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Clinical characteristics of children with juvenile dermatomyositis recruited within the first 7 months of the CARRAnet registry
© Hoeltzel et al; licensee BioMed Central Ltd. 2012
Published: 13 July 2012
Performing quality clinical and translational research in juvenile dermatomyositis (JDM) is difficult due to the rarity of the disease. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) initiated a multi-center observational cohort study to create a foundational clinical database for the major rheumatic diseases of childhood, including JDM. Initial data from the JDM cohort (prevalent and incident cases) enrolled in the first 7 months of this ongoing study are described here.
Children under the age of 21 with onset of JDM prior to 16 yrs old were included. JDM was established using modified Bohan and Peter criteria. Subjects or their guardians were consented, and clinical data were collected from the patients/guardians and medical providers using both general and JDM-specific case report forms at the time of enrollment. Data regarding demographics, disease characteristics, diagnostic assessment, and medication exposure were collected. Baseline measures of muscle strength, physical functioning, and quality of life were performed, including the Childhood Myositis Assessment Scale (CMAS), Childhood Health Assessment Questionnaire (CHAQ), Health Related Quality of Life measure (HRQOL), ACR Functional Class rating, global disease assessments, and pain scores. Data were pooled and stored in a secure centralized database and de-identified prior to analysis. IRB approval was obtained at each enrolling site.
Demographics and disease characteristics
Number (%) of subjects
Number (%) with characteristic ever
Number (%) with characteristic at enrollment
Elevated muscle enzymes
1° Family hx of autoimmunity
Median, yrs (quartiles)
V or shawl sign
10.6 (7.1, 14.8)
Age of onset
6.0 (3.3, 9.5)
3.0 (1.7, 5.7)
Malar or facial erythema
Time to rhematologie care
0.32 (0.13, 0.67)
Gottron sign, papules, or heliotrope
In 7 months, the ongoing CARRA registry of children with rheumatic diseases has collected clinical data on 102 children with JDM and has the potential to become one of the largest JDM cohorts in the world. This registry provides the infrastructure needed to advance clinical and translational research and represents a major step towards improving outcomes of children with JDM.
Mark F. Hoeltzel: None; Mara L. Becker: None; Angela B. Robinson: None; Brian M. Feldman: None; Adam Huber: None; Ann M. Reed: None; Juvenile Myositis CARRA Subgroup: None; CARRAnet Investigators: None.
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.