Análisis de los tratamientos en pacientes diagnosticados con polimiositis / Analysis of Treatments in Patients Diagnosed with Polymyositis

Introducción: La polimiositis, la frase conocida como miopatía idiopática inflamatoria, es una enfermedad poco frecuente, considerada rara y heterogénea, que se caracteriza por la debilidad muscular, por lo que puede dificultar la movilidad cotidiana Objetivo: Analizar los tratamientos farmacológicos y no farmacológicos en pacientes diagnosticados con polimiositis. Métodos: Se realizó una búsqueda bibliográfica donde se siguió la recomendación PRISMA. Las fuentes de información consultadas fueron: SciELO, LILACS, PubMed, Elsevier, EBSCO, Medline, Google Académico, en el período de 2018 a 2022. Resultados: Se consultaron un total de 14 268 artículos correspondientes a la búsqueda bibliográfica, de ellos 42 artículos cumplieron con los criterios de selección. Se utilizó el método PRISMA según su recomendación, quedaron un total de cuatro artículos científicos originales de las cuales tres describen tratamientos farmacológicos, que mencionan a los corticoides y a los inmunosupresores; sin embargo, en aquellos pacientes que no responden al tratamiento se le recomienda la intervención clínica con inmunoglobulina G (IgG), que proporciona anticuerpos como moléculas monoméricas policlonales, que son bien tolerada. Por otro lado, dos artículos describen como tratamiento no farmacológico a la rehabilitación física con el objetivo de evitar el deterioro muscular. Conclusiones: El tratamiento en los pacientes diagnosticados con polimiositis debe ser individualizado, a partir de la gravedad de dicho padecimiento. A Una mayor afectación del cuerpo del paciente a nivel muscular, menor será la respuesta al tratamiento. Es importante la rehabilitación física y el uso de fármacos para controlar y aliviar la polimiositis(AU)

Introduction: Polymyositis known as idiopathic inflammatory myopathy is a rare disease. It is heterogeneous disease, characterized by symmetrical muscle weakness, which can make daily mobility difficult. Objective: To analyze pharmacological and non-pharmacological treatments in patients diagnosed with polymyositis. Methods: A bibliographic search was carried out following PRISMA recommendation. The information sources consulted were SciELO, LILACS, PubMed, Elsevier, EBSCO, Medline, Google Scholar from 2018 to 2022. Results: 14,268 articles corresponding to the bibliographic search were consulted, only 42 met the selection criteria. PRISMA method was used according to its recommendation. Four original scientific articles remained, three of them describe pharmacological treatments mentioning corticosteroids and immunosuppressants. However, in those patients who do not respond to treatment, clinical intervention with immunoglobulin G (IgG) is recommended, which provides antibodies as polyclonal monomeric molecules, which are well tolerated. On the other hand, two articles describe physical rehabilitation as a non-pharmacological treatment with the aim of avoiding muscle deterioration. Conclusions: Treatment in patients diagnosed with polymyositis should be individualized, based on the severity of the condition. A greater involvement of the patient's body at the muscular level, the lower the response to treatment. Physical rehabilitation and the use of drugs is important to control and relieve polymyositis(AU)

Piomiositis en pediatría: experiencia de 10 años en un hospital pediátrico de alta complejidad en Argentina / Pyomyositis in pediatrics: 10-year experience in a high-complexity pediatric hospital in Argentina

INTRODUCCIÓN: La piomiositis es una infección bacteriana agudasubaguda del músculo esquelético. OBJETIVO: Estimar la incidencia de piomiositis en pacientes internados, describir e identificar factores de riesgo para bacteriemia y hospitalización, y evaluar diferencias entre Staphylococccus aureus sensible y resistente a meticilina (SASM y SARM). PACIENTES Y MÉTODOS: Estudio descriptivo, retrospectivo, observacional, con pacientes de 1 mes a 18 años de edad, internados entre el 1 de enero de 2008 y 31 de diciembre de 2018. Variables: sexo, edad, hacinamiento en el hogar, existencia de lesión previa, estacionalidad, localización anatómica e imágenes, antibioterapia previa, estadio clínico, parámetros de laboratorio, cultivos y antibiograma, días de tratamiento intravenoso (IV), de internación, de fiebre y bacteriemia. RESULTADOS: Se incluyeron 188 pacientes. Incidencia: 38,9 casos / 10.000 admisiones (IC95 % 33,7 - 44,9). Días de internación y tratamiento IV: 11 (RQ 8-15 y RQ 8-14, respectivamente). El desarrollo de bacteriemia se asoció a PCR elevada (p = 0,03) y fiebre prolongada (p < 0,001). No hubo diferencias en la evolución y parámetros de laboratorio entre SASM y SARM. La leucocitosis (p = 0,004), neutrofilia (p = 0,005) y bacteriemia (p = 0,001) se asociaron a mayor estadía hospitalaria. CONCLUSIONES: Este estudio recaba la experiencia de más de 10 años de niños internados con diagnóstico de piomiositis y proporciona información sobre sus características. Se describen parámetros asociados a bacteriemia y estadía hospitalaria.

BACKGROUND: Pyomyositis is an acute-subacute bacterial infection of skeletal muscle. AIM: To estimate the incidence of pyomyositis in hospitalized patients, describe and identify risk factors for bacteremia and hospitalization, and evaluate differences between MSSA and MRSA. METHODS: Descriptive, retrospective, observational study with patients aged 1 month to 18 years hospitalized between January, 1, 2008 and December 1, 2018. Variables: sex, age, home overcrowding, previous injury, seasonality, anatomical location and images, previous antibiotherapy, clinical stage, laboratory, cultures and antibiogram, days of intravenous (IV) treatment, hospitalization, fever and bacteremia. RESULTS: 188 patients were included. Incidence: 38.9 cases/10,000 admissions (95% CI 33.7 - 44.9). Days of hospitalization and IV treatment: 11 (RQ 8-15 and RQ 8-14, respectively). The development of bacteremia was associated with elevated CRP (p = 0.03) and prolonged fever (p < 0.001). There were no differences in the evolution and laboratory parameters between MSSA and MRSA. Leukocytosis (p = 0.004), neutrophilia (p = 0.005), and bacteremia (p = 0.001) were associated with a longer hospital stay. CONCLUSIONS: This study collects the experience of more than 10 years of hospitalized children diagnosed with pyomyositis and provides information on its characteristics. Parameters associated with bacteremia and hospital stay are described.

Polimiositis: evolución de 4 años y agudización en 2019, caso masculino en Popayán, Cauca / Polymyositis: evolution of 4-years and agudizacion in 2019, male case in Popayán, Cauca

Resumen La polimiositis es una miopatía autoinmune que causa cada año a nivel mundial 4 casos por cada millón de habitantes, es de diagnóstico clínico y necesita tratamiento rápido y agresivo porque puede llevar a desenlaces fatales. Esta patología es infrecuente en hombres con una proporción mujer/hombre de 2.5:1, por lo que el objetivo del artículo fue describir y comparar con la literatura el caso de un paciente masculino con polimiositis quien debutó con debilidad muscular y dolor poliarticular de 20 días de evolución, con valores de creatina quinasa de 24000 UI/L, asociado a pérdida de peso y respondiendo adecuadamente al tratamiento médico brindado en el momento. Después de 3 años asintomático, sufrió una agudización que fue manejada con medicamentos de primera línea, pero sin mejoría, por lo que requirió metilprednisolona oral a altas dosis e inmunomoduladores. En ningún momento presentó compromiso de órganos vitales, actualmente es sintomático y se encuentra en manejo médico. MÉD.UIS.2022;35(1):49-56.

Abstract Polymyositis is an autoimmune myopathy and each year it causes 4 cases per million in the worldwide population, it is clinically diagnosed and needs rapid and aggressive treatment because it can lead to fatal outcomes. This pathology is infrequent in men, with a proportion women/men 2.5:1, the objective of the article was to describe and compare with the literature the case of a male patient with polymyositis, who presented with muscle weakness and polyarticular pain of 20 days of evolution, with Creatine kinase values of 24,000 IU/L, associated with weight loss, and responding adequately to the medical treatment provided at the time. After 3 years asymptomatic, he suffered an acute phase that was managed with first-line medications but without improvement, for which he required oral methylprednisolone at high doses and inmunomodulators. At no time did he present vital organ involvement, he is currently symptomatic and is under medical management. MÉD.UIS.2022;35(1):49-56.

Clinical characterization of patients with inflammatory myopathy in 2 tertiary care hospitals in Colombia: A descriptive survey / Caracterización clínica de pacientes con miopatía inflamatoria en 2 instituciones de alta complejidad en Colombia: estudio descriptivo

ABSTRACT Background: There is little information on inflammatory myopathies in Colombia. The objective was to identify the demographic and clinical characteristics of these patients in two tertiary care hospitals between 2010 and 2015. Materials and methods: A descriptive, retrospective survey was carried out, by reviewing medical records and obtaining information on demographic and clinical variables. The qualitative variables were expressed using absolute and relative frequencies, and the quantitative with mean and standard deviation (SD), or median with interquartile ranges (IQR), depending on data distribution. The IBM SPSS 22 statistical package was used. Results: A total of 105 patients with a mean age of 50.4 years (SD: 15.1) were included, with 76 (72.4%) women. In total, 50 subjects (48.5%) had a definitive diagnosis. The most common inflammatory myopathy was dermatomyositis (n = 66; 62.9%). The skin was the most commonly affected organ (n=66; 62.9%). Muscle weakness was present in 60 individuals (57.1%). The most frequent alarm sign was swallowing disorder (n = 28; 26.7%). Creatine phosphokinase was higher in polymyositis, with a median of 1800IU/L (IQR: 365-6157). The most widely used drugs were glucocorticoids (n = 83; 79%). Some patients were refractory to immunosuppressive treatment, mainly in antisynthetase syndrome (n = 5; 35.7%). Five patients (4.8%) died of infections (pneumonia and bacteraemia). Conclusions: In this cohort, the most common entity was dermatomyositis, and the most affected organ was the skin. There was a significant presentation of warning signs, refractoriness to immunosuppressive treatment, and lower muscle enzyme values compared to other cohorts. Mortality was mainly due to infectious complications.

RESUMEN Introducción: Existe poca información sobre las miopatías inflamatorias en Colombia. El obje tivo fue identificar las características demográficas y clínicas de estos pacientes en dos instituciones de alta complejidad entre los arios 2010 y 2015. Materiales y métodos: Se realizó un estudio descriptivo y retrospectivo. Mediante revisión de registros médicos, se obtuvo información sobre variables demográficas y clínicas. Las variables cualitativas se expresaron mediante frecuencias absolutas y relativas, y las cuantitativas con media y desviación estándar (DE) o mediana con rangos intercuartílicos (RIQ), dependiendo de la distribución de los datos. Se utilizó el paquete estadístico IBM SPSS® v.22. Resultados: Se incluyeron 105 pacientes con edad promedio de 50,4 años (DE: 15,1); 76 mujeres (72,4%). En total, 50 sujetos (48,5%) tuvieron diagnóstico definitivo. La miopa tía inflamatoria más común fue dermatomiositis (n = 66; 62,9%). La piel fue el órgano más comúnmente afectado (n = 66; 62,9%). La debilidad muscular estuvo presente en 60 individuos (57,1%). El signo de alarma más frecuente fue el trastorno de la deglución (n = 28; 26,7%). La creatinfosfoquinasa tuvo mayor elevación en polimiositis con una mediana de 1.800 Ul/l (RIQ: 365-6.157). Los medicamentos más utilizados fueron los glucocorticoides (n = 83; 79%). Hubo refractariedad al tratamiento inmunosupresor, principalmente en síndrome antisintetasa (n = 5; 35,7%). Cinco pacientes (4,8%) murieron por infecciones (neumonía y bacteriemia). Conclusiones: En esta cohorte, la entidad más común fue la dermatomiositis y el órgano más afectado fue la piel. Hubo presentación relevante de signos de alarma, refractariedad al tratamiento inmunosupresor y valores de enzimas musculares menores comparados con otras cohortes. La mortalidad fue principalmente por complicaciones infecciosas.

Características clínicas, inmunológicas y daño de órganos en pacientes con miopatías inflamatorias idiopáticas / Organ damage clinical and immunological characteristics in patients with idiopathic inflammatory myopathies

Introducción: Las miopatías inflamatorias idiopáticas constituyen un grupo de enfermedades musculares caracterizadas por debilidad muscular crónica e inflamación muscular de etiología desconocida. Objetivo: Identificar las características clínicas e inmunológicas y su relación con el daño de órganos en los pacientes con miopatías inflamatorias idiopáticas. Métodos: Se realizó estudio observacional, descriptivo, transversal, en 52 pacientes con diagnóstico de miopatía inflamatoria idiopática, seguidos en la consulta protocolizada de Reumatología del Hospital Clínico Quirúrgico Hermanos Ameijeiras entre enero 2016 y enero 2017. Para las variables cualitativas se calcularon los porcentajes de cada grupo. Se utilizó Chi-cuadrado de Pearson (estadístico exacto de Fisher). Nivel de significación del 95 por ciento (α = 0,05) para relacionar la presencia de anticuerpos y el tipo de miopatía así como la presencia de manifestaciones clínicas de MII. Resultados: El 80,8 por ciento fueron mujeres y 86,5 por ciento de procedencia urbana. La edad media al comienzo fue 42,8 ± 13,2 años, tiempo de demora al diagnóstico de 8,8 ± 7,0 meses, tiempo medio de evolución de la enfermedad de 7,5 ± 7,1 años. El 80,8 por ciento estaba en remisión, 50 por ciento tenía anticuerpos específicos. La hipertensión arterial se encontró en 28,8 por ciento de los pacientes y 23,1 por ciento presentó neumonía intersticial. La artritis estuvo presente en 96,2 por ciento. El 26,9 por ciento presentaron anticuerpos específicos Jo-1 y 21,2 por ciento Ro 52. Conclusiones: Predominaron los pacientes del sexo femenino en la cuarta década de la vida de procedencia urbana, los anticuerpos específicos encontrados más frecuentes fue el anti Jo-1, asociado a la presencia de neumopatía intersticial(AU)

Introduction: Idiopathic inflammatory myopathies constitute a group of muscle diseases characterized by chronic muscle weakness and muscle inflammation of unknown etiology. Objective: To identify the clinical and immunological characteristics and their relationship with organ damage in patients with idiopathic inflammatory myopathies. Methods: An observational, descriptive, cross-sectional study was carried out in 52 patients with diagnosis of idiopathic inflammatory myopathy, followed in the protocolized consultation of Rheumatology at Hermanos Ameijeiras Clinical and Surgical Hospital from January 2016 to January 2017. For the qualitative variables, the percentages of each group were calculated. Pearson's Chi-square (Fisher's exact statistic) was used. 95percent significance level (α = 0.05) was used to relate the presence of antibodies and the type of myopathy as well as the presence of clinical manifestations of MII. Results: 80.8percent were women and 86.5percent of urban origin. The mean age at the beginning was 42.8 ± 13.2 years, time delay to diagnosis was 8.8 ± 7.0 months, mean time of evolution of the disease of 7.5 ± 7.1 years. 80.8percent were in remission, 50percent had specific antibodies. Hypertension was found in 28.8percent of the patients and 23.1percent had interstitial pneumonia. Arthritis was present in 96.2percent. 26.9percent had specific Jo1 antibodies and 21.2percent had Ro 52. Conclusions: Urban female patients in the fourth decade of life predominated, the most frequent specific antibodies found was anti-Jo-1, associated with the presence of interstitial lung disease(AU)

Síndrome de Sjögren primario que debuta con polimiositis mitocondrial, neuropatía axonal y parálisis hipopotasémica: reporte de caso / Onset of primary Sjögren syndrome with mitochondrial polymyositis, axonal neuropathy, and hypokalaemic paralysis: Case report

RESUMEN El síndrome de Sjögren es una entidad multisistémica de naturaleza autoinmune, clásicamente considerada una exocrinopatía debido a la alta frecuencia de síntomas secos (queratoconjuntivitis seca, xerostomía) como resultado de infiltración poliglandular por linfocitos autorreactivos. Sin embargo, menos del 10% de estos pacientes puede iniciar con manifestaciones extraglandulares severas, traducidas en peores desenlaces a largo plazo. Se presenta el caso de una gestante que inició con síndrome de debilidad aguda proximal relacionada con miositis con enfermedad mitocondrial e hipopotasemia severa, en el contexto de acidosis tubular renal distal, como manifestación extraglandular de síndrome de Sjögren primario. Se discuten brevemente manifestaciones neurológicas de esta entidad, incluyendo aquellas secundarias a trastornos metabólicos precipitados por compromiso autoinmune.

ABSTRACT Sjögren's syndrome is a multisystemic autoimmune disorder. It is classically considered as an exocrine disease, given the high frequency of dry symptoms (keratoconjunctivitis sicca, xerostomia) as a result of poly-glandular infiltration by autoreactive lymphocytes. However, less than 10% of these patients can onset with severe extra-glandular manifestations, resulting in worse long-term outcomes. The case of a pregnant woman is presented, who debuted with acute proximal weakness syndrome related to myositis with mitochondrial pathology and severe hypokalaemia in the context of distal renal tubular acidosis, as an extra-glandular manifestation of primary Sjögren's syndrome. Neurological manifestations of this condition are briefly discussed, including those secondary to metabolic disorders precipitated by autoimmune compromise.

Infección por citomegalovirus en pacientes reumatológicos: análisis de un caso y revisión de la literatura / Cytomegalovirus infection in rheumatologic patients: a case report and literature review

El Citomegalovirus es un microorganismo capaz de generar infecciones severas en pacientes inmunosuprimidos. Existe abundante información respecto a la infección en pacientes inmunosuprimidos por VIH o en relación a trasplante de órganos sólidos o hematopoyéticos. No ocurre lo mismo con los pacientes portadores de enfermedades autoinmunes. Si bien la clínica puede ser inespecífica y dificultar la sospecha diagnóstica, la clave está en determinar al paciente de riesgo para la infección y así realizar un diagnóstico precoz. Se presenta el caso de una mujer de 56 años, portadora de una polimiositis de difícil tratamiento, que en un contexto de terapia en base a corticoides e inmunosupresores (azatioprina y metotrexato), desarrolla cuadro febril asociado a fatiga, cuyo estudio concluyó una infección por Citomegalovirus, tratado exitosamente con Valganciclovir.

Cytomegalovirus is a microorganism associated with severe infections in immunosuppressed patients. There is abundant information regarding infection in HIV immunosuppressed patients or in relation to solid or hematopoietic organ transplantation. The same does not happen with patients with rheumatic diseases. Although the clinic can be nonspecific and hinder diagnostic suspicion, the key is to determine the patient at risk for the infection and thus make an early diagnosis. We present a case of a 56-year-old woman with a difficult-to-treat polymyositis, who, in a context of corticosteroid and immunosuppressive agents (azathioprine and methotrexate), develops a fever associated with fatigue, whose study con-cluded an infection due to Cytomegalovirus, successfully treated with Valganci-clovir.

Miopatías inflamatorias idiopáticas: una mirada actualizada al diagnóstico y el manejo / Idiopathic inflammatory myopathies: a review

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of acquired immune-mediated diseases, which typically involve the striated muscle with a variable involvement of the skin and other organs. Clinically, they are characterized by proximal muscle weakness, elevation of muscle enzymes, myopathic changes on electromyography and an abnormal muscle biopsy. The different IIM have been classified according to their distinctive histopathologic features in dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and immune-mediated necrotizing myopathy (IMNM). Several myositis-specific antibodies are associated with the different phenotypes, as well as with different risk of neoplastic disease and systemic complications. The basis for the treatment of DM, PM, and IMNM is immunosuppression. For IBM there are only symptomatic treatments. Steroids, associated or not with other immunosuppressant drugs, are the first line of treatment. Biologic drugs will allow future individualized therapies. The 10-year survival of DM, PM and IMNM is 62 to 90%. The leading causes of death are neoplastic, lung and cardiac complications. IBM does not impair survival, although it affects the quality of life.

Síndrome de solapamiento: polimiositis-esclerodermia. Presentación de un caso / Polymyosis-Sclerosis Overlap Syndrome. Case report and review of literature

Introducción: en el síndrome de solapamiento o superposición existen simultáneamente manifestaciones clínicas o serológicas de dos o más enfermedades autoinmunes sistémicas. Afectan al 5 por ciento de la población con predominio en mujeres. Presentación del Caso: hombre de 48 años, sin antecedentes patológicos personales. Acude con dolor en ambas manos, inflamación en las articulaciones metacarpo e interfalángicas proximales, bilateral y simétrica, acompañada de rigidez matinal que duraba casi todo el día desde hace 4 meses. Se le diagnostica artritis reumatoidea y se inicia tratamiento que abandonó. Luego aparece un cuadro que es interpretado como un solapamiento por lo que es ingresado con un cuadro clínico florido. En los exámenes de laboratorio: las transaminasas, la creatín fosfoquinasa, el lactato deshidrogenasa, se encontraban elevadas. Por la clínica y los complementarios se diagnosticó Síndrome de Solapamiento de polimiosistis-esclerodermia. Se comenzó tratamiento con inmunosupesores. El paciente evolucionó desfavorablemente y falleció. Discusión: el diagnóstico de este caso se realizó por las manifestaciones clínicas como fascie esclerodérmica, signo de sal y pimienta, fibrosis de predominio distal en ambas manos, telangiectasias peribucales. Los diagnósticos diferenciales son las enfermedades autoinmunes sistémicas cuando se presentan como cuadros únicos. El tratamiento de primera línea y el pronóstico dependen de las enfermedades que se solapen(AU)

Introduction: In the syndrome of overlap or overlap there are simultaneously clinical or serological manifestations of two or more systemic autoimmune diseases. They affect 5 percent of the population with predominance in women. Case Presentation: A 48-year-old man with no personal pathological history. He came with pain in both hands, inflammation in the metacarpal and interphalangeal proximal joints, bilateral and symmetrical, accompanied by morning stiffness that lasted almost all day for 4 months. He was diagnosed with rheumatoid arthritis and started treatment that he abandoned. Then a picture appears that is interpreted as an overlap so it is entered with a florid clinical picture. In laboratory tests: transaminases, creatine phosphokinase, lactate dehydrogenase, were elevated. Clinical and complementary diagnosis of Polymyosis-Sclerosis Overlap Syndrome was diagnosed. Treatment with immunosupers was started. The patient evolved unfavorably and died. Discussion: The diagnosis of this case was made by clinical manifestations such as sclerodermic fascia, salt and pepper sign, predominantly distal fibrosis in both hands, perioral telangiectasias. Differential diagnoses are systemic autoimmune diseases when presented as single frames. First-line treatment and prognosis depend on overlapping diseases(AU)

Guidelines of the Brazilian Society of Rheumatology for the treatment of systemic autoimmune myopathies

Abstract Background: Recommendations of the Myopathy Committee of the Brazilian Society of Rheumatology for the management and therapy of systemic autoimmune myopathies (SAM). Main body: The review of the literature was done in the search for the Medline (PubMed), Embase and Cochrane databases including studies published until June 2018. The Prisma was used for the systematic review and the articles were evaluated according to the levels of Oxford evidence. Ten recommendations were developed addressing the management and therapy of systemic autoimmune myopathies. Conclusions: Robust data to guide the therapeutic process are scarce. Although not proven effective in controlled clinical trials, glucocorticoid represents first-line drugs in the treatment of SAM. Intravenous immunoglobulin is considered in induction for refractory cases of SAM or when immunosuppressive drugs are contra-indicated. Consideration should be given to the early introduction of immunosuppressive drugs. There is no specific period determined for the suspension of glucocorticoid and immunosuppressive drugs when individually evaluating patients with SAM. A key component for treatment in an early rehabilitation program is the inclusion of strengthbuilding and aerobic exercises, in addition to a rigorous evaluation of these activities for remission of disease and the education of the patient and his/her caregivers.

The Role of Autoantibodies in Idiopathic Inflammatory Myopathies

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune muscle diseases with systemic involvement. Patients with IIM present with varying degrees of muscle disease, cutaneous manifestations, and internal organ involvement. The diagnosis and classification of IIM is based primarily on the classification system composed of clinical features, laboratory value and muscle biopsy. In addition, the identification and characterization of myositis-related autoantibodies can help diagnosis and classification. Recently, many studies have also demonstrated that the physician can define the clinical syndromes, establish treatment strategy and predict outcomes based on the patients' myositis-specific autoantibodies (MSA) and myositis-associated antibodies (MAA) profiles. MSAs are found exclusively in IIMs and facilitate the identification of subsets of patients with relatively homogeneous clinical features. MAAs are frequently found in association with other MSA; however, they may also be detected in various connective diseases.

Neuromyositis: A Rare Extramuscular Manifestation of Dermatomyositis

Dermatomyositis (DM) and polymyositis (PM) are representative idiopathic inflammatory myopathies characterized by symmetric and progressive proximal muscle weakness. Especially, DM is identified by characteristic skin lesions and has many extramuscular manifestations including various cardiac abnormalities, interstitial lung disease, and malignancy. However, involvement of peripheral nervous system in DM/PM is very rare and less known. The term “Neuromyositis” was introduced by Senator in 1893 to describe the concomitant involvement of the peripheral nervous system in DM/PM. Since then, a very few cases of neuromyositis have been reported mainly in the United States and Europe. Therefore, the pathogenetic mechanism and disease progression are unclear. In recent years, a few more cases were reported in Asia, specifically, China and Japan; however, none in Korea. Here, we describe a case of DM-associated neuromyositis in a 42-year-old man in Korea and review previous publications through literature research.

Incidence and Prevalence of Idiopathic Inflammatory Myopathies in Korea: a Nationwide Population-based Study

BACKGROUND: This study aimed to estimate the incidence and prevalence of idiopathic inflammatory myopathies (IIM) and associated comorbidities in Korea from 2006 to 2015. METHODS: IIM between 2004 to 2015 were identified using the Korean National Health Insurance Service medical claim database. The case definition required more than one visit based on diagnostic codes including juvenile dermatomyositis (JDM), dermatomyositis (DM), or polymyositis (PM) and registration in the Individual Copayment Beneficiaries Program (ICBP) for rare and intractable diseases. IIM patients with a disease-free period of 24 months before the index date were defined as incident cases. The Elixhauser comorbidity score was calculated. RESULTS: Using the base case definition, 1,150 prevalent patients with IIM (117 JDM, 521 DM, 512 PM) were recorded in 2006 and 2,210 (130 JDM, 1,101 DM, 869 PM) in 2015. The prevalence was estimated at 2.3–4.0 (0.9–1.2 for JDM, 1.2–2.7 for DM, 1.4–2.1 for PM)/100,000 person-year (PY). We identified 218 incident cases of IIM in 2006 (18 JDM, 98 DM, 102 PM) and 191 cases (7 JDM, 83 DM, 101 PM) in 2015. The incidence was estimated at 2.9–5.2 (0.7–1.9 for JDM, 1.8–4.0 for DM, 1.6–3.0 for PM)/1,000,000 PY. The mean age (± standard deviation) of prevalent patients with IIM was 51.2 (± 16.9) years, and the percentage of women was 72.1%. More than two-thirds of patients (70.7%) had more than two comorbidities. Twenty percent of patients had interstitial lung diseases. CONCLUSION: In Korea, the incidence and prevalence of IIM were 2.9–5.2/1,000,000 PY and 2.3–4.0/100,000 PY, respectively.

Advances in epigenetic markers of dermatomyositis/polymyositis / 北京大学学报(医学版)

Idiopathic inflammatory myopathy (IIM) is a rare group of autoimmune diseases, characterized by chronic muscle weakness, muscle fatigue and infiltration of single nuclear cells in skeletal muscle. Its subtypes include dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and immune-mediated necrotizing myositis (IMNM), and the most common subtypes are DM and PM. PM is an autoimmune disease mainly manifested by muscle damage. When the skin is involved, it is called DM. The incidence of IIM was relatively low, which was 1.16-19 per million people/year, but the mortality was high and the prognosis was poor. The pathogenesis of IIM is still unclear. Previous studies suggest that both immune and non-immune mechanisms are involved in its pathogenesis, especially cellular and humoral immunity. In recent years, researchers have conducted a number of studies on the pathogenesis of IIM, especially in the study of DM/PM with the application of high-throughput biometrics. Epigenetics is a discipline that refers to the genetic phenomena of DNA methylation spectrum, chromatin structure state and gene expression spectrum transferred between cells without any changes in DNA sequence, including DNA methylation, chromatin modification and non-coding RNA changes. A large number of studies have shown that epigenetic modification plays an important role in many diseases, especially in cancer. Recent studies have also found a series of epigenetic markers related to the occurrence and development of DM/PM, mainly in the aspect of non-coding RNA changes, such as miR-10a, miR-206, etc. And there has also been some research on DNA methylation. However, no studies have been reported on whether chromatin modification is involved in the pathogenesis of DM/PM. The pathogenesis of DM/PM is complex and diverse. With the development of research, certain microRNAs (miRNAs) and long non-coding RNAs (lncRNAs) may become biological markers for the early diagnosis of DM/PM. Therefore, this paper mainly expounds the research progress of the biomarkers of DM/PM from the aspect of epigenetics.

Hyaluronic acid in dermatomyositis and polymyositis: relationship with disease and cutaneous lesions

Abstract: Background: There are scarce studies in the literature about hyaluronic acid in systemic autoimmune myopathies. Objectives: To analyze the serum level of hyaluronic acid in patients with dermatomyositis and polymyositis. Methods: Cross-sectional study, single-center, that evaluated hyaluronic acid in 18 dermatomyositis and 15 polymyositis (Bohan and Peter criteria), newly diagnosed, with clinical and laboratory activity, with no previous drug treatment. The patients were also age-, gender- and ethnicity-matched to 36 healthy individuals. The hyaluronic acid was analyzed by ELISA/EIA kit anti-hyaluronic acid. Results: There was a higher serum level of hyaluronic acid in patients with autoimmune myopathies, in relation to control group (P<0.05). Moreover, the serum level of this glycosaminoglycan was higher in dermatomyositis, when compared to polymyositis. Both groups were comparable with regard to demographic, clinical and laboratory data, except for the presence of skin lesions in the first group. Study limitations. The presence of hyaluronic acid in cutaneous lesions, particularly of patients with dermatomyositis, was not analyzed neither quantified. In addition, due to disease rarity and the establishment of strict inclusion and exclusion criteria, there was a small sample in the present study. Conclusions: As an example of others systemic autoimmune diseases, it is possible that the hyaluronic acid is involved in the pathogenesis of autoimmune myopathies, and particularly when associated with cutaneous lesions.

Prevalencia y caracterización de pacientes con polimiositis dentro de un grupo de enfermedades reumatológicas de un hospital universitario / Prevalence and characterization of patients with polymyositis within a group of rheumatic diseases in a university hospital

Introducción y objetivo: La polimiosistis es una miopatía inflamatoria idiopática cuya etiología es desconocida. Afecta principalmente al músculo esquelético, la piel y otros órganos internos. En cuanto a su frecuencia, pueden considerarse dentro del grupo de enfermedades raras debido a su baja prevalencia. Su incidencia anual media es de 2,1 a 7,7 casos nuevos por millón de habitantes. Métodos: Estudio observacional, descriptivo de corte transversal retrospectivo. La población estudio fueron pacientes con Polimiositis que ingresaron a un hospital universitario de Colombia entre el período comprendido entre los años 2012-2016 en pacientes mayores de 13 años que ingresen a la institución.

Patients with pure dermatomyositis/polymyositis and anti-PM/Scl autoantibody resembling anti-synthetase syndrome / Pacientes com dermatomiosite/polimiosite pura e autoanticorpo anti-pm/scl sememlhante à síndrome anti-sintetase

OBJECTIVE: The anti-PM/Scl autoantibody has been described in patients with scleromyositis. However, there are scant studies evaluating its prevalence and reactivity in dermatomyositis and polymyositis. METHOD: A cross-sectional, single center study evaluating the anti-PM/Scl autoantibody in 85 dermatomyositis and 32 polymyositis patients, without overlapping syndrome, was conducted between 2000 and 2016. Clinical data and complementary examinations were reviewed from electronic medical records with pre-parameterized information. RESULTS: The mean age of dermatomyositis and polymyositis patients was 41.1 and 42.8 years, respectively. The presence of anti-PM/Scl was observed in 5 (5.9%) dermatomyositis and 2 (6.3%) polymyositis patients. Two of these patients also had the anti-Ku antibody. The relevant clinical manifestations of these 7 patients were constitutional symptoms (100% of cases), muscular (100%), pulmonary (85.7%) and joint (71.4%) involvement, "mechanic hands" (85.7%), Raynaud phenomenon (85.7%) and plantar hyperkeratosis (85.7%). The 7 patients had relapses of disease activity, but at conclusion of the present study, 5 had complete clinical response and 2 complete remission of the disease. CONCLUSION: There is a low frequency of the anti-PM/Scl autoantibody in dermatomyositis and polymyositis patients. In addition, patients with this autoantibody exhibit a similar pattern of manifestations to that of antisynthetase syndrome.

OBJETIVO: O autoanticorpo anti-PM/Scl foi descrito em pacientes com escleromiosite. No entanto, há escassos estudos avaliando sua prevalência e reatividade em dermatomiosite (DM) e polimiosite (PM). MÉTODOS: Estudo transversal, num único centro, que avaliou o autoanticorpo anti-PM/Scl em 85 DM e 32 PM, sem síndrome de sobreposição, no período entre 2000 e 2016. Os dados clínicos e os exames complementares foram revisados a partir de registros médicos eletrônicos com informações pré-parametrizadas. RESULTADOS: A média de idade dos pacientes com DM e PM foi, respectivamente, de 41,1 e 42,8 anos. A presença de anti-PM/Scl foi observada em 5 (5,9%) DM e 2 (6,3%) pacientes com PM. Dois desses pacientes também possuíam o anticorpo anti-Ku. As manifestações clínicas relevantes desses 7 pacientes foram sintomas constitucionais (100% dos casos), envolvimento muscular (100%), pulmonar (85,7%) e articular (71,4%), "mãos mecânicas" (85,7%), fenômeno de Raynaud (85,7 %) e hiperqueratose plantar (85,7%). Os 7 pacientes apresentaram recidivas da atividade da doença, mas, no final do presente estudo, 5 apresentaram resposta clínica completa e 2 remissões completas da doença. CONCLUSÃO: Há uma baixa freqüência do autoanticorpo anti-PM/Scl em pacientes com DM e PM. Além disso, os pacientes com este autoanticorpo apresentam um padrão semelhante de manifestações para a síndrome da antisintetase.

Efeitos dos exercícios fisioterapêuticos nas miopatias inflamatórias idiopáticas: uma revisão sistemática / Effects of physiotherapheutic exercises in idiopathicg inflamatory myopathies: a systematic review / Efectos de los ejercicios fisioterapéuticos en las miopatías inflamatórias idiopáticas: una revisión sistemática

As miopatias inflamatórias idiopáticas são doenças musculares reumáticas raras, heterogêneas, progressivas, de caráter crônico, autoimune e de acometimento musculoesquelético e sistêmico. O objetivo deste artigo foi analisar estudos que abordassem os efeitos dos exercícios fisioterapêuticos nas miopatias inflamatórias idiopáticas. Tratou-se de uma revisão sistemática nas bases de dados PubMed, MEDLINE, SciELO e LILACS, de estudos nas línguas portuguesa, inglesa ou espanhola, publicados entre janeiro de 2006 e janeiro 2016, com base nos descritores primários "miosite", "polimiosite" ou "dermatomiosite" em cruzamento com os descritores secundários "fisioterapia" e "exercício". Os resultados apontaram predominância e grande interesse pela abordagem cardiorrespiratória dos indivíduos. Apesar disso, a literatura demonstrou carência nas fontes científicas que explorassem a abordagem fisioterapêutica nas disfunções osteomioarticulares causadas pelas miopatias inflamatórias idiopáticas, visto que é um conjunto de doenças que provocam, além do comprometimento sistêmico, sérios agravamentos no sistema musculoesquelético. Concluiu-se que um programa de exercícios aeróbicos e resistidos supervisionado, com intensidade moderada e duração de 12 semanas, parece ser uma estratégia segura e produz efeitos positivos sobre o condicionamento físico, a função física, a força e resistência muscular, a qualidade de vida e as atividades de vida diária em indivíduos com miopatias inflamatórias idiopáticas, tanto na fase aguda quanto na fase crônica, sem exacerbar os sintomas da doença ou causar aumento nos indicadores inflamatórios.

Idiopathic inflammatory myopathies are rare, heterogeneous, progressive, chronic-character, autoimmune and musculoskeletal and with systemic involvement rheumatic muscle diseases. The objective this article was to recruit the evidence from literature on the effects of physical therapy to inflammatory myopathies. This is a systematic review in the databases MEDLINE, SciELO and LILACS, of studies in Portuguese, English or Spanish, published between January 2006 and January 2016, based on the primary descriptors "myositis" "polymyositis" or "dermatomyositis" in intersection with the secondary descriptor "physical therapy specialty". The results showed predominance and great interest for the cardiorespiratory approach of individuals. Despite this, the literature has demonstrated a lack in scientific sources that explore the physiotherapeutic approach in osteomioarticular dysfunctions caused by idiopathic inflammatory myopathies, since it is a set of diseases that cause, in addition to systemic impairment, serious aggravations in the musculoskeletal system. In conclusion, an aerobic and resistance supervised exercise program, with moderate intensity and 12 weeks, seems to be a safe strategy and has positive effects on physical conditioning, physical function, muscle strength and endurance, quality of life and activities of daily living in patients with idiopathic inflammatory myopathies, both in acute and in chronic phase, without exacerbating the symptoms of the disease or causing an increase in inflammatory markers.

Las miopatías inflamatorias idiopáticas son enfermedades musculares reumáticas raras, heterogéneas, progresivas, de carácter crónico, autoinmune y con afectación musculoesqueléticas y sistémica. El objetivo de este artículo fue reclutar en la literatura evidencias sobre los efectos de la terapia física en las miopatías inflamatorias. Tratase de una revisión sistemática en las bases de datos MEDLINE, SciELO y LILACS, los estudios en portugués, inglés o español, publicados entre enero de 2006 y enero de 2016, con base en los descriptores primarios "miositis" "polimiositis" o "dermatomiositis" en la intersección con el descriptor secundario "fisioterapia". Los resultados apuntaron predominancia y gran interés por el abordaje cardiorrespiratorio de los individuos. A pesar de ello, la literatura demostró carencia en las fuentes científicas que explorasen el abordaje fisioterapéutico en las disfunciones osteomioarticulares causadas por las miopatías inflamatorias idiopáticas, ya que es un conjunto de enfermedades que provoca, además del compromiso sistémico, serios agravamientos en el sistema musculoesquelético. Concluyóse que un programa de ejercicios aeróbicos y ejercicios de resistencia supervisado, con intensidad moderada y 12 semanas, parece ser una estrategia segura y tener efectos positivos sobre la condición física, la función física, la fuerza y resistencia muscular, calidad de vida y actividades de la vida diaria en pacientes con miopatías inflamatorias idiopáticas, tanto en la fase aguda y cuanto en la fase crónica, sin exacerbar los síntomas de la enfermedad o provocar un aumento de marcadores inflamatorios.

Polimiosite no diagnóstico diferencial de dor torácica aguda / Polymyositis in the differential diagnosis of acute chest pain: case report

As miosites inflamatórias idiopáticas são um grupo heterogêneo de doenças de repercussão sistêmicas. A polimiosite é a manifestação fenotípica mais comum entre as miosites inflamatórias idiopáticas. A apresentação típica é dor e fraqueza progressiva simétrica da musculatura proximal e flexora do pescoço, com evolução de semanas a meses, associada à elevação dos marcadores de lesão muscular. O presente relato demonstra um quadro de polimiosite que se manifestou como dor torácica, acompanhado de aumento de creatinofosfoquinase e creatinofosfoquinase fração MB (CKT-MB), fazendo diagnóstico diferencial com síndrome coronariana aguda. O caso motivou a realização do levantamento bibliográfico, na busca de casos semelhantes e detalhamento dos critérios diagnósticos. Fizemos uma revisão comparando os aspectos clínicos importantes para diagnóstico diferencial das miopatias inflamatórias com os da síndrome coronariana aguda, além de discutir critérios diagnósticos da miopatias inflamatórias e seu tratamento.(AU)

Idiopathic inflammatory myositis is a heterogeneous group of diseases with systemic repercussions. Polymyositis is the most common phenotypic manifestation among idiopathic inflammatory myositis. The typical presentation is pain and progressive symmetrical weakness of the proximal and flexor musculature of the neck, with progression from weeks to months, associated with elevation of the markers of muscle injury. The present report demonstrates a picture of polymyositis that manifested as chest pain, with increased creatine kinase and creatine phosphokinase MB, making a differential diagnosis with acute coronary syndrome, which motivated the bibliographic survey in search for similar cases, and detailing of the diagnostic criteria. Thus, we performed a review comparing the clinical aspects that are important for a differential diagnosis of inflammatory myopathies with those of the acute coronary syndrome, and discussed the diagnostic criteria for inflammatory myopathies and their treatment.(AU)

Efeitos dos exercícios fisioterapêuticos nas miopatias inflamatórias idiopáticas: uma revisão sistemática / Effects of physiotherapeutic exercises in idiopathic inflammatory myopathies: a systematic review

As miopatias inflamatórias idiopáticas são doenças musculares reumáticas raras, heterogêneas, progressivas, de caráter crônico, autoimune e de acometimento musculoesquelético e sistêmico. O objetivo deste artigo foi analisar estudos que abordassem os efeitos dos exercícios fisioterapêuticos nas miopatias inflamatórias idiopáticas. Tratou-se de uma revisão sistemática nas bases de dados PubMed, MEDLINE, SciELO e LILACS, de estudos nas línguas portuguesa, inglesa ou espanhola, publicados entre janeiro de 2006 e janeiro 2016, com base nos descritores primários "miosite", "polimiosite" ou "dermatomiosite" em cruzamento com os descritores secundários "fisioterapia" e "exercício". Os resultados apontaram predominância e grande interesse pela abordagem cardiorrespiratória dos indivíduos. Apesar disso, a literatura demonstrou carência nas fontes científicas que explorassem a abordagem fisioterapêutica nas disfunções osteomioarticulares causadas pelas miopatias inflamatórias idiopáticas, visto que é um conjunto de doenças que provocam, além do comprometimento sistêmico, sérios agravamentos no sistema musculoesquelético. Concluiu-se que um programa de exercícios aeróbicos e resistidos supervisionado, com intensidade moderada e duração de 12 semanas, parece ser uma estratégia segura e produz efeitos positivos sobre o condicionamento físico, a função física, a força e resistência muscular, a qualidade de vida e as atividades de vida diária em indivíduos com miopatias inflamatórias