Whipple's disease

Whipple's disease (intestinal lipodystrophy) is a systemic chronic infectious disease caused by the gram-positive rod-shaped bacterium Tropheryma whipplei. The small intestine is most affected, especially the duodenum and jejunum. Other organs are the brain, eyes, heart, lungs, spleen, colon, joints and then the whole organism.

Etiopatogenesis

 * The disease is relatively rare.
 * occurs mainly in white men between the ages of 40 and 60

The exact role of Tropheryma whipplei in the pathogenesis of the disease is unknown. A genetic predisposition to the disease is assumed.

The clinical picture [ modify | edit source ]
Pathological changes in the intestine are manifested by malabsorption, the main cause of which is probably blockage of lymphatic tissue :


 * abdominal pain ;
 * diarrhea ;
 * steatorrea ;
 * weight loss.

Most patients have:


 * remittent fever
 * migratory arthralgia or arthritis of large joints.

In the advanced stage of the disease, the nervous system and the heart are particularly affected. In the terminal stage app cachexia, memory loss and hearing impaired visus, and other signs of organic disease may appear

Diagnosis
From a small bowel biopsy;


 * PCR ;
 * PAS-reaction - detection of sickle-shaped substances in macrophages accumulated in the villi of the intestinal mucosa;
 * bacterial culture

The bacteria has Gram-positive structure of the cell wall, but Gram stain is almost non-staining.

Treatment
The prognosis of the disease is uncertain and untreated is life threatening. The drug of choice is the cephalosporin and antibiotic ceftriaxone, administered intravenously over 14 days. It can be replaced by a combination of penicillin + streptomycin. This is followed by long-term treatment with co-trimoxazole or doxycycline. The therapeutic effect should appear within 1-3 weeks of therapy. Premature termination of therapy is dangerous, which leads to frequent relapses of the disease.