Until recently, the term juvenile rheumatoid arthritis was used, inappropriately, to describe all forms of chronic childhood arthritis. The use of careful clinical evaluation, autoantibody testing, and HLA typing has revealed a heterogeneous group of diseases in which only a small proportion of affected children truly have the same disease as adults with rheumatoid arthritis. Juvenile spondyloarthropathy typically begins in late childhood or adolescence, most often in boys, with lower extremity oligoarthropathy and enthesopathy.
Spinal symptoms are rare initially but often appear years later.
Bony ankylosis of the tarsal bones has been described in some of these patients. Acute anterior uveitis is not uncommon. Such patients are seronegative for rheumatoid factor and antinuclear antibodies but are positive for HLA-B27. Less often, a chronic polyarthritis may be the presenting picture, with prominent cervical spine fusion rather than lower spine involvement. Still's disease characterized by high spiking fever, evanescent rash, hepatosplenomegaly, lymphadenopathy, and polyarthritis that is seronegative and HLA-B27 negative.