Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study

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.

Advances in Juvenile Dermatomyositis: Myositis Specific Antibodies Aid in Understanding Disease Heterogeneity

“…Soft tissue calcifications, a major contributor to morbidity and mortality, vary in frequency from 17% (57) to 44% (58), and are more severe in children of African descent (59). These dystrophic calcifications are associated with chronic cutaneous inflammation (60) as well as lipoatrophy, either focal or generalized (43).…”

“…Although the outcome of JDM has improved—the mortality rate is now 1–2% in the US—it is elevated elsewhere, approximately 8% (58). Weakness and reduced endurance was identified in 32–45% of subjects 16.8 years after the diagnosis in childhood of JDM; MRI confirmed muscle damage in 52% (128), predicted by skin inflammation 6 months after diagnosis (129).…”

Spectrum of paediatric rheumatic disorders at a tertiary hospital in Tanzania

“…Diagnosing dermatomyositis is usually challenging and rely on clinical criteria with no known confirmatory diagnostic tests requiring good clinical acumen [22]. Dermatomyositis has been described among African children as was noted by Faller et al and Okonkwo et al in Johannesburg and Cape Town respectively [23,24]. Patients with JDM may also present to neurologists and dermatologists because of various patterns of manifestations [25].…”

Juvenile dermatomyositis in Thai children: Retrospective review of 30 cases from a tertiary care center

“…The incidence of juvenile dermatomyositis is estimated to be about 2-4 per one million children, with differences among racial and ethnic groups. [2][3][4][5][6] Skin manifestations are a key feature of juvenile dermatomyositis, and they may precede, accompany or follow the onset of muscle weakness. According to the 2017 EULAR/ACR classification criteria for idiopathic inflammatory myopathy, juvenile dermatomyositis can be diagnosed with high sensitivity and specificity in the presence of three cardinal skin signs, including heliotrope rash, Gottron's papules, and Gottron's sign.…”