In a study of 23 patients with juvenile dermatomyositis (JDM), multivariate analysis of 26 histopathological parameters identified two distinct subgroups, with one cluster of patients (n = 11) having more severe disease and a higher frequency of antibodies against nuclear matrix protein 2 (NXP2) than the other cluster (n = 12). Patients with anti-NXP2 antibodies also required more aggressive treatment and had a lower rate of remission than patients with other or no myositis-specific antibodies.