Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study
Date
2016-11-11
Journal Title
Journal ISSN
Volume Title
Publisher
BioMed Central
Abstract
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.
Conclusion: 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.
Description
CITATION: Okongo, L. O., et al. 2016. Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. Pediatric Rheumatology, 14:60, doi:10.1186/s12969-016-0118-0.
The original publication is available at http://ped-rheum.biomedcentral.com
The original publication is available at http://ped-rheum.biomedcentral.com
Keywords
Juvenile dermatomyositis
Citation
Okongo, L. O., et al. 2016. Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. Pediatric Rheumatology, 14:60, doi:10.1186/s12969-016-0118-0