News & Perspective
Drugs & Diseases
CME & Education
Academy
Video
Decision Point
Edition: English

Medscape

English
Deutsch
Español
Français
Português
UKNew

Univadis

Sign Up It's Free!
English Edition

Medscape

Univadis

    X
    Univadis from Medscape

    No Results

      News & Perspective Drugs & Diseases CME & Education Academy Video Decision Point
      Drugs & Diseases > Gastroenterology

      Whipple Disease Clinical Presentation

      Updated: Oct 24, 2019
      • Author: Ingram M Roberts, MD, MBA; Chief Editor: Burt Cagir, MD, FACS  more...
      • Print
      Presentation

      History

      Whipple disease was first described by Dr. George H. Whipple in 1907. [24] The classic presentation of Whipple disease is that of a wasting illness characterized by arthralgias, arthritis, fever, and diarrhea; however, this form is rare. [15, 25] Lymphadenopathy may be present.

      If Whipple disease affects the small intestine, steatorrhea often is present.

      Approximately 90% of patients with Whipple disease present with weight loss, and 70% of patients with Whipple disease complain of either diarrhea or arthralgias. In about three quarters of patients, the joint manifestations are followed by weight loss and diarrhea, with a mean time of 6 years from onset of the joint symptoms to diagnosis of Whipple disease. [26]

      Whipple disease may mimic chronic inflammatory rheumatic disorders and present with atypical manifestations. [27, 28, 29] Case reports exist in which patients with Whipple disease were diagnosed in the context of an inflammatory disease that was refractory to treatment with anti-tumor necrosis factor alpha (anti-TNF-a). [27, 28] The joint manifestations of Whipple disease are nondeforming.

      Occult gastrointestinal (GI) bleeding can be found in 80% of patients of Whipple disease, but frank hematochezia is uncommon.

      Cardiac involvement occurs in approximately 30% of cases.

      Next:

      Physical Examination

      Swelling of the joints may occur, but frankly deforming arthritis is quite rare. [30] Sacroiliitis, pancarpal narrowing, and cervical epiphyseal fusion have been described in selected patients.

      Patients with Whipple disease may have any of the physical findings associated with malabsorption. These findings are nonspecific but include the following:

      • Cachexia

      • Distended abdomen

      • Glossitis

      • Perlèche (angular cheilitis)

      • Chvostek or Trousseau sign (secondary to hypocalcemia)

      • Gingivitis and parafollicular hemorrhages (secondary to vitamin C deficiency)

      • Night blindness (secondary to vitamin A deficiency)

      • Visible peristalsis with borborygmi

      • Hyperpigmentation around the orbital and malar areas of the face (occasionally)

      When the CNS is involved, patients may demonstrate signs of frontal release (as seen with dementia), meningoencephalitis, or ataxia and clonus (if the cerebellum is affected). [31, 32, 33] One review noted that supranuclear ophthalmoplegia and cerebellar ataxia were two of the most common neurologic findings. [34]

      In a retrospective study, neurologic symptoms in 18 patients with Whipple disease and CNS infection (median follow-up, 6 y) included the following: confusion or coma related to meningoencephalitis or status epilepticus (17%); delirium (17%); cognitive impairment (memory loss, attention defects, typical frontal lobe syndrome) (61%); hypersomnia (17%); abnormal movements (eg, myoclonus, choreiform movements, oculomasticatory myorhythmia) (39%); cerebellar ataxia (11%); upper motor neuron symptoms (44%); extrapyramidal symptoms (33%); and ophthalmoplegia, with/without progressive supranuclear palsy (17%). [35]

      Previous
      Differential Diagnoses
       
       
      Media Gallery
      of 0
      Find Us On
      About
      About Medscape Privacy Policy Editorial Policy Cookies Terms of Use Advertising Policy Help Center
      Membership
      Become a Member About You Professional Information Newsletters & Alerts Market Research
      App
      Medscape
      WebMD Network
      Medscape Live Events WebMD MedicineNet eMedicineHealth RxList WebMD Corporate
      Editions
      English Deutsch Español Français Português
      All material on this website is protected by copyright, Copyright © 1994-2022 by WebMD LLC. This website also contains material copyrighted by 3rd parties.