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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 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.

Author information

Correspondence to C Ferrari.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Keywords

  • Disease Duration
  • Functional Impairment
  • Muscle Weakness
  • Single Centre
  • Dermatomyositis