Surendran Sujit, Gundappa Chandramohan, Gandhi Arun, Kurien Anila Abraham, Fernando Edwin
Department of Nephrology, Government Stanley Medical College and Hospital, Chennai, Tamil Nadu, India.
Saudi J Kidney Dis Transpl. 2017 May-Jun;28(3):639-644. doi: 10.4103/1319-2442.206450.
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.
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。
