Schlumpf A, Marbet U A, Stöcklin E, Wegmann W, Lämmle B, Mujagic M, Jösch W, Thiel G, Thölen H, Olivieri W, Gudat F, Torhorst J, Zollinger H U, Mihatsch M J
Klin Wochenschr. 1983 Jan 3;61(1):25-33. doi: 10.1007/BF01484436.
Report is given on a 68-year-old man who suffered primarily from progressive weight loss and repeated episodes of fever and arthralgia. Later, liver dysfunction and renal insufficiency developed. Liver and kidney biopsies disclosed granulomatous hepatitis and nephritis. Because of the morphologic and clinical findings, the diagnosis of Boeck's disease was made. Shortly before death, diarrhea developed. Autopsy revealed a massive systemic involvement in Whipple's disease proven by light and electron microscopy and immunofluorescence. Tuberculoid and epitheloid cell granulomas and isolated giant cells were found in addition to the biopsy findings in skeleton muscles, the small intestine, lymphnodes and bronchi. At autopsy, the kidney showed chronic interstitial nephritis. The literature of kidney involvement in Whipple's disease is reviewed. This is the first case with granulomatous interstitial nephritis and chronic renal insufficiency in an inadequately treated Whipple's disease.
报告了一名68岁男性,主要表现为进行性体重减轻以及反复发热和关节痛。后来出现肝功能障碍和肾功能不全。肝脏和肾脏活检显示为肉芽肿性肝炎和肾炎。基于形态学和临床发现,诊断为伯克氏病。在死亡前不久,出现了腹泻。尸检显示通过光镜、电镜和免疫荧光证实为惠普尔病的广泛全身受累。除了在骨骼肌、小肠、淋巴结和支气管中的活检发现外,还发现了结核样和上皮样细胞肉芽肿以及孤立的巨细胞。尸检时,肾脏显示为慢性间质性肾炎。对惠普尔病肾脏受累的文献进行了综述。这是第一例未经充分治疗的惠普尔病出现肉芽肿性间质性肾炎和慢性肾功能不全的病例。