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Division of
Rheumatology, Department of Internal Medicine, Istanbul School of
Medicine, University of Istanbul, Istanbul, Turkey
Correspondence to: Dr Ahmet Gül, Division of Rheumatology, Department of Internal Medicine, Istanbul School of Medicine, Capa, 34390 Istanbul, Turkey E-mail: agul{at}istanbul.edu.tr
Accepted for publication 9 February 2000
OBJECTIVE
Familial
aggregation of Behçet's disease has been reported previously.
The current study aimed at investigating the sibling recurrence risk
ratio (
s) for Behçet's disease, which is of value in the
estimation of the magnitude of genetic factors in the pathogenesis of
Behçet's disease.
METHODS
170
consecutive unrelated index cases (98 male, 72 female) were interviewed
with a detailed questionnaire to ascertain their family trees and the
manifestations of Behçet's disease in their relatives. Subsequently,
the immediately older sibling, or if an older sibling was not
available, the immediately younger sibling, was selected as the second
sibling for the evaluation. These siblings were contacted by telephone,
and all subjects with recurrent oral ulcers were invited for examination.
RESULTS
31 of the 170 index cases had 51 relatives fulfilling the International Study Group
criteria. Among 166 second siblings, seven had Behçet's disease (six
male, one female) and 22 siblings (eight male, 14 female) with
recurrent oral ulcers were identified. Sibling recurrence rate
defined
as the ratio of the risk of being affected among the siblings of
patients and the risk of being affected in the general population
was
found to be 4.2%, which gives a
s value for Behçet's disease of
between 11.4 and 52.5 in Turkey.
CONCLUSIONS
A high
s value supports a strong genetic background for Behçet's disease
which will be helpful in designing genetic linkage studies.
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