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Fever, Diarrhea, and Weight Loss in a Man With Inflammatory Polyarthritis

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 61-year-old white man with seronegative rheumatoid arthritis presented with hand and wrist pain. Over 2 years, he was prescribed various therapies including prednisone, methotrexate, tumor necrosis factor inhibitors, abatacept, and anakinra; he experienced intermittent improvement in his joint pain but had persistently elevated levels of inflammatory markers. He continued to experience asymmetric small- and large-joint polyarthralgias and later developed intermittent fevers up to (temperatures up to 38.3°C [101°F]) along with a 50-lb (22.7-kg) unintentional weight loss over the course of a few months.

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Whipple disease

A. Begin antimicrobial therapy

The keys to the correct diagnosis are the endoscopic and histologic abnormalities. The endoscopic duodenal image (Figure, panel A) highlights the whitish plaques characteristic of Whipple disease. Biopsies revealed small-bowel mucosa with foamy macrophages (Figure, panel B) and periodic acid–Schiff–positive macrophages in the lamina propria (Figure, panel C), all consistent with Whipple disease. Results of polymerase chain reaction assay for Tropheryma whipplei were also positive. Intravenous immunoglobulin therapy (choice B) is appropriate for common variable immune deficiency, which manifests as chronic, opportunistic infections and absence of plasma cells on small bowel biopsy, neither of which occurred in this patient. While the patient manifested multiple symptoms, genetic evaluation for hematologic malignancy (choice C) is incorrect given no evidence of malignancy on laboratory, imaging, and tissue evaluations. Referral to a nutritionist for education on a gluten-free diet (choice D) is incorrect given the absence of positive results from celiac serologic testing (which has 99% sensitivity) and absence of typical changes on biopsy such as villous blunting and intraepithelial lymphocytosis.1

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Article Information

Corresponding Author: Arjun R. Sondhi, MD, Division of Gastroenterology, University of Michigan, 1500 E Medical Center Dr, Ann Arbor, MI 48109 (sondhia@med.umich.edu).

Published Online: January 6, 2020. doi:10.1001/jama.2019.18724

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for providing permission to share his information.

References
1.
Husby  S, Murray  JA, Katzka  DA.  AGA Clinical Practice Update on diagnosis and monitoring of celiac disease—changing utility of serology and histologic measures: expert review.  Gastroenterology. 2019;156(4):885-889. doi:10.1053/j.gastro.2018.12.010PubMedGoogle ScholarCrossref
2.
Whipple  GH.  A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues.  Bull Johns Hopkins Hosp. 1907;18:382-391.Google Scholar
3.
Paulley  JW.  A case of Whipple’s disease (intestinal lipodystrophy).  Gastroenterology. 1952;22(1):128-133. doi:10.1016/S0016-5085(19)36367-XPubMedGoogle ScholarCrossref
4.
Raoult  D, Birg  ML, La Scola  B,  et al.  Cultivation of the bacillus of Whipple’s disease.  N Engl J Med. 2000;342(9):620-625. doi:10.1056/NEJM200003023420903PubMedGoogle ScholarCrossref
5.
Schneider  T, Moos  V, Loddenkemper  C, Marth  T, Fenollar  F, Raoult  D.  Whipple’s disease: new aspects of pathogenesis and treatment.  Lancet Infect Dis. 2008;8(3):179-190. doi:10.1016/S1473-3099(08)70042-2PubMedGoogle ScholarCrossref
6.
Geissdörfer  W, Moos  V, Moter  A,  et al.  High frequency of Tropheryma whipplei in culture-negative endocarditis.  J Clin Microbiol. 2012;50(2):216-222. doi:10.1128/JCM.05531-11PubMedGoogle ScholarCrossref
7.
Dobbins  WO  III.  Whipple’s Disease. Springfield, IL: Charles C. Thomas Publisher; 1987.
8.
Marth  T.  Systematic review: Whipple’s disease (Tropheryma whipplei infection) and its unmasking by tumour necrosis factor inhibitors.  Aliment Pharmacol Ther. 2015;41(8):709-724. doi:10.1111/apt.13140PubMedGoogle ScholarCrossref
9.
Fenollar  F, Puéchal  X, Raoult  D.  Whipple’s disease.  N Engl J Med. 2007;356(1):55-66. doi:10.1056/NEJMra062477PubMedGoogle ScholarCrossref
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