To describe the demographics and clinical disease in affected sibpairs (ASPs) with juvenile rheumatoid arthritis (JRA), and to compare JRA as it occurs in ASPs with that from non-ASP JRA populations described in the literature.
A rare disease research registry was established with a focus on JRA ASPs to facilitate accrual of patients for genetic, epidemiologic, and clinical studies.
Physicians likely to care for patients with JRA were made aware of the registry and its goals by a variety of methods and asked to refer patients for entry.
To date, 71 ASPs have been registered and complete information has been obtained.
These affected sibs differed in age by a mean of 4.1 years (SD 3.4) and in age at disease onset by 2.8 years (SD 3.0).
The actual time difference between onset in sib 1 versus sib 2 averaged 4.4 years (SD 4.2).
Sixty-three percent of the sibpairs were concordant for sex, and 76% for JRA onset type.
Onset type within sibpairs did not appear to be random, based upon comparisons with non-ASP populations.
Greater than expected concordance was seen among those with pauciarticular-onset and polyarticular-onset JRA.
Seventy-nine percent of the pairs were concordant for course type.
Seven sets of twins were included (~10% of the total), all were concordant for onset and course type (6 sets with pauciarticular, 1 set with polyarticular), and disease onset was separated by a mean of only 3.3 months. (...)
Mots-clés Pascal : Arthrite chronique juvénile, Enfant, Homme, Etude familiale, Epidémiologie, Génétique, Symptomatologie, Etude comparative, Revue bibliographique, Déterminisme génétique, Chronique, Système ostéoarticulaire pathologie, Rhumatisme inflammatoire
Mots-clés Pascal anglais : Juvenile rheumatoid arthritis, Child, Human, Family study, Epidemiology, Genetics, Symptomatology, Comparative study, Bibliographic review, Genetic determinism, Chronic, Diseases of the osteoarticular system, Inflammatory joint disease
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 98-0018127
Code Inist : 002B15D. Création : 17/04/1998.