Growth and Puberty in Juvenile Dermatomyositis: A Longitudinal Cohort Study

Abstract

Objective. To study growth and puberty in a multinational longitudinal prospective cohort of children with juvenile dermatomyositis (DM).Methods. Children from 31 countries who were ages <18 years and had juvenile DM in active phase were studied, and analyses of height, weight, and pubertal development were conducted in those who had follow-up visits during a 2-year period and for whom anthropometric data was available.Results. A total of 196 of 275 children (71%) were included. We found a significant reduction in parent-adjusted height Z score over time in female patients (P < 0.0001) and male patients (P = 0.001), but with catch-up growth at the final study visit. Median body mass index Z score peaked at 6 months (P < 0.0001) and was still significantly above baseline at the final study visit, which was at a median of 26 months after baseline (P = 0.007), with no difference between sexes. Female patients with a disease duration ≥12 months after onset had significantly lower parent-adjusted height Z score (P = 0.002) and no 2-year catch-up growth. At the final study visit, growth failure was seen in 20 of 97 female patients (21%) and in 11 of 73 male patients (15%). Height deflection (∆height Z score less than -0.25/year) was observed in 29 of 116 female patients (25%) and 25 of 80 male patients (31.3%). Delayed puberty was seen in 20 of 55 female patients (36.4%) and in 11 of 31 male patients (35.5%). Children in early pubertal stage at baseline had the highest risk of growth failure.Conclusion. Juvenile DM in the active phase and/or its treatment has a significant impact on growth and puberty in affected children. Children with recent onset of puberty or previous growth failure have the highest risk of delayed pubertal development and further growth retardation.

Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care

“…Functional impairment is usually mild, but reported in up to 41% of patients and can be associated with increased pain and decreased quality of life 16 , 17 , 19 , 20 , 23 – 25 . Children can be affected by impaired growth or delayed puberty, particularly if there is preceding growth failure or if the active phase of disease occurs during early puberty 26 . In a report of adults who had JDM and were surveyed at an average age of 20 years, 59% perceived that their myositis was still active and 65% were still taking immunosuppressive medication 27 .…”

Juvenile dermatomyositis. Where are we now?

“…Delayed puberty was seen in 20/55 (36.4%) female patients and 11/31 (35.5%) of male patients. Children with recent onset of puberty during the active phase of treatment or previous growth failure had the highest risk of delayed pubertal development and further growth retardation (64).…”

Coordinated immune dysregulation in Juvenile Dermatomyositis revealed by single-cell genomics

“…While mortality is low with corticosteroid treatment, long-term patient follow-up studies have reported that 60-70% of patients have cumulative tissue damage (6,7) with the risk of damage increasing almost linearly for each year after diagnosis (8). This finding highlights the importance of early disease intervention, the need to limit the harmful effects of long-term corticosteroid use in children (9), and the need for a personalized approach to disease management to improve upon these outcomes.…”