Schönermarck Ulf, Grahovac Maja, Sárdy Miklós, Dolch Michael, Wollenberg Andreas
Nephrology Division, Department I of Internal Medicine.
Department of Dermatology and Allergy.
NDT Plus. 2010 Dec;3(6):567-9. doi: 10.1093/ndtplus/sfq145. Epub 2010 Aug 5.
Wegener's granulomatosis is an ANCA-associated small vessel vasculitis. Because histologically immune complex deposits are frequently lacking, the term pauci-immune has been introduced for this subgroup. We report a patient with fulminant, severe PR3-ANCA-positive Wegener's granulomatosis and multi-organ involvement (upper respiratory tract, lung, kidneys, skin and general symptoms), who showed pronounced immunoglobulin and complement deposits within the skin biopsy. Our observation supports the hypothesis that immune complex deposits may be under-recognized in early lesions of ANCA-associated Wegener's granulomatosis.
韦格纳肉芽肿病是一种与抗中性粒细胞胞浆抗体（ANCA）相关的小血管炎。由于组织学上常缺乏免疫复合物沉积，因此该亚组被称为寡免疫性。我们报告了一名患有暴发性、严重的抗蛋白酶3（PR3）-ANCA阳性韦格纳肉芽肿病且有多器官受累（上呼吸道、肺、肾、皮肤及全身症状）的患者，其皮肤活检显示有明显的免疫球蛋白和补体沉积。我们的观察结果支持这样一种假说，即免疫复合物沉积在ANCA相关的韦格纳肉芽肿病早期病变中可能未得到充分认识。
