ObjectiveTo describe the course and the 4-year outcome of juvenile spondyloarthritis (jSpA).MethodsPatients with a diagnosis of jSpA and an age at onset ≤16 years were included in the German Spondyloarthritis Inception cohort (GESPIC) and followed up prospectively for 4 years.Results118 patients (73% men, 66% HLA-B27 positive, mean age 13.5 years, mean symptom duration 2.2 years) were enrolled in 2 study centres: 52% of patients with jSpA were captured by the enthesitis-related arthritis subgroup of the International League of Associations for Rheumatology classification criteria. At inclusion, the majority of patients had active peripheral arthritis (75.4%), followed by inflammatory back pain (IBP) (19.5%) and enthesitis (16.1%). There was a significant improvement in clinical manifestations and in patient-reported outcomes over time. During the 4-year follow-up, 85% of the patients had at least 1 period of remission on drug ≥6 months, and 46% of the patients achieved remission ≥12 months without medication, of whom 68% kept this status and 32% worsened. At the end of 4 years of observation, 23% of the patients were in remission without medication, but 57% still suffered from active disease. Patients with peripheral arthritis had a likelihood of 29% for having peripheral arthritis after 4 years, whereas the likelihood of IBP persistence was 53% for those with IBP at enrolment.ConclusionsAlthough 1 quarter of patients with jSpA achieved remission off medication after 4 years, the likelihood of having recurrent or persistent disease into adulthood is substantial, particularly for jSpA with IBP.Trial registration numberNCT 01277419.