Meyrier A, Français P, Lesavre P, Mougenot B, Ronco P, Jeanmougin M, Français C
Nephrologie. 1984;5(1):1-7.
A 58 year-old woman had atypical chronic urticaria, arthralgias and abdominal pain. Attacks of angioneurotic edema occurred. Proteinuria was discovered. She had clinical and biological signs of inflammation, leukoneutropenia , antileukocyte antibodies and low CH 50, C1q and C4 levels without functional C1 esterase deficiency. C1q precipitins were not detectable. Skin biopsy disclosed angiitis and by immunofluorescence a lupus band test was positive. Serologic investigations in search of SLE were negative. Renal biopsy showed mesangial deposits, capillary loop thickening and mesangial fixation of anti-IgG, C3 C1q and C4 antisera. In the interstitium, voluminous perivenular inflammatory infiltrates were visible. With corticosteroid treatment clinical manifestations subsided and proteinuria disappeared. This observation of McDuffie 's angiitis with renal venulitis leads to a review of the literature with discussion of the mechanisms of hypocomplementemia.
一名58岁女性患有非典型慢性荨麻疹、关节痛和腹痛。出现血管神经性水肿发作。发现蛋白尿。她有炎症的临床和生物学体征、白细胞减少、抗白细胞抗体以及低CH 50、C1q和C4水平，且无功能性C1酯酶缺乏。未检测到C1q沉淀素。皮肤活检显示血管炎，免疫荧光检查狼疮带试验呈阳性。寻找系统性红斑狼疮的血清学检查为阴性。肾活检显示系膜沉积物、毛细血管袢增厚以及抗IgG、C3、C1q和C4抗血清的系膜固定。在间质中，可见大量静脉周围炎性浸润。经皮质类固醇治疗后，临床表现消退，蛋白尿消失。对麦克达菲氏血管炎合并肾静脉炎的这一观察结果促使我们回顾文献并讨论补体减少的机制。
