Spondyloarthritis (SpA) is a blanket term encompassing entities such as enthesitis-related arthritis, nonradiographic axial SpA, and ankylosing spondylitis. These diseases share many clinical features, including a predilection for inflammation of the entheses and the sacroiliac joints. The nomenclature is based on the evolution of the classification of the disease and the age of the patient. SpA has a prevalence of approximately 1% of the population of the United States, with 10% to 20% of patients experiencing the onset during childhood. Children with onset of arthritis before age 16 years are classified as having juvenile idiopathic arthritis. Children with enthesitis and/or sacroiliitis are further classified as belonging to the enthesitis-related arthritis subtype of juvenile idiopathic arthritis. The initial manifestations can be subtle and will usually include a peripheral pattern of arthritis and enthesitis. It may take several years for axial disease to develop in children. Except for an association with the human leukocyte antigen (HLA-B27) serotype, there are no laboratory markers for the disease, and the radiographic findings are often negative. A careful clinical evaluation for evidence of inflammation in the entheses and the joints and a search for comorbidities are required. Magnetic resonance imaging facilitates the early detection of sacroiliitis, an important feature that may be clinically silent. Because recent studies indicate that earlier introduction of therapy can help achieve better outcomes, rapid identification and treatment of children with SpA is essential.

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