Autoimmune enteropathy in Swedish children, 1985-2002: a call for strict diagnostic criteria.
OBJECTIVE
To investigate the incidence, prevalence and the long-term outcome of autoimmune enteropathy in Sweden.


METHODS
In 2002 a questionnaire was sent to all paediatric departments in Sweden asking them to report all known cases of this condition from the period 1985-2002.


RESULTS
The response rate was 92%. Five patients were reported and 3 were included in the study. Only one patient fulfilled all the diagnostic criteria and two were considered as possible cases of autoimmune enteropathy. The incidence was 0.06 to 0.12 x 10(-5) and the prevalence was 0.05 to 0.10 x 10(-5) for children aged 0-16 years. At the end of the study period all 3 patients were still alive. Two boys were receiving immunosuppressive treatment and one girl was in remission and functioning well on a gluten-free diet only. One of the patients had adrenalitis. This combination has not been reported previously in autoimmune enteropathy.


CONCLUSIONS
Autoimmune enteropathy in its severe forms is a rare disease in Sweden. None of the patients reported died during the study period. Comparative studies are difficult as different diagnostic criteria are used to diagnose this disease.
Marthinsen
Lars M
LM
Department of Paediatrics, Halmstad Hospital, Halmstad, Sweden.
Scott
Helge
H
Ejderhamn
Jan
J
D000293:Adolescent; D017677:Age Distribution; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000453:epidemiology* / Q000276:immunology; D001707:Biopsy, Needle; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007410:Intestinal Diseases / Q000175:diagnosis* / Q000453:epidemiology* / Q000276:immunology; D007413:Intestinal Mucosa / Q000473:pathology*; D008297:Male; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D017678:Sex Distribution; D013548:Sweden / Q000453:epidemiology