Whipple's disease

Whipple's disease is a systemic bacterial infection and the common though n ot invariable manifestations are diarrhoea, weight loss, abdominal pain, an d arthralgia. Arthritis or arthralgia may be the only presenting symptom, p redating other manifestations by years. Virtually all organs in the body ma y be affected, with protean clinical manifestations. Various immunological abnormalities, some of which may be epiphenomena, are described. The causat ive organism is Tropheryma whippelii. The disease is uncommon though lethal if not treated. Recent data suggest t he disease occurs in an older age group than previously described. The char acteristic histopathological features are found most often in the small int estine. These are variable villous atrophy and distension of the normal vil lous architecture by an infiltrate of foamy macrophages with a coarsely gra nular cytoplasm, which stain a brilliant magenta colour with PAS. These pat hognomonic PAS positive macrophages may also be present in the peripheral a nd mesenteric lymph nodes and various other organs. The histological differ ential diagnoses include histoplasmosis and Mycobacterium aviumintercellula re complex. The clinical diagnosis of Whipple's disease may be elusive, especially if g astrointestinal symptoms are not present. A unique sign of CNS involvement, if present, is oculofacial-skeletal myorhythmia or oculomasticatory myorhy thmia, both diagnostic of Whipple's disease. A small bowel biopsy is often diagnostic, though in about 30% of patients no abnormality is present. In p atients with only CNS involvement, a stereotactic brain biopsy can be done under local anaesthetic. A recent important diagnostic test is polymerase c hain reaction of the 16S ribosomal RNA of Tropheryma whippelii. Whipple's disease is potentially fatal but responds dramatically to antibio tic treatment. In this review the current recommended treatments are presen ted. The response to treatment should be monitored closely, as relapses are common. CNS involvement requires more vigorous treatment because there is a high rate of recurrence after apparently successful treatment.