Yoshikawa Y, Watanabe T
Virchows Arch A Pathol Anat Histopathol. 1984;402(4):361-72. doi: 10.1007/BF00734634.
Review of the kidneys in 24 autopsy cases of Wegener's granulomatosis revealed a significant granulomatous glomerular lesion in eight of the cases. To gain a better understanding of this peculiar lesion, focusing on its pathogenesis, we attempted a thorough investigation on both glomerular and vascular lesions of the kidneys. Semiquantitative analysis of the glomerular lesions indicated that existence of a severe glomerular damage probably constitutes a necessary condition in the development of granulomatous glomerulonephritis, because the granulomatous glomerular lesion was typically seen in company with a widely distributed glomerular lesion represented by thrombotic and necrotic occlusion of capillary tufts and crescent formation. Necrotizing vasculitis in the kidney was always encountered, especially in small branches of renal arteries and vasa recta. A serial section study of the two most typical cases indicated that granulomatous inflammation apparently originated in hilar arteriolitis , which extended along the pericapsular space and developed into diffusely circumferential periglomerular inflammation. We conclude that two factors are jointly at work, one inside and the other outside of the glomerulus in the pathogenesis of granulomatous glomerulonephritis: there is a thrombotic and necrotic lesion of the glomerular tuft, on the one hand and pericapsulitis originating in hilar arteriolitis on the other.
对24例韦格纳肉芽肿病尸检病例的肾脏进行检查发现，其中8例存在显著的肉芽肿性肾小球病变。为了更好地理解这种特殊病变，重点关注其发病机制，我们对肾脏的肾小球和血管病变进行了全面研究。肾小球病变的半定量分析表明，严重的肾小球损伤的存在可能是肉芽肿性肾小球肾炎发生发展的必要条件，因为肉芽肿性肾小球病变通常与以毛细血管袢血栓形成和坏死性闭塞以及新月体形成为特征的广泛分布的肾小球病变同时出现。肾脏坏死性血管炎总是会出现，尤其是在肾动脉小分支和直小血管中。对两个最典型病例的连续切片研究表明，肉芽肿性炎症显然起源于肾门小动脉炎，炎症沿肾包膜间隙扩展并发展为弥漫性的肾小球周围炎症。我们得出结论，在肉芽肿性肾小球肾炎的发病机制中，有两个因素共同起作用，一个在肾小球内部，另一个在肾小球外部：一方面是肾小球袢的血栓形成和坏死性病变，另一方面是起源于肾门小动脉炎的肾包膜炎。
