Manifestations articulaires de la maladie de Whipple

Abstract

Whipple disease is a chronic, curable, systemic infection caused by Tropheryma whipplei, which mostly affects middle-aged men and places the rheumatologist in the front line. The historical form typically associates weight loss and diarrhea preceded in 3/4 of the cases by a stereotypical articular involvement. This one evolves on average for 6 years before the diagnosis, with oligoarthritis or seronegative chronic polyarthritis affecting large joints but which is intermittent, at least at the beginning. A chronic septic destructive polyarthritis can then arise in the absence of diagnosis. A presentation with spondyloarthritis is also possible. Some cases of infectious spondylodiscitis have been described and even exceptional cases of hypertrophic osteo-arthropathy. In most of the patients with classic Whipple disease, duodeno-jejunal biopsies provide evidence of mucosa infiltration by foamy macrophages stained with periodic acid-Schiff, which correspond to bacterial structures. Nevertheless, the gastrointestinal involvement can be absent clinically and even sometimes in histology or even with PCR tests. In spite of the absence of digestive involvement, the rheumatologist should raise the diagnosis of Whipple disease in case of unexplained intermittent arthritis in a middle-aged man. Polymerase chain reaction (PCR) tests for T. whipplei from saliva, stools and synovial liquid are now included among the initial diagnostic tests, even if the bacterium is not frequently involved during oligoarthritis or unexplained seronegative polyarthritis in men. The PCR test facilitates the early diagnosis of the disease before the appearance of the severe systemic complications, which are still sometimes fatal.