Maladie de Whipple et infections à Tropheryma whipplei. Quand l’interniste doit y penser ? Comment les traiter ?

Abstract

Tropheryma whipplei culture and genome sequencing have, thanks to new tools, rationalized both diagnosis and treatment of Whipple's disease. Whipple's disease involves mainly Caucasian male, approximately 50-years-old, suffering from arthralgia, weight loss and diarrhea. A worsening of clinical manifestations after immunosuppressive therapy is frequently observed while antibiotics prescribed for another infection improves the clinical status. Clinical manifestations may more rarely suggest lymphoma or sarcoidosis. Positive T. whipplei performed on both saliva and stool samples are strongly suggestive of Whipple's disease. The diagnosis is confirmed by positive periodic acid Schiff staining or immunohistochemistry performed on small-bowel biopsies. Localized chronic infections are defined by the absence of histological duodenal involvement. Endocarditis mainly occurs in 60-years-old men with arthralgia, cardiac failure or embolic events. Encephalitis causes mainly cognitive and psychiatric involvement, and sometimes dementia, ataxia and weight gain. Uveitis and arthritis evolve chronically, and are frequently resistant to immunosuppressive treatment. PCR and culture (particularly for cerebrospinal fluid) performed on various tissues and fluids allow the localized infections diagnosis. In classic Whipple's disease, the treatment with doxycycline (200mg/day) and hydroxychloroquine (600mg/day) for a length of 12months followed by a lifetime treatment by doxycycline (200mg/day) should be recommended to avoid reinfection. In localized infections, a treatment with doxycycline (200mg/day) and hydroxychloroquine (600mg/day) is proposed for 12 to 18months followed by a lifetime follow-up.