title: [Whipple's Disease: retrospective analysis of twelve cases and review of the literature].
abstract: BACKGROUND
  Whipple's disease is a rare and chronic bacterial disease with multiorganic involvement. Although there is no valid estimate of its actual prevalence, only about 1,000 cases have been reported.
  
  
  OBJECTIVE
  To describe the demographic, clinical, laboratory, endoscopic and pathologic features, type of treatment used, its duration and response.
  
  
  METHODS
  Based on the duodenal histology, we identified twelve cases of Whipple's disease in the Hospital Centre of Vila Nova de Gaia, between 1997 and 2010. RESULTS. Nine patients (75%) were male and the mean age at diagnosis was 58 years. All patients experienced at least one gastrointestinal and general symptom. Arthralgia were reported in four patients (33%) and occurred on average six years before the onset of gastrointestinal and general symptoms. In 10 patients the initial treatment was trimethoprim-sulfamethoxazole. The initial treatment duration was one year in 8 cases (80%). Seven patients (70%) had a symptomatic resolution between the third and sixth months of antibiotic therapy, as well as an endoscopic and histological improvement. Even in these patients, there was maintenance of periodic acid-Schiff positive macrophages, although in small number and with a more diffuse distribution. Gastrointestinal symptoms predominated in cases of clinical relapse.
  
  
  CONCLUSIONS
  The gastrointestinal and general symptoms as well as the arthralgia were the predominant manifestations. The majority of patients showed clinical and endoscopic improvement in response to treatment with trimethoprim-sulfamethoxazole. However, in these cases periodic acid-Schiff positive macrophages can remain for years. Thus, in the absence of clinical deterioration, the presence of these structures is not indicative ofactive disease.
authors.forename: Iolanda
authors.initials: I
authors.lastname: Ribeiro
authors.forename: Ana
authors.initials: A
authors.lastname: Luis
authors.forename: Sofia
authors.initials: S
authors.lastname: Campelos
authors.forename: Agripino
authors.initials: A
authors.lastname: Oliveira
authors.forename: António
authors.initials: A
authors.lastname: Couceiro
authors.forename: Vitor
authors.initials: V
authors.lastname: Dias
mesh_terms: D000328:Adult; D000368:Aged; D000900:Anti-Bacterial Agents / Q000627:therapeutic use*; D001706:Biopsy; D005260:Female; D006801:Humans; D007413:Intestinal Mucosa / Q000473:pathology; D007421:Intestine, Small / Q000473:pathology; D008264:Macrophages / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D015662:Trimethoprim, Sulfamethoxazole Drug Combination / Q000627:therapeutic use*; D008061:Whipple Disease / Q000188:drug therapy* / Q000473:pathology*
chemical_list: D000900:Anti-Bacterial Agents; D015662:Trimethoprim, Sulfamethoxazole Drug Combination