JUVENILE ARTHRITIS IN TURNERS-SYNDROME - A MULTICENTER STUDY

Objective Turner's syndrome (TS) is a disorder associated with charact eristic defects in the X chromosome. Autoimmune conditions such as thy roiditis, inflammatory bowel diseases and diabetes have been described in association with TS. Methods We have studied the association betwe en TS and juvenile arthritis (JA) by using a survey in which 28 pediat ric rheumatology centers (15 in the USA, 10 in Europe, and 3 in Canada ) participated. Results Eighteen cases of TS in a population of approx imately 15,000 JRA patients have been found. Two different patterns of arthritis were present: polyarticular (7) and oligoarticular (11). Ch ildren with polyarticular disease had early onset, seronegative, progr essively deforming arthritis and growth retardation. Those with oligoa rticular arthritis had a benign course and were ANA+ (8/11). The oligo articular children had varying karyotypes whereas almost all of the po lyarthritic patients shared the same 45X0 karyotype (6/7). The light a nd electron microscopic studies of synovium performed in two patients showed chronic inflammation and hyperplasia of the synovial lining cel ls, vascular proliferation and infiltration with lymphocytes, plasma c ells and mononuclear phagocytes. Conclusion Juvenile arthritis is a ne w autoimmune condition associated with Turner's syndrome. The prevalen ce seems to be at least six times greater than would be expected if th e two conditions were only randomly associated. This is the first desc ription of the synovium in Turner's syndrome; no differences from othe r forms of juvenile rheumatoid arthritis were found.