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.
Subject(s)
Whipple Disease/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Anti-Bacterial Agents/therapeutic use , Biopsy , Child , Child, Preschool , DNA, Bacterial/analysis , Diarrhea/etiology , Duodenum/microbiology , Duodenum/pathology , Female , Humans , Infant , Lymph Nodes/microbiology , Lymph Nodes/pathology , Lymphatic Diseases/etiology , Male , Middle Aged , Organ Specificity , Polymerase Chain Reaction , Recurrence , Retrospective Studies , Spain/epidemiology , Treatment Outcome , Weight Loss , Whipple Disease/diagnosis , Whipple Disease/drug therapy , Whipple Disease/pathology , Young AdultABSTRACT
Fundamento: conocer las características epidemiológicas, clínicas,diagnósticas y terapéuticas de la enfermedad de Whipple enEspaña.Pacientes y método: se revisan los casos de enfermedad deWhipple de la literatura española que cumplen criterios histológicoso 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 entornoambiental. Los síntomas y signos más habituales fueron: adelgazamiento(80%), diarrea (63%), adenopatías (35%), cutáneos(32%), dolor abdominal (27%), fiebre (23%), articulares (20%) yneurológicos (16%). Artralgias, diarrea y fiebre se referían previamenteal diagnóstico en el 58, 18 y 13% de los enfermos, respectivamente.El diagnóstico fue histológico en todos salvo en dosque se diagnosticaron por PCR. La biopsia intestinal fue positivaen el 94%. Las biopsias de adenopatías (mesentéricas o periféricas)fueron orientadoras en un 13%. El tratamiento fue eficaz enel 89%. Hubo 9 recidivas, 4 neurológicas, estas antes de la introduccióndel cotrimoxazol.Conclusiones: la enfermedad de Whipple no es tan infrecuente.Se precisa un alto índice de sospecha para diagnosticarlaen ausencia de síntomas digestivos. El método diagnóstico másempleado y más sensible es la biopsia duodenal. Se empieza a introducirla técnica de PCR para confirmar el diagnóstico y comocontrol terapéutico. El tratamiento antibiótico inicial con antibióticosque pasan la barrera hematoencefálica como cotrimoxazol yceftriaxona es determinante para la curación de los pacientes yevitar las recidivas (AU)
Background: to determine the epidemiological, clinical, diagnosticand therapeutic characteristics of Whipples disease in Spain.Patients and method: cases of Whipples disease reportedin the Spanish literature between 1947 and 2001 which meet histologicalor 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 professionor surroundings. The most common symptoms and signswere: weight loss (80%), diarrhoea (63%), adenopathies (35%),skin problems (32%), abdominal pain (27%), fever (23%), jointproblems (20%) and neurological problems (16%). Arthralgias, diarrhoeaand fever were noted prior to diagnosis in 58, 18 and13% of patients, respectively. Diagnosis was histological in all casesexcept two, which were diagnosed by PCR. Intestinal biopsywas positive in 94%. Adenopathic biopsies (mesenteric or peripheral)were suggestive in 13% of cases, and treatment was effectivein 89%. There were nine relapses, four of which were neurological,although all occurred before the introduction of cotrimoxazole.Conclusions: Whipples disease is not uncommon, althoughit requires a high degree of suspicion to be diagnosed in the absenceof digestive symptoms. The most common and most sensitivediagnostic method is duodenal biopsy. PCR is beginning to beintroduced to confirm the diagnosis and as a therapeutic control.Initial antibiotic treatment with drugs that cross the blood-brainbarrier, such as cotrimoxazole and ceftriaxone, is key to achievinga cure and avoiding relapses (AU)