At the October 25th Town Hall, Dr. Christian Lood, professor and researcher at the University of Washington and Seattle Children’s Hospital, presented on the topic of treating a potential complication of juvenile myositis known as calcinosis. Calcinosis is the accumulation of calcium phosphate crystals in soft tissue (skin, muscle). It appears as hard, irregular nodules (bumps) in or under the skin and various parts of the body. Up to 40% of children with JDM will develop calcinosis. Though many children may develop calcinosis, the problem remains that we know very little about calcinosis.
What we do know is that it is associated with certain autoantibodies, especially NDA5 and MPX2. We also know that delayed treatment, frequent trauma (physical use), or hypoxia can cause an increase in calcinosis. With more research needed on calcinosis, it can be difficult to treat, leading to treatments being prescribed on a case-by-case basis.
Tras la presentación del Dr. Lood, el Dr. Adam Schiffenbauer presentó información detallada sobre el estado actual de la gestión de la calcinosis, los posibles tratamientos y la importancia de una intervención precoz y un tratamiento agresivo. A continuación figura una lista detallada de los medicamentos y las medidas de tratamiento que se debatieron:
Terapias antiinflamatorias: Anti-inflammatory therapies involve treatments aimed at reducing inflammation in the body, which can help manage symptoms of JDM and calcinosis simultaneously.
- Inmunoglobulina intravenosa (IVIG)
- Abatacept Genérico: Orencia
- Talidomida
- Infliximab Genérico: Remicade
- Colchicina Marca: Colcrys & Mitigare
- Anakinra Genérico: Kineret
- Inhibidores JAK: Tofacitinib, Ruxolitinib, Baricitinib e infliximab (inyectable)
Medicamentos dirigidos al propio calcio/calcinosis: Estos medicamentos se utilizan específicamente para tratar los nódulos de calcio presentes en el organismo del niño y se utilizarían además de la medicación y los tratamientos de la DMJ del niño.
- Probenecid Genérico: Benemid & Benuryl
- Calcium Blockers: Diltiazem Marca: Cardizem CD y Taztia XT
- Treprostinil Marca: Remodulin
- Bifosfonatos (pamidronato, etidronato, alendronato)
- Tiosulfato de sodio
- Hidróxido de aluminio
Medida mecánica: Mechanical measures would be taken as a last result and in more extreme cases of calcinosis.
- Amortiguación/protección frente a microtraumatismos
- Resección quirúrgica
- Litotricia
- Terapia con láser de dióxido de carbono
- Oxígeno hiperbárico
Algunos puntos clave que nos han dejado tanto el Dr. Lood como el Dr. Schiffenbauer’s presentation is that many JDM children will develop calcinosis during their lives and that treatment must be done on a trial and effort basis. More importantly, early treatment of the JDM and calcinosis is most important for all children.
