Niemczyk Stanislaw, Filipowicz Ewa, Wozniacki Lukasz, Grochowski Janusz, Zaleski Leszek, Grzejszczak Agnieszka, Ptasinska Agnieszka Perkowska, Koperski Lukasz, Rowinska Joanna Matuszkiewicz
Department of Nephrology, Dialysis Therapy and Internal Medicine, Medical University of Warsaw, 1a Banacha Street, 02-097 Warsaw, Poland.
Cases J. 2009 Sep 17;2:8444. doi: 10.4076/1757-1626-2-8444.
Whipple disease is a rare systemic infection caused by Tropheryma whippelii that usually manifests with joint pain, weight loss, diarrhoea and abdominal pain. However, in some cases the infection may involve other organs and tissues.
We report on a 44-year-old man with Whipple disease which led to renal amyloidosis and end-stage renal failure. In this case, the patient was diagnosed with Whipple disease and commenced on a 12-month trimetoprime-sulfametoxasole therapy with good result. Six months after cessation of therapy the patient was readmitted to hospital due to signs of renal failure. An urgent kidney biopsy was performed which revealed secondary amyloidosis. Despite intensive immunosuppressive treatment, renal parameters gradually deteriorated and haemodialysis was started eventually. Three months later the patient's general condition dramatically worsened with bloody diarrhoea, bilious vomiting and progressive malnutrition. The repeated endoscopic examination confirmed severe recurrence of Whipple disease. Ceftriaxone and total parenteral nutrition was started what greatly improved patient's state.
To our knowledge based on systematic review, this is the first case report on Whipple disease complicated by secondary amyloidosis and kidney failure maintained on permanent renal replacement therapy. It is strongly suspected that the use of immunosuppressive treatment in such cases may exacerbate the course of Whipple disease and cause life-threatening complications.
惠普尔病是一种由惠普尔嗜组织菌引起的罕见的全身性感染，通常表现为关节疼痛、体重减轻、腹泻和腹痛。然而，在某些情况下，感染可能累及其他器官和组织。
我们报告一例44岁患惠普尔病的男性患者，该病导致肾淀粉样变性和终末期肾衰竭。在该病例中，患者被诊断为惠普尔病，并开始接受为期12个月的甲氧苄啶-磺胺甲恶唑治疗，效果良好。治疗停止6个月后，患者因肾衰竭迹象再次入院。进行了紧急肾活检，结果显示为继发性淀粉样变性。尽管进行了强化免疫抑制治疗，但肾脏参数仍逐渐恶化，最终开始进行血液透析。3个月后，患者的一般状况急剧恶化，出现血性腹泻、胆汁性呕吐和进行性营养不良。重复内镜检查证实惠普尔病严重复发。开始使用头孢曲松和全胃肠外营养，患者状况大为改善。
据我们基于系统评价的了解，这是首例关于惠普尔病并发继发性淀粉样变性和肾衰竭并需长期肾脏替代治疗的病例报告。强烈怀疑在此类病例中使用免疫抑制治疗可能会加重惠普尔病的病程并导致危及生命的并发症。
