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Whipple’s disease

Whipple’s disease is an uncommon chronic systemic disorder caused by the gram-positive bacillus Tropheryma whipplei (from the Greek trophe meaning nourishment and eryma meaning barrier, which refers to the malabsorption seen in this disease). It can present with polyarthritis, fever, malabsorption, and central nervous system (CNS) manifestations. Because of its nonspecific presentation, the condition is usually diagnosed when it is in an advanced stage. The typical patient is a middle-aged white man presenting with abdominal pain, diarrhea, weight loss, and arthritis. Treatment consists of prolonged antibiotic therapy.

The multisystem manifestations of Whipple’s disease can be remembered using the following mnemonic:

Wasting/weight loss

Diarrhea

Hyperpigmentation (skin)

Interstitial nephritis

Intestinal pain

Skin rashes

Pleurisy

Eye inflammation

Pneumonitis

Arthritis

Lymphadenopathy

Subcutaneous nodules

Encephalopathy

Endocarditis

Steatorrhea

Arthritis associated with Whipple’s disease:

Seronegative migratory oligo- or polyarthritis/arthralgia primarily involving large joints (knees) characterized by brief episodic attacks lasting a few days in a pattern akin to palindromic rheumatism is associated with Whipple’s disease.

Arthritis/arthralgia is the presenting symptom in 60% of 70% of reported cases and is present in 90% of all patients. It does not correlate with intestinal symptoms and can precede the intestinal manifestations in 75% of patients by a mean interval of 6 years. Sacroiliitis is present in 7% and spondylitis in 4% of cases. Joint fluid examination may reveal periodic acid–Shiff (PAS)-positive material; however, joint fluid cultures are negative. Radiographs usually remain unremarkable.


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