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

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dc.contributor.authorOkong’o, Lawrence Owino
dc.contributor.authorEsser, Monika
dc.contributor.authorWilmshurst, Jo
dc.contributor.authorScott, Christiaan
dc.date.accessioned2021-10-08T07:08:34Z
dc.date.available2021-10-08T07:08:34Z
dc.date.issued2016
dc.description.abstractAbstract 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.
dc.identifier.apacitation, Esser, M., Wilmshurst, J., & Scott, C. (2016). Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. <i>Pediatric Rheumatology</i>, 14(1), 174 - 177. http://hdl.handle.net/11427/34607en_ZA
dc.identifier.chicagocitation, Monika Esser, Jo Wilmshurst, and Christiaan Scott "Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study." <i>Pediatric Rheumatology</i> 14, 1. (2016): 174 - 177. http://hdl.handle.net/11427/34607en_ZA
dc.identifier.citation, Esser, M., Wilmshurst, J. & Scott, C. 2016. Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. <i>Pediatric Rheumatology.</i> 14(1):174 - 177. http://hdl.handle.net/11427/34607en_ZA
dc.identifier.issn1546-0096
dc.identifier.ris TY - Journal Article AU - Okong’o, Lawrence Owino AU - Esser, Monika AU - Wilmshurst, Jo AU - Scott, Christiaan AB - 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. DA - 2016 DB - OpenUCT DP - University of Cape Town IS - 1 J1 - Pediatric Rheumatology LK - https://open.uct.ac.za PY - 2016 SM - 1546-0096 T1 - Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study TI - Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study UR - http://hdl.handle.net/11427/34607 ER - en_ZA
dc.identifier.urihttp://hdl.handle.net/11427/34607
dc.identifier.vancouvercitation, Esser M, Wilmshurst J, Scott C. Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. Pediatric Rheumatology. 2016;14(1):174 - 177. http://hdl.handle.net/11427/34607.en_ZA
dc.language.isoeng
dc.publisher.departmentDepartment of Paediatrics and Child Health
dc.publisher.facultyFaculty of Health Sciences
dc.sourcePediatric Rheumatology
dc.source.journalissue1
dc.source.journalvolume14
dc.source.pagination174 - 177
dc.source.urihttps://dx.doi.org/10.1186/s12969-016-0118-0
dc.subject.otherAfrica
dc.subject.otherJDM
dc.subject.otherJuvenile dermatomyositis
dc.subject.otherOutcome
dc.subject.otherAge of Onset
dc.subject.otherAnti-Inflammatory Agents
dc.subject.otherChild, Preschool
dc.subject.otherCross-Sectional Studies
dc.subject.otherDermatologic Agents
dc.subject.otherDermatomyositis
dc.subject.otherFemale
dc.subject.otherHumans
dc.subject.otherInfant
dc.subject.otherMale
dc.subject.otherRegistries
dc.subject.otherRetrospective Studies
dc.subject.otherSouth Africa
dc.subject.otherTreatment Outcome
dc.subject.otherJuvenile dermatomyositis
dc.titleCharacteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study
dc.typeJournal Article
uct.type.publicationResearch
uct.type.resourceJournal Article
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