HLA-B27 typing of all arthritic children helped to identify and focus attention on a subset whose disease was pathogenetically related to and demonstrated clinical features of ankylosing spondylitis and Reiter syndrome, but only rarely fulfilled current diagnostic criteria for those disorders (spondyloarthritis). In contrast to other forms of childhood arthritis, enthesopathy (inflammation at the sites of attachment of ligaments and tendons to bone) was a prominent feature in 75%; a family history of similar arthritis was obtained from 60%; boys were more frequently affected (2:1); urethritis, acute iritis, conjunctivitis, or keratoderma blennorrhagicum occurred at some time in 42%; and the initial attack followed an unexplained febrile illness, known dysentery or urethritis, or severe musculoskeletal trauma in 41%. The arthritis was generally pauciarticular, asymmetric, and primarily in the feet and large joints of the lower extremities. Distinctive radiographic features included periostitis, severe osteopenia, calcaneal erosions, and heel spurs; three of 58 had rapid destruction of a single joint. Only ten patients (all boys) were found to have radiographic sacroiliitis after an average of five years of disease, and only three had the Reiter triad. The lifetime risk of sacroiliitis and spinal ankylosis can only be determined by long-term follow-up of such prospectively identified groups of spondyloarthritic children.