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  • Oral presentation
  • Open Access

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

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Pediatric Rheumatology20086 (Suppl 1) :S19

https://doi.org/10.1186/1546-0096-6-S1-S19

  • Published:

Keywords

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

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

(*At onset)

M. weak'ss

Cont'd activity

Damage

Funct. Impairm'nt

HRQL Impairm'nt

Female sex

+

-

-

+

-

Onset after 2000

+

-

-

-

-

Onset age < 5 yrs

-

-

-

-

-

Onset type

-

+

-

-

+

Dysphonia*

+

-

-

-

-

Mm/skin severity*

-

-

-

-

+

Chronic Course

+

+

+

+

+

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.

Authors’ Affiliations

(1)
Italian Pediatric Rheumatology Study Group, Italy, Italy
(2)
Great Hormond Street Hospital, London, UK
(3)
Hospital General de Ninos Pedro de Elizalde, Buenos Aires, Argentina
(4)
Hospital Garrahan, Buenos Aires, Argentina
(5)
Hospital Pequeno Principe, Curitiba, Brazil
(6)
Hospital da Universidade, Riberao Preto, Brazil
(7)
Universidade Federal do Rio de Janeiro, Rio da Janeiro, Brazil
(8)
Hospital das Clínicas UNESP, Botucatu, Brazil
(9)
Hospital das Clínicas, Sao Paulo, Brazil
(10)
CMN Siglo XXI, Mexico City, Mexico
(11)
Hospital General de México, Mexico City, Mexico
(12)
CMN La Raza, Mexico City, Mexico

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