Clasificación y manejo actual en dermatomiositis juvenil / Classification and current management in juvenile dermatomyositis

La Dermatomiositis Juvenil representa el 75-80% de las miopatías inflamatorias juveniles. Si bien, tiene baja incidencia y prevalencia, presenta importante morbilidad dada por sus manifestaciones cutáneas, musculares, pulmonares, gastrointestinales, cardiacas, entre otras. Corresponde a un desorden poligénico con múltiples factores gatillantes, que determina el desarrollo de una vasculopatía que lleva a atrofia muscular, inflamación y activación de vías del IFN-1. Actualmente su diagnóstico se basa en las guias EULAR/ACR (2017). En los últimos años, se han descubiertos distintos subtipos de la enfermedad, basados en el perfil de autoanticuerpos específicos de miositis, lo que ha permitido establecer pronóstico y estrategias terapéuticas personalizadas. El manejo farmacológico continúa basándose principalmente en el uso de corticoesteroides y DMARDs, así como también terapia biológica; en los últimos años, los inhibidores JAK han mostrado resultados promisorios, convirtiéndose en la más nueva alternativa terapéutica para el control de la enfermedad.

Juvenile Dermatomyositis represents 75-80% of juvenile inflammatory myopathies. Although it has a low incidence and prevalence, it presents significant morbidity due to its cutaneous, muscular, pulmonary, gastrointestinal and cardiac manifestations, among others. It corresponds to a polygenic disorder with multiple triggering factors, which determines the development of a vasculopathy that leads to muscle atrophy, inflammation and activation of IFN-1 pathways. Currently its diagnosis is based on the EULAR/ACR guidelines (2017). In recent years, different subtypes of the disease have been discovered, based on the profile of myositis-specific autoantibodies, which has made it possible to establish prognosis and personalized therapeutic strategies. Pharmacological management continues to be based mainly on the use of corticosteroids and DMARDs, as well as biological therapy; In recent years, JAK inhibitors have shown promising results, becoming the newest therapeutic alternative for disease control.

Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil

BACKGROUND: Dermatomyositis affects striated muscles, skin and other organs. OBJECTIVE: To characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results, therapeutic and prognostic findings compared to those in the literature. METHODS: Data were obtained from medical records of 109 patients who were classified into five groups: 23 juvenile dermatomyositis; 59 primary idiopathic dermatomyositis; 6 amyopathic dermatomyositis; 7 dermatomyositis associated with neoplasms and 14 dermatomyositis associated with other connective tissue diseases. RESULTS: Sixty patients were classified as "definite" diagnosis; 33 as "possible"; four as "probable" and 12 and as amyopathic. The average age at diagnosis was 36 years. Cutaneous manifestations occurred in all patients; the most frequent symptom was loss of proximal muscle strength; the most common pulmonary disorder was interstitial lung disease, and gastritis was the most prevalent digestive manifestation. Tumors were documented in 6.42% of cases. Lactate dehydrogenase was the muscle enzyme most frequently elevated in the majority of cases. Skin biopsies were performed in 68 patients; muscle biopsies in 53; and electroneuromyographies in 58 patients. The most commonly used treatment was corticotherapy and the mortality rate was 14.7%. CONCLUSION: in this sample, the disease appeared in younger individuals, was more frequent in women and the association with cancer was small. .

Skin eruption with myalgia and proximal muscle weakness

This article, Skin eruption with myalgia and proximal muscle weakness is designated as CME/CPD. It provides the reader with the opportunity to obtain credits under the MPC Program of KIMS. Readers who study it, answer the questions related to it on page 49, and send a copy of the Answer Sheet [page 50] to the CME Center of KIMS become eligible for 0.5 CME/CPD credit in Category 1. To claim credit, the reader has to be registered in the MPC Program, the answer sheet should be received by the CME Center before 31[st] May 2005, and all questions should have been attempted. Readers would then receive a certificate from the CME Center indicating the credit data

Dermatomiositis juvenil: presentación de 10 casos

Se presenta una serie de 10 pacientes con Dermatomiositis juvenil diagnosticados en el Hospital Universitario Ramón Gonzalez Valencia (HURGV) de Bucaramanga en el periódo de 13 años, estudio retrospectivo cuyo objeto es analizar las características clínicas, de laboratorio terapéutica y evolución. Los resultados mostraron predominio de mujeres (9 casos), procedentes de áreas rurales de Santander con una edad promedio de 8.8 años (rango de 6-12), con manifestaciones clínicas múltiples: lesión en piel, debilidad muscular, artralgia, fiebre, fotosensibilidad, disfagia, fenómeno de Raynaud y epistaxis. La velocidad de sedimentación globular (VSG), creatinkinasa (CK) estuvieron elevadas en todos los pacientes; la electromiografía mostró un patrón miopático. En la mayoría de pacientes hubo buenas respuestas a esteroides y evolución clínica satisfactoria. La elaboración detallada de la historia clínica con el examen físico minucioso y la elevación de enzimas musculares son de gran ayuda para el diagnóstico de esta enfermedad

Dermatomiositis y neoplasias: revisión de la literatura a proposito de un caso / Dermatomyositis and neoplasia: report of a case and review of the literature

La internación en nuestro servicio de un paciente con dermatomiositis y el hallazgo de una neoplasia concomitante, de localización poco frecuente, motivó la revisión de la literatura al respecto y la posterior comunicación de este caso

Dermatomyositis of children and adults

Los autores refieren un caso de dermatomiositis del niño y uno del adulto, ambos de sexo femenino. Se establecen las características de la afección más notorias en estas dos etapas de la vida y se hace referencia a la clasificación de las miositis de Bohan y colaboradores