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.

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.

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.

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.

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.

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

Analysis of sexual function of patients with dermatomyositis and polymyositis through self-administered questionnaires: a cross-sectional study / Análise da função sexual de pacientes com dermatomiosite e polimiosite através de questionários autoaplicados: um estudo transversal

Abstract Introduction: To date, there are no descriptions in the literature on gynecologic and sexual function evaluation in female patients with dermatomyositis (DM) and polymyositis (PM). Objective: To assess sexual function in female patients with DM/PM. Patients and methods: This is a monocentric, cross-sectional study in which 23 patients (16 DM and 7 PM), with ages between 18 and 40 years, were compared to 23 healthy women of the same age group. Characteristics on sexual function were obtained by applying the questionnaires Female Sexual Quotient (FSQ) and Female Sexual Function Index (FSFI) validated for the Brazilian Portuguese language. Results: The mean age of patients was comparable to controls (32.7 ± 5.3 vs. 31.7 ± 6.7 years), as well as the distribution of ethnicity and socioeconomic class. As for gynecological characteristics, patients and healthy controls did not differ with respect to age at menarche and percentages of dysmenorrhea, menorrhagia, premenstrual syndrome, pain at mid-cycle, mucocervical secretion, and vaginal discharge. The FSQ score, as well as all domains of the FSFI questionnaire (desire, arousal, lubrication, orgasm and satisfaction), were significantly decreased in patients vs. controls, with 60.9% of patients showing some degree of sexual dysfunction. Conclusions: This was the first study to identify sexual dysfunction in patients with DM/PM. Therefore, a multidisciplinary approach is essential for patients with idiopathic inflammatory myopathies, in order to provide prevention and care for their sexual life, providing a better quality of life, both for patients and their partners.

Resumo Introdução: Até o presente momento, não há descrições na literatura da avaliação ginecológica e da função sexual em pacientes do sexo feminino com dermatomiosite (DM) e polimiosite (PM). Objetivos: Avaliar a função sexual em pacientes do sexo feminino com DM/PM. Casuística e métodos: Estudo transversal unicêntrico em que 23 pacientes (16 DM e sete PM), entre 18 e 40 anos, foram comparadas com 23 mulheres saudáveis, com a mesma faixa etária. As características sobre a função sexual foram obtidas por meio da aplicação dos questionários Female Sexual Quotient (FSQ) e Female Sexual Function Index (FSFI) validados para a língua portuguesa do Brasil. Resultados: A média de idade das pacientes foi comparável à dos controles (32,7 ± 5,3 vs. 31,7 ± 6,7 anos), assim como a distribuição de etnia e da classe socioeconômica. Quanto às características ginecológicas, pacientes e controles saudáveis não apresentaram diferenças em relação à idade na menarca e às porcentagens de dismenorreia, menorragia, síndrome pré-menstrual, dor no meio do ciclo, secreção mucocervical e corrimento vaginal. O escore de pontuação do FSQ, assim como todos os domínios do questionário do FSFI (desejo, excitação, lubrificação, orgasmo e satisfação), estavam significantemente diminuídos nas pacientes comparativamente com os controles, 60,9% das pacientes apresentavam algum grau de disfunção sexual. Conclusões: Este foi o primeiro estudo que identificou disfunção sexual nas pacientes com DM/PM. Assim, uma abordagem multidisciplinar é essencial para pacientes com miopatias inflamatórias idiopáticas para fornecer medidas de prevenção e cuidados para sua vida sexual e propiciar uma melhor qualidade de vida das pacientes e de seus parceiros.

Could C-reactive protein and erythrocyte sedimentation rate support monitoring of dermatomyositis and polymyositis activity? / É possível supor que a proteína c reativa e a hemossedimentação possam ser usadas para monitorar a atividade da dermatomiosite e da polimiosite?

OBJECTIVES: To evaluate serum levels of C-reactive protein and erythrocyte sedimentation rates in patients with untreated newly diagnosed dermatomyositis or polymyositis and their correlation with clinical and laboratory parameters. METHODS: A cross-sectional study including 48 consecutive patients with untreated newly diagnosed dermatomyositis and polymyositis reviewed between 2002 and 2015 was conducted. Fifty healthy subjects were enrolled as controls. RESULTS: Patients with dermatomyositis and polymyositis had higher levels of C-reactive protein and erythrocyte sedimentation rate than healthy controls, but these values were not associated with clinical or laboratory parameters of disease activity either for dermatomyositis or for polymyositis. Additionally, erythrocyte sedimentation rate values correlated with pulmonary involvement as evidenced through computer tomography imaging (OR 1.15; 95%CI 1.01-1.31) only in patients with polymyositis. CONCLUSIONS: Although elevated, C-reactive protein and erythrocyte sedimentation rate are not sensitive parameters for measuring clinical and laboratory activity of dermatomyositis nor for polymiositis. However, erythrocyte sedimentation rate may be a valid parameter for screening pulmonary involvement, particularly in patients with polymyositis.

OBJETIVOS: Avaliar os níveis séricos da proteína C reativa (PCR) e da velocidade de hemossedimentação (VHS) em pacientes recém-diagnosticados com dermatomiosite (DM) e polimiosite (PM), sem tratamento prévio, correlacionando-os com parâmetros clínico-laboratoriais. MÉTODOS: Estudo transversal que incluiu 48 pacientes consecutivos com DM e PM (critérios de Bohan e Peter) recém-diagnosticados, sem tratamento medicamentoso, no período de 2002 a 2015. Foram incluídos 50 indivíduos saudáveis como grupo controle. RESULTADOS: Os pacientes apresentaram níveis mais elevados de VHS e PCR comparativamente aos controles saudáveis. Estes valores, porém, não se correlacionaram com os parâmetros clínicos e laboratoriais da atividade da doença (DM e PM). Somente em pacientes com PM a VHS apresentou relação com acometimento pulmonar na tomografia computadorizada [OR 1,15 (IC 95% 1,01-1,31)]. CONCLUSÕES: Apesar de aumentadas, a PCR e a VHS não são parâmetros sensíveis para a mensuração da atividade clínica e laboratorial de DM e PM., No entanto, a VHS pode ter validade no rastreio do acometimento pulmonar, particularmente em pacientes com PM.

Miopatías inflamatorias con evolución recidivante / Relapses in inflammatory myopathies

La mayoría de los estudios de tratamiento de las miopatías inflamatorias son de corte y no permiten establecer su eficacia en largo plazo. En este trabajo, describimos el seguimiento de siete pacientes con miopatías inflamatorias, 5 polimiositis y 2 dermatomiositis. Determinamos su presentación, su seguimiento clínico mediante el examen físico, las enzimas musculares y la respuesta al tratamiento. Esta última la definimos como cursos de tratamiento, donde cada curso termina al aumentar los corticoides o al colocar una nueva medicación inmunosupresora debido al empeoramiento clínico o aumento sostenido de las enzimas musculares. El tratamiento instaurado puede remitir, controlar parcialmente, o fracasar en controlar la enfermedad en cuanto se normalicen, estabilicen, o no modifiquen respectivamente tanto la clínica como las enzimas musculares. Se analizaron 20 ciclos, en 14 se logró la remisión, en cinco se controló parcialmente y en uno fracasó el tratamiento. La remisión se logró en un tiempo promedio de 139 ± 98 días y el control en un promedio de 160 ± 100 días. Excepto en una ocasión, todos los ciclos de tratamiento, independientemente del que fuera, remitieron o controlaron los síntomas, pero en el tiempo todos los pacientes recidivaron en su enfermedad.

Most studies about treatment of inflammatory myopathies consist of cross-sectional analyses that do not assess long-term efficacy. In the present study we describe the follow-up of seven patients with inflammatory myopathies, 5 polymyositis and 2 dermatomyositis. We describe their clinical features, follow-up, muscle enzyme levels, and treatment responses. We define the latter as treatment cycles, every one of which end when steroid doses need to be increased or a new immunosuppressive drug has to be added because of clinical worsening or sustained increases in muscle enzyme levels. Treatment can cause remission, partially control, or fail in achieving myositis improvement when it normalizes, stabilizes, or does not affect muscle enzymes or clinical features, respectively. We analyzed 20 cycles, in which remission was achieved in 14 cases, partial control in 5 instances, and treatment failure in one case. Remission occurred after an average of 139 ± 98 days, whereas partial control took place in 160 ± 100 days. Except in one case, all treatment cycles controlled or remitted the symptoms. However, in all patients the illness recurred with time.

Polimiositis secundaria a tuberculosis en un niño de 11 años / Polymyositis secondary to tuberculosis in an 11-year-old boy

Resumen:La polimiositis es una de las miopatías inflamatorias idiopáticas. Tiene una incidencia mundial estimada en 4 casos por cada millón de habitantes al año. Es considerada un diagnóstico de exclusión y se ha establecido una asociación con la infección por micobacterias. Se reporta el caso de un niño de 11 años de edad con polimiositis secundaria a tuberculosis. La polimiositis tuvo una adecuada respuesta a los corticosteroides, pero estos no fueron necesarios después del diagnóstico de una infección pulmonar por Mycobacterium tuberculosis. El tratamiento antifímico, sin la terapia con esteroides, permitió una resolución de ambas patologías y la evolución favorable del paciente.

Abstract:Polymyositis is one of the idiopathic inflammatory myopathies. It has an estimated worldwide incidence of 4 cases per million population per year. It is considered an exclusion diagnosis and a relationship with mycobacterial infection has been established. This article reports the case of an 11-year-old boy with polymyositis secondary to tuberculosis. Polymyositis had an adequate response to corticosteroids, but these were not needed after the diagnosis of a Mycobacterium tuberculosis lung infection. Anti-tuberculosis treatment without steroid therapy, allowed a resolution of both conditions and the favorable outcome of the patient.

Coexistencia de neuropatía periférica secundaria a intoxicación crónica por plomo y polimiositis: reporte de caso / Co-existence of peripheral neuropathy secondary to lead poisoning and chronic polymyositis: Case report

Se reporta el caso de un hombre de 47 arios con intoxicación crónica por plomo, secundaria a exposición laboral de 5 años, con sintomatología típica de saturnismo. Consultó por debilidad muscular generalizada, disfagia y parestesias en extremidades. Se documentaron altos niveles de plomo en sangre, asociados a neuropatía periférica, confirmada por electromiografía, y disminución de la fuerza muscular en cintura escapular y pélvica (deltoides y vasto medial), así como atrofia de músculos del cuello (flexores y extensores) manifestada como cefaloparesia. Adicional al cuadro de saturnismo se diagnosticó miopatía inflamatoria con base en la elevación de enzimas musculares, miositis por resonancia magnética nuclear y biopsia muscular compatible, siendo, hasta donde se sabe, el primer reporte conocido de la coexistencia de estas 2 enfermedades

A case is presented on a 47-year-old man with chronic lead poisoning with typical symptoms after 5 years of occupational exposure. He consulted for generalised muscle weakness, early dysphagia, cephaloparesia, and paresthesias in upper and lower limbs. He also had atrophy and decreased proximal muscle strength (deltoid and medial vast) and in both flexor and extensor muscles of the neck. He had a history of high blood lead levels and peripheral neuropathy documented by electromyography. In addition to the diagnosis of lead poisoning, inflammatory myopathy was confirmed based on muscle enzyme elevation, muscular inflammation in magnetic resonance imaging, and typical findings in a muscle biopsy. To our knowledge, this is the first report where both conditions are documented in one patient

Autoantibodies in adult patients with idiopathic inflammatory myopathies in Buenos Aires

The idiopathic inflammatory myopathies(IIM) are a heterogeneous group of diseases of the skeletal muscle. On the basis of clinical, serologic and histological differences, they are classified in dermatomyositis (DM), polymyositis (PM), inclusion body myositis and immunomediated necrotizing myopathy. Autoantibodies directed against nuclear and cytoplasmic antigens are present with variable frequencies among studies. Myositis-specific antibodies (MSAs) are useful in IIM because they contribute to the diagnosis, help to identify different clinical subsets, and have prognostic value. This study aimed to explore the frequency of autoantibodies, especially MSAs, and their relationship with clinical features in adult patients with DM, PM and overlap syndrome. Medical records were reviewed. Myositis-associated antibodies (non-specific) and MSAs (anti Jo-1, PL-7, PL-12, Mi-2 and SRP) were measured using commercial kits. Twelve patients had MSAs, an overall frequency similar to those of international series, but PL-12 and Mi-2 were more frequent than Jo-1, which is the most frequently observed elsewhere. All five patients with Mi-2 had classical DM with a favorable response to treatment. Interstitial pneumonia (n: 4) and/or treatment-refractory disease (n: 3) were found in the presence of anti-PL-12, alone or associated with anti-SRP and/or Jo-1. In conclusion, the coexistence of AEM, a rare finding, was found in three patients. The presence of MSAs aided to the diagnosis of IIM, in particular in those patients without available or conclusive biopsy results.

Las miopatías inflamatorias idiopáticas (MII) comprenden un grupo heterogéneo de enfermedades adquiridas del músculo esquelético. Según sus características clínicas, serológicas e histológicas se las clasifica en dermatomiositis (DM), polimiositis (PM), miopatía necrotizante autoinmune y miositis por cuerpos de inclusión. Los anticuerpos específicos de miositis (AEMs) contribuyen al diagnóstico, permiten distinguir formas clínicas y tienen valor pronóstico. Con el objetivo de explorar la frecuencia de autoanticuerpos, en particular AEMs, y su relación con las características clínicas de las MII del adulto, se revisaron las historias clínicas de 25 pacientes con DM, PM y síndromes de superposición, asistidos en nuestro centro entre 1999 y 2013. La presencia de autoanticuerpos asociados a miositis (no específicos) y AEMs (anti Jo-1, PL-7, PL-12, Mi-2, SRP) se investigó utilizando kits comerciales. Doce pacientes presentaron AEMs, frecuencia global similar a la encontrada en series internacionales, pero a diferencia de lo observado en otros países, anti-PL-12 y anti-Mi-2 fueron más frecuentes que anti-Jo-1. Los cinco pacientes con anti-Mi-2 tuvieron DM clásica y buena evolución clínica. Anti-PL-12, ya sea solo o asociado a anti-SRP y/o anti-Jo-1, estuvo presente en pacientes con neumonía intersticial (n:4) y/o enfermedad refractaria al tratamiento (n: 3). En conclusión, la coexistencia de AEM, hallazgo raro, se encontró en tres pacientes. La presencia de AEMSs contribuyó al diagnóstico de MII, en particular en aquellos casos sin resultados concluyentes de biopsia de músculo.

Electrocardiographic changes in dermatomyositis and polymyositis / Alterações eletrocardiográficas em dermatomiosite e polimiosite

ABSTRACT Introduction: Cardiac involvement is frequent in inflammatory myopathies. Electrocardiogram (ECG) may show evidence of this involvement and its changes should be well-known and described. Objectives: Due to the lack of studies in the literature, we conducted an analysis of the ECG findings in patients with dermatomyositis (DM) and polymyositis (PM), comparing them with a control group. Methods: This cross-sectional study compared the ECG of 86 individuals with no rheumatic disorders (controls) with 112 patients (78 DM and 34 PM), during 2010 to 2013. The ECG findings between DM and PM were also compared. Results: Demographic characteristics, comorbidities and ECG abnormalities were similar between controls and patients (p > 0.05), except for a higher frequency of left ventricular hypertrophy (LVH) in patients (10.7% vs. 1.2%, p = 0.008). Demographic characteristics, comorbidities, clinical and laboratory manifestations, were also similar between the groups PM and DM, except for the presence of cutaneous lesions only in DM. One third of the patients had ECG abnormalities, which were more prevalent in PM than DM (50% vs. 24.4%, p = 0.008). LVH, left atrial enlargement, rhythm and conduction abnormalities were more frequent in PM than DM (p < 0.05 for all), especially the left anterior fascicular block. Conclusions: We showed distinct ECG changes between DM and PM and a higher frequency of LVH in patients compared to controls. Investigation of cardiac involvement should be considered even in asymptomatic patients, especially PM. Further studies are necessary in order to determine the correlation of ECG findings with other complementary tests, clinical manifestations, disease activity and progression to other cardiac diseases.

RESUMO Introdução: Acometimento cardíaco nas miopatias inflamatórias é frequente. Eletrocardiograma (ECG) pode mostrar indícios desse acometimento e suas alterações devem ser bem conhecidas e descritas. Objetivos: Devido à escassez de trabalhos na literatura, analisamos as alterações de ECG em pacientes com dermatomiosite (DM) e polimiosite (PM) e as comparamos com um grupo controle. Métodos: Este estudo transversal comparou ECGs de 86 indivíduos sem doenças reumatológicas (controles) com 112 pacientes (78 DM e 34 PM), de 2010 a 2013. Também comparamos os ECGs entre DM e PM. Resultados: Características demográficas, comorbidades e alterações de ECG foram semelhantes entre controles e pacientes (p > 0,05), exceto pela maior frequência de sobrecarga de ventrículo esquerdo (SVE) nos pacientes (10,7% vs. 1,2%; p = 0,008). Características demográficas, comorbidades, manifestações clínicas e laboratoriais também foram semelhantes entre os grupos PM e DM, exceto por lesões cutâneas apenas em pacientes com DM. Um terço dos pacientes apresentou alterações de ECG, que foram mais prevalentes em PM do que em DM (50% vs. 24,4%, p = 0,008). Sobrecarga de câmaras esquerdas (SCE), distúrbios do ritmo e da condução foram mais encontrados em PM do que em DM (p < 0,05 para todos), sobretudo o bloqueio divisional do ramo anterossuperior. Conclusões: Encontramos alterações distintas de ECG entre PM e DM e frequência aumentada de SVE em pacientes quando comparados com controles. Investigação do acometimento cardíaco nessas doenças deve ser considerada mesmo em pacientes assintomáticos, especialmente em se tratando de PM. Mais estudos são necessários para correlacionar os achados de ECG com outros exames complementares, manifestações clínicas, atividade das miopatias e evolução para outras doenças cardíacas.