Whipple disease and arthritis

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Whipple disease and arthritis

Xavier Puéchal, MD

Service de Rhumatologie, Centre Hospitalier du Mans, Le Mans, France

CURRENT OPINION IN RHEUMATOLOGY 2001;13:74-79

Whipple disease is a chronic, multisystem, curable, bacterial infection that

usually affects middle-aged men and has a wide range of clinical manifestations.

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 patients, periodic

acid-Schiff staining of proximal small bowel biopsy specimens reveals inclusions

within the macrophages, corresponding to bacterial structures. However, patients

with various manifestations of the disease may have no gastrointestinal symptoms

and negative jejunum biopsy results. Before the onset of gastrointestinal

symptoms, a strong index of clinical suspicion is the key to diagnosis. The

classic setting is long-term, unexplained, seronegative oligoarthritis or

polyarthritis with a palindromic or relapsing course, although chronic

destructive polyarthritis and spondyloarthropathy have been repeatedly reported.

Identification of the Whipple bacterium, Tropheryma whippelii, has led to the

development of polymerase chain reaction as 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 bacterium in many tissues and fluids, including synovial

tissue and fluid. The recent cultivation of the Whipple bacillus should lead to

the development of serologic 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

sometimes fatal forms of the disease.