Whipple's disease in Spain: a clinical review of 91 patients diagnosed between 1947 and 2001.
BACKGROUND
To determine the epidemiological, clinical, diagnostic and therapeutic characteristics of Whipple s disease in Spain.


METHODS
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.
Ojeda
E
E
Department of Internal Medicine, Donostia Hospital, San Sebastián, Guipúzcoa, Spain.
Cosme
A
A
Lapaza
J
J
Torrado
J
J
Arruabarrena
I
I
Alzate
L
L
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D004269:DNA, Bacterial / Q000032:analysis; D003967:Diarrhea / Q000209:etiology; D004386:Duodenum / Q000382:microbiology / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008198:Lymph Nodes / Q000382:microbiology / Q000473:pathology; D008206:Lymphatic Diseases / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009928:Organ Specificity; D016133:Polymerase Chain Reaction; D012008:Recurrence; D012189:Retrospective Studies; D013030:Spain / Q000453:epidemiology; D016896:Treatment Outcome; D015431:Weight Loss; D008061:Whipple Disease / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology; D055815:Young Adult
D000900:Anti-Bacterial Agents; D004269:DNA, Bacterial