Grosser G, Mundinger A, Pumpe C, Herbst E W, Kuhlmann M, Kröpelin T
Abt. Röntgendiagnostik, Universität Freiburg.
Rofo. 1989 Aug;151(2):167-70. doi: 10.1055/s-2008-1047153.
Microaneurysms of renal and visceral arteries are characteristic signs of periarteritis nodosa. Normally they are not found in Wegener's disease, where glomerulonephritis is commonly observed. We report on a patient with vasculitis of the upper and lower respiratory tract, focal glomerulonephritis, prostatic and pulmonary granulomas and anti-cytoplasm antibodies corresponding to Wegener's disease. The most striking findings in angiography were multiple small aneurysms of the peripheral branches of the renal artery. At necroscopy these angiographic findings were histologically proven as necrosis of the arterial wall with destruction of the elastic lamina, causing local vascular ectasia. These renal vascular changes are a characteristic sign of periarteritis nodosa. The combination of clinical, laboratory, radiologic and histologic findings in our patient can be explained as an overlap-syndrome of Wegener's disease and periarteritis nodosa. We assume that the combination of pathologic findings in our patient correspond to a rare atypical renal manifestation of Wegener's disease.
肾动脉和内脏动脉的微动脉瘤是结节性多动脉炎的特征性体征。通常在韦格纳肉芽肿病中不会发现微动脉瘤,该病常见肾小球肾炎。我们报告了1例上下呼吸道血管炎、局灶性肾小球肾炎、前列腺和肺部肉芽肿以及与韦格纳肉芽肿病相符的抗细胞质抗体患者。血管造影最显著的发现是肾动脉外周分支多发小动脉瘤。尸检时,这些血管造影结果经组织学证实为动脉壁坏死伴弹力层破坏,导致局部血管扩张。这些肾血管改变是结节性多动脉炎的特征性体征。我们患者的临床、实验室、放射学和组织学检查结果可解释为韦格纳肉芽肿病和结节性多动脉炎的重叠综合征。我们认为患者的病理检查结果组合符合韦格纳肉芽肿病罕见的非典型肾脏表现。