Abstract
BackgroundJuvenile 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.MethodsWe 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.ResultsTwenty 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.ConclusionThe 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.
Highlights
Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory childhood myopathy of uncertain aetiology
A better understanding of the epidemiology and clinical outcomes of JDM has been enhanced by the establishment of multicenter and multinational collaborations such as the paediatric rheumatology international trials organization (PRINTO) and international myositis assessment and clinical studies group (IMACS)
Higher rates of dystrophic calcinosis have been reported among indigenous African compared to JDM patients of other ancestral backgrounds [16]
Summary
Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory childhood myopathy of uncertain aetiology. Juvenile dermatomyositis (JDM) is an immune mediated vasculopathic disease of childhood characterized by inflammation of the striated muscles, skin and internal organs. The most common clinical manifestations are proximal muscle weakness and characteristic cutaneous lesions such as heliotrope rash, Gottron’s papules and calcinosis. The criteria include presence of characteristic rashes with any of symmetrical proximal muscle weakness, elevated serum muscle enzymes, electromyographic changes and features of inflammatory myositis on muscle biopsy. The presence of the skin manifestations together with two of the other features is required for classification of a patient as having probable JDM. The characteristic skin lesions may occur without apparent muscle involvement and is referred to as amyopathic dermatomyositis [7]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
