Juvenile-onset chronic arthritis: Clinical and roentgenographic features of a unique HLA-B27 subset

Abstract

Previous studies of HLA-B27 in children with juvenile-onset chronic arthritis have shown an association in boys with pauciarticular disease which may progress to ankylosing spondylitis. The spectrum of B27-associated disease, however, especially as it relates to arthritis persisting into adulthood, has not been fully clarified. Thus, clinical, roentgenographic, serologic and immunogenetic studies were performed in 54 patients (39 adults and 15 children) with persistent juvenile-onset chronic arthritis. All had had the onset of disease in peripheral or axial joints, and patients with typical febrile Still's disease were excluded. Thirty-five patients (65 percent) were seronegative for both rheumatoid factor and antinuclear antibodies. HLA-B27 (B27) was present in 20 of these 35 seronegative patients (54 percent) (p < 0.005). Four of the 20 (20 percent) had an axial onset and typical ankylosing spondylitis. Peripheral arthritis, however, dominated the course of the remaining 16 patients who had the B27 antigen. Polyarticular disease (nine patients) with “rheumatoid-like” hand deformities (eight patients) occurred primarily in female patients. In addition, sacroiliitis or spondylitis occurred in 14 and acute iritis in five. Cervical apophyseal joint fusion, especially at the level of the C2-C3, a lesion previously ascribed to juvenile rheumatoid disease, was common in seronegative patients and correlated with the presence of B27 (p < 0.01) and sacroiliitis (p < 0.0005). These data suggest that the presence of B27 may mark a group of young women with a dominant peripheral and cervical spondylitic syndrome, often beginning in childhood, which mimics rheumatoid arthritis. Determining the absence or presence of the B27 antigen may be a useful test in the proper diagnosis and classification of such patients.