CASE REPORT
The First Reported Case of Juvenile Dermatomyositis in an Adolescent with Down Syndrome and the Clinical Considerations for Therapy
Jordan T. Jones1, 2, *
Article Information
Identifiers and Pagination:
Year: 2021Volume: 15
First Page: 65
Last Page: 68
Publisher ID: TORJ-15-65
DOI: 10.2174/1874312902115010065
Article History:
Received Date: 10/03/2021Revision Received Date: 24/5/2021
Acceptance Date: 07/6/2021
Electronic publication date: 15/10/2021
Collection year: 2021
open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Abstract
Background:
Down Syndrome (DS) is one of the most common birth conditions in the United States of America, with approximately 5300 births annually, resulting in an estimated birth prevalence of 12.6 per 10,000 live births and a population prevalence in the USA since 2010 of 6.7 per 10,000 inhabitants. Children with DS have complex medical challenges that present due to changes in their immune system that results in increased rates of infection, malignancy, and autoimmune disease. Juvenile Dermatomyositis (JDM) is a rare, autoimmune disease, and the most common inflammatory myopathy of childhood. Reports suggest an increased incidence of arthritis in children with DS, but there have been no reports of JDM in children with DS. Additionally, those with DS pose unique challenges with an increase in adverse effects and ineffectiveness of immunosuppressive therapy.
Case :
We describe the first case of an adolescent female with DS who developed JDM with a positive anti-p155/140 antibody and characteristic clinical phenotype. We discuss increased awareness of autoimmune disease in adolescents with DS and clinical considerations for therapy with immunosuppression.
Conclusion:
Adolescents with DS are at increased risk for autoimmune disease, including JDM. Awareness, early recognition of the signs and symptoms of autoimmune disease in those with DS is important, so appropriate therapy can be implemented.
