• J-C Lagier, F Fenollar, and D Raoult.
• CNRS 7278, IRD 198, Inserm 1095, unité de recherche sur les maladies infectieuses et tropicales emergentes (URMITE) UM63, Aix-Marseille université, 13005 Marseille, France; Pôle infectieux, CHU Timone, AP-HM, 13005 Marseille, France.
• Rev Med Interne. 2014 Dec 1; 35 (12): 801-7.

AbstractTropheryma 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 (600 mg/day) for a length of 12 months followed by a lifetime treatment by doxycycline (200 mg/day) should be recommended to avoid reinfection. In localized infections, a treatment with doxycycline (200mg/day) and hydroxychloroquine (600 mg/day) is proposed for 12 to 18 months followed by a lifetime follow-up.Copyright © 2014 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

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