Kanfer A, Sraer J D, Feintuch M J, Morel-Maroger L, Beaufils P, Richet G
Nouv Presse Med. 1976 Sep 18;5(30):1883-8.
In 9 patients aged 16 to 71 years with acute renal failure, histological examination disclosed polyarteritis nodosa. Symptoms of rapidly progressive glomerulonephritis (GN) were present in 7 cases. Hypertension was a constant feature. Fever and muscular articular and cutaneous signs occured at times simulating Henoch-Schönlein purpura. Necrotizing angiitis was a constant finding, with perivascular granuloma in 7 cases. Lesions affecting smaller arteries were associated with diffuse or segmental extracapillary GN, and lesions of larger arteries with ischaemic appearance of the glomeruli. Diagnosis was obtained during life by percutaneous renal biopsy in 5 patients and muscular or cutaneous biopsy in 2 other patients, whereas the evidence was only obtained on post mortem examination in the 2 remaining patients. In the 6 renal biopsies examined by immunofluorescence, fibrinogen was always present in glomeruli and/or arteries. In one patient there was a linear deposit of IgG along glomerular basement membrane (GBM) and there were circulating antibodies directed against GBM. Arteries of different sizes may be damaged in various organs, as showed by arteriography and autopsy. Steroid therapy and immunosuppressive drugs had no effect on renal symptoms. 8 out of 9 patients died rapidly, in 6 the death was due to extrarenal localization of the disease, even though uraemia was controlled by haemodialysis. The last patient died 2 years after onset of the disease, while being on maintenance haemodialysis treatment.
在9例年龄为16至71岁的急性肾衰竭患者中,组织学检查发现结节性多动脉炎。7例患者出现快速进行性肾小球肾炎(GN)症状。高血压是一个持续存在的特征。有时会出现发热、肌肉关节和皮肤症状,类似过敏性紫癜。坏死性血管炎是一个持续的发现,7例患者伴有血管周围肉芽肿。影响较小动脉的病变与弥漫性或节段性毛细血管外GN相关,而较大动脉的病变与肾小球缺血外观相关。5例患者通过经皮肾活检在生前获得诊断,另外2例患者通过肌肉或皮肤活检获得诊断,而其余2例患者仅在尸检时获得证据。在通过免疫荧光检查的6例肾活检中,纤维蛋白原始终存在于肾小球和/或动脉中。1例患者沿肾小球基底膜(GBM)有IgG线性沉积,并且存在针对GBM的循环抗体。动脉造影和尸检显示,不同大小的动脉可能在各个器官中受损。类固醇疗法和免疫抑制药物对肾脏症状无效。9例患者中有8例迅速死亡,6例患者的死亡是由于疾病的肾外定位,尽管通过血液透析控制了尿毒症。最后1例患者在疾病发作2年后死亡,当时正在接受维持性血液透析治疗。