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Risk factors associated with calcinosis of juvenile dermatomyositis
Adriana M E Sallum1, Francine C M M Pivato, Ulysses Doria-Filho
1Departamento de Pediatria, Faculdade de Medicina, Universidade de São Paulo (USP), São Paulo, SP, Brazil. adrimaluf@icr.hcnet.usp.br
Insights
Calcinosis is common in juvenile dermatomyositis, often appearing later in the disease. Risk factors include cardiac involvement and the need for immunosuppressants, indicating more severe disease.
Area of Science:
- Pediatric Rheumatology
- Dermatology
- Immunology
Background:
- Juvenile dermatomyositis (JDM) is a rare autoimmune disease affecting children.
- Calcinosis, the deposition of calcium in tissues, is a significant complication of JDM.
- Identifying risk factors for calcinosis is crucial for improving patient outcomes.
Purpose of the Study:
- To determine the risk factors associated with the development of calcinosis in pediatric patients diagnosed with JDM.
- To analyze the clinical, laboratory, and treatment data of JDM patients with and without calcinosis.
Main Methods:
- Retrospective review of medical records for 54 JDM patients.
- Data collected included demographics, clinical features (muscle strength, pulmonary, GI, cardiac), lab tests (muscle enzymes), and treatments (corticoids, hydroxychloroquine, immunosuppressants).
- Univariate and multivariate analyses were performed to identify associations with calcinosis.
Main Results:
- Calcinosis occurred in 43% of JDM patients, with 74% developing it post-diagnosis.
- Univariate analysis showed associations between calcinosis and cardiac involvement, pulmonary involvement, and use of immunosuppressants.
- Multivariate analysis confirmed cardiac involvement (OR=15.56) and use of immunosuppressants (OR=4.01) as independent risk factors for calcinosis.
Conclusions:
- Calcinosis is a frequent complication in JDM, often developing as the disease progresses.
- Cardiac involvement and the requirement for immunosuppressive therapy are significant indicators of increased calcinosis risk in JDM.
- These findings highlight the association of calcinosis with more severe JDM cases requiring intensive treatment.
Objective:
To identify risk factors associated with calcinosis in children and adolescents with juvenile dermatomyositis.
Methods:
A review was carried out of the medical records of 54 patients with juvenile dermatomyositis. Data were collected on demographic characteristics, clinical features: muscle strength (stages I to V of the Medical Research Council scale), pulmonary involvement (restrictive pulmonary disease with presence or absence of anti-Jo1 antibodies), gastrointestinal problems (gastroesophageal reflux) and/or heart disease (pericarditis and/or myocarditis); laboratory tests: elevated muscle enzyme levels in serum (creatine phosphokinase, aspartate aminotransferase, alanine aminotransferase and/or lactate dehydrogenase); and on the treatments given: corticoid therapy in isolation or associated with hydroxychloroquine and/or immunosuppressants. The patients were divided into two groups, depending on presence or absence of calcinosis and data were evaluated by both univariate and multivariate analyses.
Results:
Calcinosis was identified in 23 (43%) patients, and in six (26%) patients it had emerged prior to diagnosis while in 17 (74%) it was post diagnosis. The univariate analysis revealed that cardiac (p = 0.01) and pulmonary (p = 0.02) involvement and the need for one or more immunosuppressor (methotrexate, cyclosporine A and/or pulse therapy with intravenous cyclophosphamide) to treat juvenile dermatomyositis (p = 0.03) were all associated with an increased incidence of calcinosis. The multivariate analysis then demonstrated that only cardiac involvement (OR = 15.56; 95%CI 1.59-152.2) and the use of one or more immunosuppressor (OR = 4.01; 95%CI 1.08-14.87) were independently associated with the presence of calcinosis.
Conclusions:
Calcinosis was a frequent development among these juvenile dermatomyositis cases, generally emerging as the disease progressed. Calcinosis was associated with the more severe cases that also had cardiac involvement and where immunosuppressors had to be included in the treatment.
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