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Whipple disease

  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Last reviewed: 17 Sep 2025
Last updated: 20 Sep 2023

Summary

Definition

History and exam

Key diagnostic factors

  • diarrhea
  • weight loss
  • arthralgia
  • supranuclear ophthalmoplegia
Full details

Other diagnostic factors

  • abdominal pain
  • lymphadenopathy
  • fever
  • steatorrhea
  • anemia
  • skin darkening
  • confusion, memory impairment, altered level of consciousness, or dementia
  • apathy
  • anxiety, depression, hypomania, psychosis, change in personality
  • myoclonic signs
  • seizures
  • nystagmus
  • brisk reflexes, extensor plantar responses, weakness predominating in arm extensors and leg flexors, hypertonia
  • amenorrhea, polydipsia, hyperphagia, decreased libido
  • ataxia
  • headaches
  • oculomasticatory and oculofacioskeletal myorhythmias
  • hemiparesis
  • cranial nerve involvement
  • extrapyramidal movement disorder
  • peripheral neuropathies
Full details

Risk factors

  • age >50 years
  • male sex
  • genetic factors
Full details

Diagnostic tests

1st tests to order

  • CBC
  • serum albumin
  • serum CRP
  • serum ESR
  • upper GI endoscopy
  • Periodic acid-Schiff (PAS) staining of duodenal biopsies
  • PCR
  • Tropheryma whipplei-specific immunohistochemistry
Full details

Emerging tests

  • electron microscopy
  • culture
  • serology

Treatment algorithm

ACUTE

patients without CNS involvement

patients with CNS involvement

Contributors

Authors

Thomas Schneider, MD, PhD

Professor

Medical Department I

Charité - University Medicine Berlin

CBF

Berlin

Germany

Disclosures

TS is an author of a number of references cited in this topic.

Verena Moos, PhD

Scientist

Medical Department I

Charité - University Medicine Berlin

CBF

Berlin

Germany

Disclosures

VM is an author of a number of references cited in this topic.

Peer reviewers

Stephen G. Baum, MD

Professor of Medicine

Department of Microbiology and Immunology

Albert Einstein College of Medicine

Bronx

NY

Disclosures

SGB declares that he has no competing interests.

Chris Huston, MD

Assistant Professor of Medicine

Division of Infectious Diseases

University of Vermont

Burlington

VT

Disclosures

CH declares that he has no competing interests.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Schneider T, Moos V, Loddenkemper C, et al. Whipple's disease: new aspects of pathogenesis and treatment. Lancet Infect Dis. 2008 Mar;8(3):179-90. Abstract

Fenollar F, Puechal X, Raoult D. Whipple's disease. N Engl J Med. 2007 Jan 4;356(1):55-66.

Dobbins WO. Whipple's disease. Springfield, IL: Thomas; 1987.

Louis ED, Lynch T, Kaufmann P, et al. Diagnostic guidelines in central nervous system Whipple's disease. Ann Neurol. 1996 Oct;40(4):561-8. Abstract

Feurle GE, Junga NS, Marth T. Efficacy of ceftriaxone or meropenem as initial therapies in Whipple's disease. Gastroenterology. 2010 Feb;138(2):478-86; quiz 11-2.Full text  Abstract

Feurle GE, Moos V, Bläker H, et al. Intravenous ceftriaxone, followed by 12 or three months of oral treatment with trimethoprim-sulfamethoxazole in Whipple's disease. J Infect. 2013 Mar;66(3):263-70. Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

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