Hirano K, Tomino Y, Murai T, Wang L N, Shirato I, Koide H
Department of Internal Medicine, Juntendo University School of Medicine, Japan.
Nihon Jinzo Gakkai Shi. 1990 Mar;32(3):339-44.
A case of Wegener's granulomatosis with renal dysfunction is described. Granulomatous lesions of the lung, left eyelid and left leg, and chronic sinusitis were observed at the time of admission. Renal and skin biopsy specimens revealed typical features of Wegener's granulomatosis on light microscopy and immunofluorescence microscopy. A dramatic response occurred following corticosteroid and cyclophosphamide therapy, characterized by improvement of proteinuria and renal function, and disappearance of the granulomas and 67gallium citrate accumulations in both the eyes and nose. Furthermore, the levels of anticytoplasmic antibodies (ACPA) in the serum samples were significantly decreased after such therapy. It appears that combined therapy with corticosteroid and cyclophosphamide can be effective in improving proteinuria and renal dysfunction, and in reducing of ACPA in patients with Wegener's granulomatosis.
本文描述了一例伴有肾功能不全的韦格纳肉芽肿病病例。入院时观察到肺部、左侧眼睑和左腿的肉芽肿性病变以及慢性鼻窦炎。肾脏和皮肤活检标本在光学显微镜和免疫荧光显微镜下显示出韦格纳肉芽肿病的典型特征。皮质类固醇和环磷酰胺治疗后出现显著反应，表现为蛋白尿和肾功能改善，肉芽肿以及双眼和鼻部的枸橼酸镓积聚消失。此外，治疗后血清样本中的抗细胞质抗体（ACPA）水平显著降低。看来皮质类固醇和环磷酰胺联合治疗对改善韦格纳肉芽肿病患者的蛋白尿和肾功能不全以及降低ACPA可能有效。
