BackgroundAxial involvement in children with enthesitis-related arthritis (ERA) has characteristics that differ from those of peripheral involvement. This study characterized their clinical characteristics and treatment.MethodsPatients with ERA at the Children’s Hospital of Nanjing Medical University between January 2018 and December 2020 were included. The ERA cohort was divided into two based on the presence or absence of axial joint involvement. Demographic characteristics, clinical features, and treatments were described and compared.ResultsIn total, 105 children with ERA were enrolled (axial ERA, n = 57; peripheral ERA, n = 48). The age at disease onset of the axial group tended to be higher (11.93 ± 1.72 vs. 11.09 ± 1.91 years) and the diagnosis delay was bigger in patients with axial ERA (10.26 ± 11.66 months vs. 5.13 ± 7.92 months). The inflammatory marker levels were significantly higher in patients with axial. There were no differences in HLA-B27 positivity between the groups (34 [59.65%] vs. 28 [58.33%], P > 0.05). Hip involvement was more frequent in the axial group (52.63% vs 27.08%; X2 = 7.033). A total of 38 (66.67%) and 10 (20.83%) patients with axial and peripheral ERA, respectively, were treated with biological disease-modifying anti-rheumatic drugs (DMARDs) at diagnosis. The administration of biologics increased gradually in the axial ERA group, peaking at 18 months and decreasing thereafter, whereas that in the peripheral ERA group peaked at 6 months and began to decline thereafter.ConclusionsAxial ERA is a persistent active disease and requires a more aggressive treatment. Classification and early recognition of axial involvement may help with timely diagnosis and appropriate management.