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Z Gastroenterol 1998 Jul;36(7):553-8
Families with multiple cases of gluten-sensitive enteropathy.
Korponay-Szabo I, Kovacs J, Lorincz M, Torok E, Goracz GY
Heim Pal Children's Hospital, Budapest, Hungary.

Early detection of oligosymptomatic gluten-sensitive enteropathy (GSE) may
contribute to the prevention of late complications, such as malignancy.
Family members of known GSE patients are at higher risk of being affected.
To evaluate the frequency and clinical significance of multiple occurrence,
we routinely offered an antiendomysium antibody (EmA)-based non-invasive
screening to affected families. Among 997 family members of 396 GSE
patients, we identified 89 subjects with EmA positivity and/or severe
jejunal villous atrophy. In 83 cases GSE has been verified, four patients
refused the biopsy and two subjects are under further observation for
latent celiac disease. Prevalence of GSE was 8.5% (80/943) among the
first-degree relatives, with significantly higher values in the siblings
(13.8%) and offsprings (12.0%) than in the parents (4.2%) of the probands
(p < 0.001). In 55 families (13.9% of the families studied) two, in ten
families (2.5%) three, in one family four and in one other family six
members were affected. Combinations of the clinical presentations of index
and screening-detected cases were highly variable, with a high percentage
of silent and atypical forms in the relatives. GSE cases presenting both
with and without dermatitis herpetiformis occurred in 15 families. Six GSE
cases with atypical or mild dermatitis herpetiformis were detected in
consequence of the screening. CONCLUSIONS: EmA-assisted family screening
resulted in the detection of a clinically significant number of additional
GSE patients.

PMID: 9738302, UI: 98410045