Whipple disease is a chronic, multisystem, curable, bacterial infection tha
t usually affects middle-aged men and has a wide range of clinical manifest
ations. The most common symptoms are weight loss and diarrhea, preceded in
three quarters of cases by arthritis for a mean of 6 years. In most patient
s, periodic acid-Schiff staining of proximal small bowel biopsy specimens r
eveals inclusions within the macrophages, corresponding to bacterial struct
ures. However, patients with various manifestations of the disease may have
no gastrointestinal symptoms and negative jejunum biopsy results. Before t
he onset of gastrointestinal symptoms, a strong index of clinical suspicion
is the key to diagnosis. The classic setting is longterm, unexplained, ser
onegative oligoarthritis or polyarthritis with a palindromic or relapsing c
ourse, although chronic destructive polyarthritis and spondyloarthropathy h
ave been repeatedly reported. Identification of the Whipple bacterium, Trop
heryma whippelii, has led to the development of polymerase chain reaction a
s a diagnostic tool in patients in the early stages of the disease or with
atypical Whipple disease. This technique can be used to detect the bacteriu
m in many tissues and fluids, including synovial tissue and fluid. The rece
nt cultivation of the Whipple bacillus should lead to the development of se
rologic tests, further facilitating diagnosis. These recent major advances
may show that the infection is more frequent than previously suspected and
may expand the clinical spectrum of the disease. It may also allow earlier
diagnosis, thereby preventing the development of the severe systemic and so
metimes fatal forms of the disease. Curr Opin Rheumatol 2001, 13:74-79 (C)
2001 Lippincott Williams & Wilkins, Inc.