Clinical Characteristics of Children With Juvenile Dermatomyositis: The Childhood Arthritis and Rheumatology Research Alliance Registry

Abstract

Objective To investigate aspects of juvenile dermatomyositis (DM), including disease characteristics and treatment, through a national multicenter registry. Methods Subjects meeting the modified Bohan and Peter criteria for definite juvenile DM were analyzed from the cross-sectional Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry between 2010 and 2012 from 55 US pediatric rheumatology centers. Demographics, disease characteristics, diagnostic assessments, and medication exposure data were collected at enrollment. Results A total of 384 subjects met the criteria for analysis. At enrollment, the median Childhood Myositis Assessment Scale score was 51 (interquartile range [IQR] 46–52), the median Childhood Health Assessment Questionnaire score was 0 (IQR 0–0.5), and the median physician and subject global assessment scores were 1 (IQR 0–2) and 1 (IQR 0–3), respectively, out of a maximum of 10. Of the diagnostic assessments, magnetic resonance imaging was more likely than electromyography or muscle biopsy to show abnormalities. A total of 329 subjects had ≥2 diagnostic studies performed, and >34% of these subjects reported ≥1 negative study. Ninety-five percent had been treated with corticosteroids and 92% with methotrexate, suggesting that these medications were almost universally prescribed for juvenile DM in the US. Conclusion In 2 years, the ongoing CARRA Registry has collected clinical data on 384 children with juvenile DM and has the potential to become one of the largest juvenile DM cohorts in the world. More research is needed about prognostic factors in juvenile DM, and differences in therapy based on manifestations of disease need to be explored by practitioners. This registry provides the infrastructure needed to advance clinical and translational research and represents a major step toward improving outcomes of children with juvenile DM.

Childhood Arthritis and Rheumatology Research Alliance consensus clinical treatment plans for juvenile dermatomyositis with skin predominant disease

“…Malar rash and nailbed capillary changes are also frequently present [3]. JDM patients typically have proximal muscle weakness, but a subset of patients present with skin disease without any significant weakness or muscle inflammation [4, 5]. …”

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

“…Data from multicenter patient registries indicate that the average age of onset of JDM is seven years with greater incidence in girls than boys (ratio of 2:1) [8–11]. However, regional differences exist and studies from India and Saudi Arabia have reported higher incidence rates in male children [12, 13].…”

A 5-Year-Old Girl with Fatigue, Diffuse Edema, and Weakness

“…MRI is the most common diagnostic modality used in the pediatric population, as it is more likely to show abnormalities consistent with JDM compared to electromyography or muscle biopsy. [1][2][3] Our patient exhibited a rare presentation of JDM, as the predominant clinical feature prompting her to seek medical evaluation was diffuse subcutaneous edema. Although localized edema is common in JDM, generalized edema has been reported rarely in the literature.…”

“…[1][2][3]14 Intravenous gamma globulin use ranges from 17% to 39% of cohorts and is typically used for more severe presentations of JDM or in symptomatic patients refractory to standard treatment. 15 Once the myositis improves and enzyme levels normalize, corticosteroid treatment can be slowly tapered.…”