Whipple’s disease

Abstract

The primary aim in treating Whipple’s disease is the eradication of Tropheryma whippelii, a bacillus that infects the small bowel, brain, synovial membranes, heart valves, and many other tissues in patients afflicted with this disease. Antibiotic therapy tends to be prolonged, 1 year or more in duration. If symptoms are primarily gastrointestinal, trimethoprim-sulfamethoxazole is the drug of choice. For patients who are allergic to or intolerant of this medication, a third-generation cephalosporin is used. If presentation also suggests central nervous system involvement, an initial 2-week course of intravenous ceftriaxone, or alternatively a combination of intramuscular benzathine penicillin and streptomycin, should be prescribed prior to a yearlong course with an oral antibiotic. In endocarditis, duration of parental treatment is prolonged to a 6-week course, followed by an oral antibiotic. In rare cases of intolerance to trimethoprim-sulfamethoxazole and cephalosporins, oral combination with tetracycline and rifampicin is reasonable. However, close follow-up and monitoring of T. whippelii eradication should be performed with a polymerase chain reaction (PCR)-based test if tetracycline is used. Knowing whether T. whippelii has been eradicated is also important in a relapse, especially one that involves the central nervous system. Surgery may be performed to remove infected heart valves and joints in rare complicated cases. The role of immunomodulatory treatment is promising but is limited by experience. Nutritional support is a critical part of treatment, especially in patients with diarrhea and weight loss due to malabsorption.