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Calcinosis in Adult and Juvenile Dermatomyositis: Pathogenesis and Potential Therapeutic Targets
Belina Y Yi1, Christian Lood2, Antonia Valenzuela3
1Division of Pediatric Allergy, Immunology, and Rheumatology, Johns Hopkins University School of Medicine, Johns Hopkins Children's Center, 200 North Wolfe Street, The David M. Rubenstein Child Health Building, Baltimore, MD 21287, USA.
Abstract:
Calcinosis is a severe complication of adult and juvenile dermatomyositis (DM/JDM), characterized by the deposition of calcium in the skin, muscles, and soft tissues, leading to significant morbidity. This review explores the pathogenesis of DM/JDM-associated calcinosis, highlighting the roles of chronic inflammation, neutrophil extracellular traps, proinflammatory cytokines, and mitochondrial stress. Current therapeutic approaches including anti-inflammatory agents and emerging treatments such as chimeric antigen receptor T (CAR-T) cells are reviewed. Additional therapies targeting calcium metabolism are also examined. Despite the variety of treatments, there is a need for controlled trials with validated outcomes to understand the efficacy and safety of these therapies.
Insights
Calcinosis in dermatomyositis involves calcium deposits due to inflammation and cellular stress. New treatments like CAR-T cells show promise, but more research is needed to confirm efficacy and safety.
Area of Science:
- Rheumatology
- Immunology
- Cell Biology
Background:
- Calcinosis is a severe complication of dermatomyositis (DM/JDM), causing calcium deposition in tissues and significant morbidity.
- The pathogenesis involves chronic inflammation, neutrophil extracellular traps, pro-inflammatory cytokines, and mitochondrial stress.
Purpose of the Study:
- To review the pathogenesis of DM/JDM-associated calcinosis.
- To explore current and emerging therapeutic strategies for calcinosis.
- To identify the need for further clinical trials.
Main Methods:
- Literature review of pathogenesis and treatment of DM/JDM-associated calcinosis.
- Analysis of current anti-inflammatory agents and novel therapies.
- Examination of treatments targeting calcium metabolism.
Main Results:
- Pathogenesis involves inflammation, NETs, cytokines, and mitochondrial stress.
- Current treatments include anti-inflammatory agents.
- Emerging therapies include CAR-T cells and calcium metabolism modulators.
Conclusions:
- DM/JDM-associated calcinosis has complex pathogenesis.
- Various therapeutic options exist, but efficacy requires validation.
- Controlled trials with validated outcomes are essential to assess treatment effectiveness and safety.
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