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  • Open Access

Inflammatory myositis in the pediatric rheumatology clinical practice – a case series

Pediatric Rheumatology20119 (Suppl 1) :P59

https://doi.org/10.1186/1546-0096-9-S1-P59

  • Published:

Keywords

  • Lipodystrophy
  • Calcinosis
  • Inflammatory Myopathy
  • Juvenile Dermatomyositis
  • Muscular Function

Background

Inflammatory myopathies (IM) in children comprise a heterogeneous group of disorders, the most common being juvenile dermatomyositis and to a lesser degree juvenile polymyositis.

Aim

To assess the clinical characteristics and treatment response of a cohort of IM patients.

Methods

Clinical chart review of clinical, laboratory and treatment related parameters of IM patients treated at 2 referral centers for the last 12 years.

Outcome measures included disease remission and muscular function.

Results

17 IM patients (12F/5M, median age at diagnosis-8years(2-16years)) were followed for a mean of 6,3years (1-12years): 3 were labeled as polymyositis and 14 as juvenile dermatomyositis.

Positive diagnostic criteria: typical skin lesions-14/17, proximal muscle weakness-15/17, elevated muscle enzymes-16/17, EMG-10/10, muscle biopsy-9/9.

Frequent presenting symptoms included: proximal muscle weakness-16/17, skin lesions-11/17, lethargy-8/17, fever-7/17. During follow-up, patients presented with: muscle weakness (17/17), skin lesions (14/17), lipodystrophy (2/17), arthralgia (6/17), arthritis(4/17), vasculitis (4/17), gastro-intestinal vasculitis (1/17), restrictive pulmonary disease (1/17), calcinosis (4/17).

Laboratory: ESR was raised in 6/17, muscle enzymes in 17/17 (CK-16/17, median-1149; LDH 16/17, median-875; aldolase-7/17); Positive auto-antibodies: ANA-11/17, SSA(Ro-52)-2/17; 10/17 underwent muscle biopsy and 9/17 EMG.

Therapeutic regimens included more commonly steroids, methotrexate (17/17) and CyclosporinA (16/17).

Regarding disease activity, 9/17 patients have inactive disease, 6 of which are in remission without treatment; 6/17 have permanent loss of muscular function.

Conclusion

IM are potentially severe, incapacitating diseases. All patients with polymyositis in this series have loss of muscular function, contrasting with 3/14 of JDM patients.

About half of this cohort is asymptomatic and it should be stressed that early diagnosis and aggressive treatment are important prognostic factors.

Authors’ Affiliations

(1)
Department of Pediatrics, Centro Hospitalar Oeste Norte, Portugal
(2)
Department of Pediatrics, Hospital de Dona Estefânia, Portugal
(3)
Pediatric Rheumatology Clinic, Instituto Português de Reumatologia, Lisbon, Portugal

Copyright

© Gomes et al; licensee BioMed Central Ltd. 2011

This article is published under license to BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://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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