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Table 3 Outcome at 2 and >4 years post diagnosis for affected children with and without anti-MDA5 antibodies

From: Anti-MDA5 autoantibodies in juvenile dermatomyositis identify a distinct clinical phenotype: a prospective cohort study

  Two years post diagnosis More than four years post diagnosisa
Anti-MDA5 present Anti-MDA5 absent Anti-MDA5 present Anti-MDA5 absent
Inactive disease 6 (50%)b 30 (22%) 6 (66.6%) 59 (46%)
Active disease 6 (50%)b 109 (78%) 3 (33.3%) 68 (54%)
PRINTO inactive disease 7 (87%)b 44 (43%) 4 (100%) 51 (71%)
PRINTO active disease 1 (13%)b 59 (57%) 0 (0%) 21 (29%)
  1. Results are presented as number of patients (%). aAverage 7.1 years anti-MDA5-positive and 7.9 years in anti-MDA5-negative; bP <0.05. Inactive disease is defined as childhood myositis assessment score (CMAS) >48, absent skin disease and physician global assessment score (PGAS) <1. Paediatric Rheumatology International Trials Organisation (PRINTO) criteria for inactivity is defined as at least three of the following; creatinine kinase (CK) ≤150, CMAS ≥48, manual muscle testing score ≥78 and PGAS ≤0.2. Patients with anti-MDA5 were more likely to have inactive disease at the time points analysed. This was statistically significant at 2 years post diagnosis.