息

Department of Nephrology, Government Stanley Medical College and Hospital, Chennai, Tamil Nadu, India.

出版信息

Saudi J Kidney Dis Transpl. 2017 May-Jun;28(3):639-644. doi: 10.4103/1319-2442.206450.

Abstract

We present a case report of overlap of granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome). We report a 45-year-old female who presented with rapidly progressive renal failure associated with fever, polyarthralgia, and respiratory symptoms with cytoplasmic antineutrophilic cytoplasmic antibody (ANCA) and proteinase (PR-3) antigen positivity. Computerized tomography scan of the chest showed diffuse alveolar hemorrhage with renal biopsy revealing pauci-immune necrotizing crescentic glomerulonephritis with intense eosinophilic infiltration suggestive of eosinophilic GPA (EGPA). Our patient had ANCA-associated vasculitis (AAV) with features suggestive of both GPA and EGPA. She was treated with methylprednisolone and cyclophosphamide and attained remission after 2 weeks of therapy. This is a rare report of a patient with AAV having features of both EGPA and GPA.

摘要

我们报告一例肉芽肿性多血管炎(GPA,既往称为韦格纳肉芽肿)和嗜酸性肉芽肿性多血管炎(EGPA,既往称为变应性肉芽肿性血管炎)重叠的病例。我们报道一名45岁女性,表现为快速进展性肾衰竭,伴有发热、多关节痛和呼吸道症状,胞浆型抗中性粒细胞胞浆抗体(ANCA)及蛋白酶(PR-3)抗原阳性。胸部计算机断层扫描显示弥漫性肺泡出血,肾活检显示寡免疫性坏死性新月体性肾小球肾炎,伴有强烈嗜酸性粒细胞浸润,提示嗜酸性GPA(EGPA)。我们的患者患有ANCA相关性血管炎(AAV),具有GPA和EGPA两者的特征。她接受了甲泼尼龙和环磷酰胺治疗,治疗2周后病情缓解。这是一例罕见的具有EGPA和GPA特征的AAV患者的报告。

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