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Arch Dis Child 98:A103 doi:10.1136/archdischild-2013-304107.243
  • British Society for Paediatric and Adolescent Rheumatology

G231 Mortality Patterns in Children with Juvenile Dermatomyositis: Two Decades of Experience from a Single Tertiary Care Centre

  1. R Manojkumar2
  1. 1Department of Paediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India
  2. 2Department of Cardiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Abstract

Introduction Juvenile dermatomyositis (JDM), is a chronic inflammatory disease characterised by proximal muscle weakness and pathognomonic rash. Although there have been significant advances in management of JDM, the condition still has significant morbidity and mortality associated with it. Complications like breakthrough infections, gastrointestinal tract vasculitis, interstitial pneumonitis and myocarditis pose significant challenges to treating physicians. We report our experience of mortality patterns in this condition over the last 2 decades. There is paucity of literature on this aspect of JDM.

Patients and methods Case files of 72 patients diagnosed to have JDM at a single centre during the period January 1993-November 2012 were reviewed. The total follow-up amounts to 256 patient years. The diagnosis of JDM was based on Bohan and Peter criteria. Standard treatment protocols (based on glucocorticoids and methotrexate) were used in management and the senior author was involved in the care of all these children. We recorded 7 deaths amongst these patients over the last 2 decades.

Results Amongst the 7 children who died, there were 5 boys and 2 girls. The mean age at diagnosis was 10.2 years (range 7–12 years) and duration of treatment ranged from 1 month to 9 years. The causes of death included: upper gastrointestinal vasculitis (3 patients) – of the latter, 1 had perforation peritonitis and 1 had massive upper gastrointestinal bleeding leading to sudden death even before medical aid could be provided; interstitial lung disease with secondary infection (2 patients); bronchiolitis obliterans with organising pneumonia and pneumothorax (1 patient); progressive muscle disease with cardiomyopathy refractory to immunosuppesive therapy (1 patient) – this child died after 9 years of follow-up. Intercurrent and breakthrough infections were recorded amongst 5 of these 7 children.

Conclusion JDM is associated with significant mortality – 13.8% at our centre over the last 2 decades. Gastrointestinal and pulmonary involvement remain the most common causes of death in this condition. Infections are also an important contributory cause. In our experience, delays in diagnosis and referral contribute significantly to mortality in this condition.