Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study.
BACKGROUND
Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory childhood myopathy of uncertain aetiology. The demographic and clinical presentation of JDM may differ by race and geographic regions. Few studies have described the characteristics of JDM patients from Africa.


METHODS
We conducted a retrospective observational study to determine clinical characteristics and outcomes of patients satisfying the Bohan and Peter criteria for probable JDM seen between 2004 and 2013 in three hospitals in Cape Town, South Africa.


RESULTS
Twenty five cases were identified: 16 female and 9 male; thirteen (52 %) were of indigenous African, eleven (44 %) mixed and one (4 %) European ancestry. The median ages at disease onset and diagnosis were 6.75 (range 2.0-9.7) and 7.9 (range 3.4-9.75) years respectively. Eleven patients had calcinosis while the mortality was 2/25 (8 %). Only 40 % of the patients had clinically inactive disease by PRINTO criteria (modified) at last review. There was no statistically significant difference in racial distribution (p-value = 1), age at disease onset (p-value = 0.87) and disease duration prior to treatment initiation (p-value = 0.75) between patients who had clinically active and inactive disease.


CONCLUSIONS
The demographic characteristics of children with JDM were similar to that from most other regions of the world with female predominance and similar age at onset. Majority of the patients remained with clinically active disease, which put them at risk of further disease complications. Long term follow up and use of appropriate treatment guidelines may be indicated in management of JDM patients for optimum treatment outcomes.
Paediatric Rheumatology Service, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital and the University of Cape Town, Cape Town, South Africa. owino-okongo@uonbi.ac.ke.|Department of Paediatrics and Child Health, University of Nairobi, Nairobi, Kenya. owino-okongo@uonbi.ac.ke.
Lawrence Owino
LO
Okong'o
NHLS, Tygerberg Hospital and Stellenbosch University, Stellenbosch, South Africa.
Monika
M
Esser
Paediatric Neurology Service, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital and the University of Cape Town, Cape Town, South Africa.
Jo
J
Wilmshurst
Paediatric Rheumatology Service, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital and the University of Cape Town, Cape Town, South Africa.
Christiaan
C
Scott
D017668:Age of Onset; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003879:Dermatologic Agents / Q000627:therapeutic use; D003882:Dermatomyositis / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012042:Registries; D012189:Retrospective Studies; D013019:South Africa / Q000453:epidemiology; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D003879:Dermatologic Agents
Africa; JDM; Juvenile dermatomyositis; Outcome