Is juvenile dermatomyositis a different disease in children up to three years of age at onset than in children above three years at onset A retrospective review of 23 years of a single center’s experienceReport as inadecuate

Is juvenile dermatomyositis a different disease in children up to three years of age at onset than in children above three years at onset A retrospective review of 23 years of a single center’s experience - Download this document for free, or read online. Document in PDF available to download.

Pediatric Rheumatology

, 10:34

First Online: 20 September 2012Received: 08 April 2012Accepted: 13 August 2012


BackgroundWe tested the hypothesis that the course and outcome of juvenile dermatomyositis JDM in children seen at one center with the JDM disease onset at or below three years of age is different from that in the children with disease onset at greater than three years of age.

MethodsInstitutional Review Board approval was obtained to retrospectively review the charts of 78 patients from age 0–18 years with JDM seen in the pediatric rheumatology clinic at Nationwide Children’s Hospital in Columbus, Ohio over the past 23 years from January 1988. The diagnosis was made by the treating pediatric rheumatologist. Not all the patients met the Bohan and Peter criteria, as muscle biopsy and EMG were not always performed and we utilized a modified JDM criteria. The data regarding disease course and outcome were collected as of the last clinic follow-up or to July 1, 2010. We used the Wilcoxon Two-Sample test to compare numerical variables between two age groups, and used logistic regression to compare categorical variables between two age groups in SAS 9.1.3. Minitab-16 was used to calculate various mean, median, modes, standard deviations and range. For survival analysis, we used Kaplan-Meier method with log-rank test.

ResultsThe mean age of onset in the two groups at Nationwide Children’s Hospital was 27 months and 91 months. The mean times between onset of symptoms to diagnosis in the younger and older age groups was 5.6 months and 4.5 months, respectively, not a statistically significant difference. The younger onset group had more females p=0.05 and their disease onset occurred less frequently during the typical winter-spring seasons p=0.031. The younger onset group was more likely to have a preceding fever p=0.029 and family history of autoimmune diseases p=0.012. The younger onset group was less likely to have heliotrope rash p=0.04, Gottron’s sign p=0.049, capillary loop abnormalities p=0.010, or elevations in creatine kinase CK, p=0.022, aspartate aminotransferase AST, p=0.021 or aldolase p=0.035. The younger onset group was treated less often with pulse methylprednisolone at diagnosis p=0.043 and less often with hydroxychloroquine p=0.035. There were no differences between the two groups regarding initial oral steroid dose p=0.8017, number of patients who received methotrexate at diagnosis p=0.709, and the number who ever received other immunosuppressants p=0.323. The mean and maximum duration mean duration 24.3 months vs. 35.2 months, maximum duration 51 vs. 124 months in younger and older onset group respectively of methotrexate therapy, and the mean and maximum duration of oral steroid therapy Mean duration 16.8 months vs. 33.3 months, maximum duration 50 vs. 151 months in younger and older onset group respectively, was shorter in the younger group. The younger onset patients were less likely to have active disease at 5 years 9% vs. 35.7%, p=0.015 and 10 years post-diagnosis 9% vs. 45.1%, p=0.011, Table 7. The younger patients were less likely to have osteonecrosis p=0.023. Two disease-related deaths occurred in the younger group, none in the older group. The results of the survival analysis showed that the difference between the age groups was statistically significant p < 0.012. The sex and race were not significant p> 0.26 and p>0.95, respectively.

ConclusionsThere were significant differences between JDM patients with disease onset at or below age three years at our center, compared to their older counterparts. Younger patients in our cohort had fewer typical findings at diagnosis and a milder disease course without needing as long a duration of corticosteroids and immunosuppression. Patients with a younger onset had a higher mortality rate but mortalities were unusual and numbers small. The younger group had a similar complication rate compared to the older onset patients, except for osteonecrosis which was higher in the older onset group. These findings differ from the previous reports that a younger age of onset in JDM is often associated with a more severe disease, as results at our center suggest that children with younger onset JDM appear to be atypical but may do well compared to the older JDM patients.

KeywordsJuvenile dermatomyositis Young age at onset Vasculopathy Disease outcome AbbreviationsJDMJuvenile dermatomyositis

AVNAvascular necrosis

ASTAspartate aminotransferase

LDHLactate dehydrogenase

CKCreatine kinase


MRIMagnetic resonance imaging

TNFTumor necrosis factor

MMFMycophenolate mofetil

MACMembrane attack complex

St DevStandard deviation.

Electronic supplementary materialThe online version of this article doi:10.1186-1546-0096-10-34 contains supplementary material, which is available to authorized users.

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Author: Anjali Patwardhan - Robert Rennebohm - Igor Dvorchik - Charles H Spencer


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