[Whipple disease. A rare systemic disorder with multiple manifestations].

Mb. Whipple is a rare systemic disorder with multiple manifestations. We present a case-story demonstrating the typical course: migrating, non-deforming arthralgies are years later followed by diarrhoea, loss of weight, fatigue and pronounced biochemical disturbances. Intestinal biopsy shows numerous PAS-positive, diastaseresistent macrophages, and antibiotic treatment is initiated. After a somewhat prolonged course, complicated with Giardiasis and endocarditis, the patient recovers. Four months after the cessation of antibiotic treatment, however, the patient shows clinical signs of relapse, and treatment is restarted. The etiological agent has recently been identified as a gram-positive actinomycete called Tropheryma Whippleii. There are some, but not unequivocal, signs of a cellular immunodeficiency, perhaps predestinating certain patients to the disease. The course is usually favourable, when treated with relevant antibiotics. Relapse is not uncommon, and is very problematic when the CNS is involved. Therefore, a combination treatment with good penetration of the blood-brain barrier is recommended--e.g. two weeks treatment with parenterally administered streptomycin and benzylpenicillin followed by sulphamethoxazole-trimethoprim orally for one year.