Leidig M, Schmiedecke T, John S, Mann J, Weber M
Medizinische Klinik IV mit Poliklinik, Universität Erlangen-Nürnberg.
Dtsch Med Wochenschr. 1992 Mar 20;117(12):455-9. doi: 10.1055/s-2008-1062333.
A 51-year-old woman had been suffering from blood-stained purulent sinusitis and antibiotic-resistant bouts of fever for 4 months. She had microhematuria and serological evidence of inflammation (erythrocyte sedimentation rate [ESR] 92/135 mm, C-reactive protein 5.0 mg/dl). When she was admitted to hospital suspected of having postinfectious glomerulonephritis she complained of spontaneous colic-like pains in the left flank. Within one day the haemoglobin concentration fell from 10 to 6.5 g/dl. Ultrasound and computed tomography demonstrated a large space-occupying lesion around the left kidney. At operation this was found to be a rupture of the kidney with perirenal bleeding which was treated without removing the kidney. No biopsy was taken, but serological tests showed antineutrophil cytoplasmatic antibodies (cANCA), indicating Wegener's granulomatosis as the cause of the compensated renal insufficiency and spontaneous renal rupture. Under immunosuppressive treatment the inflammatory signs (ESR 18/44 mm), fever, chronic maxillary sinusitis, raised serum creatinine concentration and the ANCA titre all regressed, while proteinuria of about 4 g/24 h persisted. There was no recurrence during a follow-up period of 15 months. Serological signs of marked inflammatory activity, urinary sediments of nephritis and spontaneous retroperitoneal bleeding should suggest that, in addition to lupus erythematodes and panarteritis nodosa, Wegener's granulomatosis be included in the differential diagnosis.
一名51岁女性患血脓性鼻窦炎且抗生素治疗无效的发热症状长达4个月。她有镜下血尿及炎症的血清学证据(红细胞沉降率[ESR]92/135mm,C反应蛋白5.0mg/dl)。当她因疑似感染后肾小球肾炎入院时,主诉左侧胁腹部突发绞痛样疼痛。一天内血红蛋白浓度从10g/dl降至6.5g/dl。超声和计算机断层扫描显示左肾周围有一巨大占位性病变。手术中发现是肾破裂伴肾周出血,未切除肾脏进行了相应治疗。未进行活检,但血清学检查显示抗中性粒细胞胞浆抗体(cANCA)阳性,提示韦格纳肉芽肿是导致代偿性肾功能不全和自发性肾破裂的原因。在免疫抑制治疗下,炎症指标(ESR 18/44mm)、发热、慢性上颌窦炎、血清肌酐浓度升高及ANCA滴度均有所下降,而约4g/24h的蛋白尿持续存在。随访15个月期间无复发。显著炎症活动的血清学指标、肾炎尿沉渣及自发性腹膜后出血提示,除红斑狼疮和结节性多动脉炎外,韦格纳肉芽肿也应列入鉴别诊断。