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Clinical features and disease course of patients with juvenile dermatomyositis
Author(s) -
Gowdie Peter J.,
Allen Roger C.,
Kornberg Andrew J.,
Akikusa Jonathan D.
Publication year - 2013
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/1756-185x.12107
Subject(s) - medicine , juvenile dermatomyositis , dermatomyositis , muscle biopsy , rash , creatine kinase , cohort , retrospective cohort study , biopsy , magnetic resonance imaging , surgery , gastroenterology , dermatology , radiology
Objective To describe the clinical features and course of a cohort of patients with juvenile dermatomyositis ( JDM ) at a tertiary referral pediatric centre in A ustralia and examine changes in diagnostic and therapeutic approach over time. Methods Retrospective review of patients diagnosed with JDM at the R oyal C hildren's H ospital, M elbourne, between 1989 and 2010. Results Fifty‐seven patients were identified. The female : male ratio was 2 : 1 and median age at diagnosis was 7.1 years (2.2–15.3). At diagnosis, 95% had weakness, all had typical rash and 68% had nailfold capillary changes. Calcinosis was not present in any patients at diagnosis and occurred in 18% over time. Creatine kinase, lactate dehydrogenase, aspartate aminotransferase, alanine aminotransferase and aldolase levels were abnormal in 65%, 92%, 88%, 58% and 100%, respectively. Magnetic resonance imaging ( MRI ) was abnormal in 97% of patients, electomyograph ( EMG ) in 83% and muscle biopsy in all four patients in whom it was performed. MRI was used in 86% (24/28) of patients diagnosed after 2000. Muscle biopsy was used in four and EMG in no patients over the same period. Treatment used throughout the disease course included oral steroids (93%), high‐dose pulse intravenous steroids (82%), methotrexate (63%), intravenous immunoglobulin (32%) and cyclosporin (18%). The disease was monophasic in 46.7% (21/45), polyphasic in 17.7% (8/45) and chronic in 35.5% (16/45). Conclusions Australian patients with JDM have similar characteristics to previously described cohorts. In practice, MRI has replaced the invasive diagnostic tests included in the Bohan and Peter criteria for the diagnosis of JDM . The early use of disease‐modifying anti‐rheumatic drugs has become the most common treatment approach.