Department of Nephrology, Hospital de Santo António, Porto, Centro Hospitalar do Porto, Portugal.
Amyloid. 2011 Dec;18(4):240-4. doi: 10.3109/13506129.2011.614651. Epub 2011 Oct 13.
Whipple's disease (WD) is a chronic infection caused by Thropheryma whipplei that usually manifests with intestinal, articular, pulmonary, neurological and cardiac abnormalities. Rarely, WD has been associated with renal AA amyloidosis.We report a 50 year-old male with nephrotic syndrome and renal failure whose renal biopsy revealed extensive AA amyloidosis. Amyloid was not found in other organs, namely in gastrointestinal tract and bone marrow. There was no evidence of chronic inflammatory disease, and despite detailed investigation, the diagnosis of the underlying disease remained obscure. Eight months after referral he started peritoneal dialysis. Three years later he developed anorexia, weight loss, anemia, and recurrent attacks of non-bloody diarrhea. A biopsy of the small intestine showed typical histological findings of WD and PCR was positive for T. whipplei. He was treated with ceftriaxone followed by co-trimoxazole, with remission of complaints and histological features. Three years later the patient underwent successful cadaveric kidney transplantation. In this case, AA amyloidosis preceded the manifestations of WD. To the best of our knowledge, this is the first report of kidney transplantation in a patient with amyloidosis due to WD. Recurrence of amyloidosis in renal graft is not expected.
惠普尔病(WD)是一种由 Tropheryma whipplei 引起的慢性感染,通常表现为肠道、关节、肺部、神经和心脏异常。罕见情况下,WD 与肾 AA 淀粉样变性有关。我们报告了一例 50 岁男性肾病综合征和肾衰竭患者,其肾活检显示广泛的 AA 淀粉样变性。其他器官,如胃肠道和骨髓中均未发现淀粉样蛋白。没有慢性炎症性疾病的证据,尽管进行了详细的调查,但潜在疾病的诊断仍不清楚。转诊 8 个月后,他开始接受腹膜透析。3 年后,他出现厌食、体重减轻、贫血和反复发作的无血腹泻。小肠活检显示典型的 WD 组织学表现,PCR 检测 T. whipplei 阳性。他接受头孢曲松和复方磺胺甲噁唑治疗,症状和组织学特征缓解。3 年后,患者成功接受了尸体肾移植。在这种情况下,AA 淀粉样变性先于 WD 表现。据我们所知,这是首例因 WD 导致的淀粉样变性患者进行肾移植的报告。预计肾移植后不会出现淀粉样变性复发。