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Case Reports
. 2009 Sep 17:2:8444.
doi: 10.4076/1757-1626-2-8444.

Renal amyloidosis in Whipple disease: a case report

Stanislaw Niemczyk  1 , Ewa Filipowicz, Lukasz Wozniacki, Janusz Grochowski, Leszek Zaleski, Agnieszka Grzejszczak, Agnieszka Perkowska Ptasinska, Lukasz Koperski, Joanna Matuszkiewicz Rowinska
Affiliations
Case Reports

Renal amyloidosis in Whipple disease: a case report

Stanislaw Niemczyk et al. Cases J. .

Abstract

Introduction: 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.

Case presentation: 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.

Conclusions: 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.

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Figures

Figure 1.
Figure 1.
Immunohistochemical staining of biopsy specimen - glomerulus with amyloid-A (AA) deposits.
Figure 2.
Figure 2.
Duodenum mucosa with extended intestinal villi (A) and numerous PAS-positive macrophages (B). Immunohistochemical staining for CD68 - markers of macrophages (C).
Figure 3.
Figure 3.
Computed tomography scans of the abdomen shows enlargement of mesenteric and retroperitoneal lymph nodes with high amount of lipids (exacerbation of Whipple disease) (A). Marked reduction of lymph nodes on one-year follow-up (B).
See this image and copyright information in PMC

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