whipple's disease in spain: a clinical review of 91 patients diagnosed between 1947 and 2001 enfermedad de whipple en españa: revisión clínica de 91 pacientes diagnosticados durante 1947-2001

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ID: 194719
2010
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Abstract
Background: to determine the epidemiological, clinical, diagnostic and therapeutic characteristics of Whipple's disease in Spain. Patients and method: cases of Whipple's disease reported in the Spanish literature between 1947 and 2001 which meet histological or PCR criteria are reviewed. Results: 91 cases were included, 87.5% of which were male. The maximum incidence was between 40 and 60 years of age (68%). There was no family clustering or susceptibility by profession or surroundings. The most common symptoms and signs were: weight loss (80%), diarrhoea (63%), adenopathies (35%), skin problems (32%), abdominal pain (27%), fever (23%), joint problems (20%) and neurological problems (16%). Arthralgias, diarrhoea and fever were noted prior to diagnosis in 58, 18 and 13% of patients, respectively. Diagnosis was histological in all cases except two, which were diagnosed by PCR. Intestinal biopsy was positive in 94%. Adenopathic biopsies (mesenteric or peripheral) were suggestive in 13% of cases, and treatment was effective in 89%. There were nine relapses, four of which were neurological, although all occurred before the introduction of cotrimoxazole. Conclusions: Whipple's disease is not uncommon, although it requires a high degree of suspicion to be diagnosed in the absence of digestive symptoms. The most common and most sensitive diagnostic method is duodenal biopsy. PCR is beginning to be introduced to confirm the diagnosis and as a therapeutic control. Initial antibiotic treatment with drugs that cross the blood-brain barrier, such as cotrimoxazole and ceftriaxone, is key to achieving a cure and avoiding relapses.
Fundamento: conocer las características epidemiológicas, clínicas, diagnósticas y terapéuticas de la enfermedad de Whipple en España. Pacientes y método: se revisan los casos de enfermedad de Whipple de la literatura española que cumplen criterios histológicos o de PCR desde 1947 hasta 2001. Resultados: se incluyeron 91 casos. El 87,5% eran hombres. La incidencia máxima fue entre los 40 y 60 años de edad (68%). No hubo agregación familiar ni preferencia por profesión o entorno ambiental. Los síntomas y signos más habituales fueron: adelgazamiento (80%), diarrea (63%), adenopatías (35%), cutáneos (32%), dolor abdominal (27%), fiebre (23%), articulares (20%) y neurológicos (16%). Artralgias, diarrea y fiebre se referían previamente al diagnóstico en el 58, 18 y 13% de los enfermos, respectivamente. El diagnóstico fue histológico en todos salvo en dos que se diagnosticaron por PCR. La biopsia intestinal fue positiva en el 94%. Las biopsias de adenopatías (mesentéricas o periféricas) fueron orientadoras en un 13%. El tratamiento fue eficaz en el 89%. Hubo 9 recidivas, 4 neurológicas, estas antes de la introducción del cotrimoxazol. Conclusiones: la enfermedad de Whipple no es tan infrecuente. Se precisa un alto índice de sospecha para diagnosticarla en ausencia de síntomas digestivos. El método diagnóstico más empleado y más sensible es la biopsia duodenal. Se empieza a introducir la técnica de PCR para confirmar el diagnóstico y como control terapéutico. El tratamiento antibiótico inicial con antibióticos que pasan la barrera hematoencefálica como cotrimoxazol y ceftriaxona es determinante para la curación de los pacientes y evitar las recidivas.
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Authors ;E. Ojeda;A. Cosme;J. Lapaza;J. Torrado;I. Arruabarrena;L. Alzate
Journal chemistry of heterocyclic compounds
Year 2010
DOI
DOI not found
URL
http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082010000200006
Keywords
clinical review Gastroenterology

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