Prevalence and Clinical Implications of Autoantibodies in Juvenile Dermatomyositis

Abstract

Objective ​​This study aimed to characterize the profile of myositis-specific and myositis-associated autoantibodies (MSAs/MAAs) in an Indian cohort of juvenile dermatomyositis (JDM) patients and correlate them with clinical features and outcomes. Methods Forty-three children diagnosed with JDM were enrolled for this observational study. Clinical details (presentation, course, and outcome) were noted in a predesigned proforma. Serum samples were tested for 16 MSAs/MAAs by line immunoassay. MSAs/MAAs were correlated with clinical features and outcome (defined as a complete clinical response [≥6 months' disease inactivity on medication] or complete remission [≥6 months' inactivity off all drugs]). Results Thirty-five subjects (81.4%) had at least 1 MSA/MAA detected. The most common antibodies were anti-NXP2 (n = 13, 30.2%), anti-TIF1γ (n = 10, 23.2%), and anti–MDA-5 (n = 8, 18.6%). No patient had anti-Ku, anti-Pm Scl-100, anti–PL-12, anti-EJ, anti-OJ, or anti-Ro52. Thirty-two patients (74.4%) attained a complete clinical response over a median follow-up duration of 14 months, among which 6 (13.9%) achieved complete remission over a median follow-up duration of 30 months. Anti-TIF1γ was associated with younger age at onset (≤3 years) (odds ratio [OR], 6.25; 95% confidence interval [CI], 1.15–34.12; p = 0.034) and disease flares after attaining complete response (OR, 10.18; 95% CI, 1.64–70.93; p = 0.013). Patients with anti-NXP2 had higher odds of severe muscular weakness (OR, 3.73; 95% CI, 0.95–14.59; p = 0.058) and truncal weakness (OR, 3.89; 95% CI, 0.97–15.64; p = 0.056). One child with anti–MDA-5 positivity had interstitial lung disease. We found no association between the MSA/MAA profile and the achievement of complete clinical response or remission. Conclusions MSAs/MAAs were identified in 81% of children with JDM in our study, which is higher than most other studies. The most frequently observed antibodies displayed a pattern consistent with other studies. Anti-TIF1γ was associated with a younger age at onset and disease flares even after attaining a complete clinical response. Anti-NXP2 had higher odds of severe muscular weakness. These observations suggest consistency in certain phenotypic associations observed across geographic boundaries.