Maladie de Whipple - 02/04/09

[5-0453] - Doi : 10.1016/S1634-6939(09)49785-0

J. Le Scanff ^⁎ , I. Durieu
Service de médecine interne, pavillon 1 k, Centre hospitalier Lyon Sud, 69495 Pierre-Bénite cedex, France

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Articolo archiviato , inizialmente pubblicato nel trattato EMC : AKOS (Traité de Médecine) e sostituito da un altro articolo più recente: cliccare qui per aprirlo

Résumé

La maladie de Whipple (MW) est une maladie rare due à une bacille à Gram positif nommé Tropheryma whipplei de prévalence encore inconnue. Les manifestations cliniques de la MW sont multiples et non spécifiques. Trois grands tableaux cliniques peuvent cependant être individualisés : la MW classique polyviscérale responsable d'un amaigrissement, de diarrhées et d'une malabsorption et de signes articulaires, l'endocardite à hémocultures négatives et les formes neurologiques isolées. Grâce à une démarche rigoureuse basée sur les données cliniques, histologiques (coloration à l'acide périodique de Schiff), immunohistochimiques et la recherche d'acide désoxyribonucléique bactérien par polymerase chain reaction, le diagnostic de maladie de Whipple doit être un diagnostic de certitude et conduire à un traitement antibiotique adapté et efficace.

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Mots clés : Maladie de Whipple, Tropheryma whipplei, Diagnostic de la maladie de Wipple, Traitement de la maladie de Wipple

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Introduction

Épidémiologie^{[Fenollar F., Puéchal X., Raoult D. Whipple's disease N. Engl. J. Med. 2007 ; 356 : 55-66

, Marth T., Raoult D. Whipple's disease Lancet 2003 ; 361 : 239-246

, Raoult D., Birg M., La Scola B., Fournier P.E., Enea M., Lepidi H., e al. Cultivation of the bacillus of Whipple's disease N. Engl. J. Med. 2000 ; 342 : 620-625

, Schneider T., Moos V., Loddenkemper C., Marth T., Fenollar F., Raoult D. Whipple's disease: new aspects of pathogenesis and treatment Lancet Infect. Dis. 2008 ; 8 : 179-190

, Maizel H., Uffin J.M., Dobbins W.O. Whipple's disease: a review of 19 patients from one hospital and review of the literature since 1950 Medicine 1993 ; 72 : 343-355

, Fleming J.L., Wiesner R.H., Shorter R.G. Whipple's disease: clinical, biochemical and histopathologic features and assessment of treatment in 29 patients Maya Clin. Proc. 1988 ; 63 : 539-551

, Durand D.V., Lecomte C., Cathébras P., Rousset H., Godeau P. Whipple disease: clinical review of 52 cases Medicine 1997 ; 76 : 170-178

, Chears W.C., Hargrove M.D., Verner J.V., Smith A.G., Ruffin J.M. Whipple's disease: a review of twelve patients from one service Am. J. Med. 1961 ; 30 : 226-234

, Enzinger F.M., Helwig E.B. Whipple's disease: a review of the literature and report fifteen patients Virchows Arch. Path. Anat. Physiol. Klin. Med. 1963 ; 336 : 238-269

]}

Physiopathologie^{[Fenollar F., Puéchal X., Raoult D. Whipple's disease N. Engl. J. Med. 2007 ; 356 : 55-66

, Marth T., Raoult D. Whipple's disease Lancet 2003 ; 361 : 239-246

, Raoult D., Birg M., La Scola B., Fournier P.E., Enea M., Lepidi H., e al. Cultivation of the bacillus of Whipple's disease N. Engl. J. Med. 2000 ; 342 : 620-625

, Schneider T., Moos V., Loddenkemper C., Marth T., Fenollar F., Raoult D. Whipple's disease: new aspects of pathogenesis and treatment Lancet Infect. Dis. 2008 ; 8 : 179-190

]}

Micro-organisme

Habitat, source et transmission de Tropheryma whipplei

Hôte

Description clinique^{[Fenollar F., Puéchal X., Raoult D. Whipple's disease N. Engl. J. Med. 2007 ; 356 : 55-66

, Marth T., Raoult D. Whipple's disease Lancet 2003 ; 361 : 239-246

, Schneider T., Moos V., Loddenkemper C., Marth T., Fenollar F., Raoult D. Whipple's disease: new aspects of pathogenesis and treatment Lancet Infect. Dis. 2008 ; 8 : 179-190

, Maizel H., Uffin J.M., Dobbins W.O. Whipple's disease: a review of 19 patients from one hospital and review of the literature since 1950 Medicine 1993 ; 72 : 343-355

, Fleming J.L., Wiesner R.H., Shorter R.G. Whipple's disease: clinical, biochemical and histopathologic features and assessment of treatment in 29 patients Maya Clin. Proc. 1988 ; 63 : 539-551

, Durand D.V., Lecomte C., Cathébras P., Rousset H., Godeau P. Whipple disease: clinical review of 52 cases Medicine 1997 ; 76 : 170-178

, Chears W.C., Hargrove M.D., Verner J.V., Smith A.G., Ruffin J.M. Whipple's disease: a review of twelve patients from one service Am. J. Med. 1961 ; 30 : 226-234

, Enzinger F.M., Helwig E.B. Whipple's disease: a review of the literature and report fifteen patients Virchows Arch. Path. Anat. Physiol. Klin. Med. 1963 ; 336 : 238-269

, Gerard A., Sarrot-Reynauld F., Liozon E., Cathebras P., Besson G., Robin C., e al. Neurological presentation of Whipple disease. Report of 12 cases and review of the literature Medicine 2002 ; 81 : 443-457

, Kelly J.J., Weisiger B.B. The arthritis of Whipple's disease Arthritis Rheum. 1963 ; 6 : 615-632

, Aiouaz H., Celard M., Puget M., Vandenesch F., Mercusot A., Fenollar F., e al. Whipple's disease endocarditis: report of 5 cases and review of the literature Rev. Med. Interne 2005 ; 26 : 784-790

, Fenollar F., Lepidi H., Raoult D. Whipple's endocarditis: review of the literature and comparisons with Q fever, Bartonella infection, and blood culture-positive endocarditis Clin. Infect. Dis. 2001 ; 33 : 1309-1316

]}

Symptômes gastro-intestinaux

Atteinte du système nerveux central^{[Gerard A., Sarrot-Reynauld F., Liozon E., Cathebras P., Besson G., Robin C., e al. Neurological presentation of Whipple disease. Report of 12 cases and review of the literature Medicine 2002 ; 81 : 443-457

]}

Atteinte articulaire^{[Kelly J.J., Weisiger B.B. The arthritis of Whipple's disease Arthritis Rheum. 1963 ; 6 : 615-632

]}

Endocardites^{[Aiouaz H., Celard M., Puget M., Vandenesch F., Mercusot A., Fenollar F., e al. Whipple's disease endocarditis: report of 5 cases and review of the literature Rev. Med. Interne 2005 ; 26 : 784-790

, Fenollar F., Lepidi H., Raoult D. Whipple's endocarditis: review of the literature and comparisons with Q fever, Bartonella infection, and blood culture-positive endocarditis Clin. Infect. Dis. 2001 ; 33 : 1309-1316

]}

Autres signes

Signes biologiques

Diagnostic^{[Fenollar F., Puéchal X., Raoult D. Whipple's disease N. Engl. J. Med. 2007 ; 356 : 55-66

, Marth T., Raoult D. Whipple's disease Lancet 2003 ; 361 : 239-246

, Schneider T., Moos V., Loddenkemper C., Marth T., Fenollar F., Raoult D. Whipple's disease: new aspects of pathogenesis and treatment Lancet Infect. Dis. 2008 ; 8 : 179-190

, Le Scanff J., Gaultier J.B., Vital Durand D., Durieu I., Celard M., Benito Y., e al. False positive PCR detections of Tropheryma whipplei in diagnostic samples are rare Rev. Med. Interne 2008 ; [Epub ahead of print].

, Ehrbar H.U., Bauerfeind P., Dulty F., Koelz H.R., Altwegg M. PCR-positive tests for Tropheryma whipplei in patients without Whipple disease Lancet 1999 ; 353 : 2214

, Rolain J.M., Fenollar F., Raoult D. False positive PCR detection of Tropheryma whipplei in the saliva of healthy people BMC Microbiol. 2007 ; 7 : 48

, Müller S.A., Steffen R., Altwegg M., Seebach J.D. Deadly carousel or difficult interpretation of new diagnostic tools for Whipple's disease: case report and review of the literature Infection 2005 ; 33 : 39-42

]}

Diagnostic histopathologique

« Polymerase chain reaction »

Immunohistochimie

Microscopie électronique et culture

Diagnostic différentiel

Évolution et traitement^{[Fenollar F., Puéchal X., Raoult D. Whipple's disease N. Engl. J. Med. 2007 ; 356 : 55-66

, Marth T., Raoult D. Whipple's disease Lancet 2003 ; 361 : 239-246

, Schneider T., Moos V., Loddenkemper C., Marth T., Fenollar F., Raoult D. Whipple's disease: new aspects of pathogenesis and treatment Lancet Infect. Dis. 2008 ; 8 : 179-190

]}

Conclusion

Esportazione

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Avant-propos
Professeur Thomas Papo, Julie Cosserat, Emmanuelle Girard, David Letscher

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