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Initial manifestations and risk factors for calcinosis in juvenile dermatomyositis: A retrospective multicenter study
Mustafa Cakan1, Semanur Ozdel2, Serife Gul Karadag3
1Department of Pediatric Rheumatology, University of Health Sciences, Zeynep Kamil Women and Children's Diseases Training and Research Hospital, Istanbul, Turkiye.
Insights
Calcinosis in juvenile dermatomyositis (JDM) remains a challenge, with factors like diagnostic delay and specific symptoms increasing risk. Early detection and treatment are crucial for managing JDM complications.
Area of Science:
- Pediatric Rheumatology
- Autoimmune Diseases
- Dermatology
Background:
- Juvenile dermatomyositis (JDM) is a rare autoimmune disease affecting children.
- Calcinosis, the deposition of calcium in soft tissues, is a significant complication of JDM.
- Understanding the risk factors for calcinosis is essential for improving patient outcomes.
Purpose of the Study:
- To identify initial clinical manifestations of JDM.
- To report follow-up outcomes in children with JDM.
- To investigate risk factors associated with the development of calcinosis in JDM patients.
Main Methods:
- Retrospective review of medical records of children diagnosed with JDM between 2005 and 2020.
- Analysis of demographic data, clinical features at diagnosis, disease course, and development of calcinosis.
- Statistical analysis including multivariate logistic regression to identify independent risk factors.
Main Results:
- The study included 48 children (33 girls, 15 boys) with a mean age of 7.6 years at onset.
- Calcinosis developed in 22.9% of patients.
- Factors associated with higher calcinosis risk included myalgia, livedo racemosa, skin hypopigmentation, lower ALT levels, higher physician visual analog scores, diagnostic delay, and chronic persistent disease course.
Conclusions:
- While JDM mortality has decreased, calcinosis rates remain concerning.
- Long duration of active, untreated disease is a primary risk factor for calcinosis.
- Specific clinical findings at diagnosis may indicate a higher risk for calcinosis development in JDM.
Objective:
This study aimed to look for the initial manifestations of juvenile dermatomyositis (JDM), give follow-up results, and search for risk factors for the development of calcinosis.
Methods:
The files of children with JDM diagnosed between 2005 and 2020 were reviewed retrospectively.
Results:
The study included 48 children, 33 girls and 15 boys. The mean age at the onset of the disease was 7.6±3.6 years. The median duration of follow-up was 35 (6-144) months. Twenty-nine patients (60.4%) had monocyclic, 7 (14.6%) patients had polycyclic, and 12 (25%) patients had chronic persistent disease course. At the time of enrollment, 35 (72.9%) patients were in remission, while 13 (27.1%) patients had active disease. Calcinosis developed in 11 patients (22.9%). Children having myalgia, livedo racemosa, skin hypopigmentation, lower alanine aminotransferase (ALT) levels, and higher physician visual analog scores at the time of diagnosis had a higher risk for calcinosis. Calcinosis was also more common in children with diagnostic delay and chronic persistent disease course. None of these parameters remained independent risk factors for calcinosis in multivariate logistic regression analysis.
Conclusion:
The rate of mortality has decreased dramatically over decades in JDM, but the rate of calcinosis has not changed proportionately. Long duration of active, untreated disease is accepted as the main risk factor for calcinosis. We have seen that calcinosis was more common in children having myalgia, livedo racemosa, skin hypopigmentation, lower ALT levels, and higher physician visual analog scores at the time of diagnosis.
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