8.5 Predictors of long-term outcome of Juvenile Dermatomyositis (JDM): a Multicenter, Multinational Study of 490 patients

Background and objective

Little information exists on long-term outcome of JDM. Furthermore, most studies have been conducted in single centres or have involved a few patients. Objective of the study is to identify predictors of a poorer long-term outcome of JDM in a multicenter cohort of patients.

Methods

490 patients with JDM and disease duration > 2 years seen in 27 centers in 5 countries (Italy, UK, Argentina, Brazil, Mexico) after 1980 were identified. Outcomes included muscle weakness (MMT), continued activity (DAS), cumulative damage (MDI), calcinosis, lipodystrophy, functional impairment (CHAQ), and health-related quality of life (HRQL) impairment (CHQ). Predictors included: continent (Europe vs. Latin America), gender, year of onset, onset age, onset type (acute vs. insidious), onset manifestations, severity of muscle/skin manifestations at onset, and course type (monocyclic, polycyclic, chronic continuous).

Results

Table 1 shows significant predictors for each outcome.

Table 1

Conclusion

The chronic continuous course predicted all outcomes, which highlights the critical need for treatments and treatment strategies that have the ability to better control disease activity over time.