External validation of the 2017 ACR/EULAR classification criteria for inflammatory myopathies in a Mexican cohort: Role of autoantibodies in the diagnosis and classification of patients with inflammatory myopathies.

OBJECTIVE: This retrospective study aimed to perform the first external validation of the ACR/EULAR classification criteria for inflammatory myopathy (IIM) in a Mexican dynamic cohort where the patients were evaluated with clinical and laboratory values. As secondary objectives, we presented the clinical characteristics of the patients and included antibodies other than anti Jo1 to evaluate their impact on our population. METHODOLOGY: This study included 70 patients with IIM and 70 patients with differential diagnoses of IIM, according to the absolute score of the classification criteria. We obtained sensitivity and specificity in the modality without biopsy, and as an exploratory analysis, we added other antibodies from the myositis extended panel. We analyzed the area under the curve (AUC) of three models: score without antibodies, with anti Jo1 and with any antibody. RESULTS: The ACR/EULAR criteria showed increased specificity and at least similar sensitivity to that of the original cohort (85% sensitivity and 92% specificity), with a cohort point of >55%. When we classified patients into definite, probable, possible, and no IIM categories, by adding the extended myopathy panel, 6 of the 10 patients initially classified as "no IIM" changed their classification to "Probable IIM" and 4 to "Definite IIM"; of the 16 patients classified as "probable IIM," 15 changed their classification to "Definite IIM." CONCLUSION: Considering the limitations of this study, we concluded that the 2017 EULAR/ACR criteria for IIM classification are sensitive and specific for classifying patients with IIM in the Mexican population. Additionally, the addition of antibodies other than anti-Jo1 may improve performance in certain populations.

External validation of the 2017 ACR/EULAR classification criteria for inflammatory myopathies in a Mexican cohort: Role of autoantibodies in the diagnosis and classification of patients with inflammatory myopathies / Validación externa de los criterios de clasificación de la ACR/EULAR de 2017 para miopatías inflamatorias en una cohorte mexicana. Papel de los anticuerpos en el diagnóstico y la clasificación de pacientes con miopatías inflamatorias

Objective: This retrospective study aimed to perform the first external validation of the ACR/EULAR classification criteria for inflammatory myopathy (IIM) in a Mexican dynamic cohort where the patients were evaluated with clinical and laboratory values. As secondary objectives, we presented the clinical characteristics of the patients and included antibodies other than anti Jo1 to evaluate their impact on our population. Methodology: This study included 70 patients with IIM and 70 patients with differential diagnoses of IIM, according to the absolute score of the classification criteria. We obtained sensitivity and specificity in the modality without biopsy, and as an exploratory analysis, we added other antibodies from the myositis extended panel. We analyzed the area under the curve (AUC) of three models: score without antibodies, with anti Jo1 and with any antibody. Results: The ACR/EULAR criteria showed increased specificity and at least similar sensitivity to that of the original cohort (85% sensitivity and 92% specificity), with a cohort point of >55%. When we classified patients into definite, probable, possible, and no IIM categories, by adding the extended myopathy panel, 6 of the 10 patients initially classified as “no IIM” changed their classification to “Probable IIM” and 4 to “Definite IIM”; of the 16 patients classified as “probable IIM,” 15 changed their classification to “Definite IIM.” Conclusion: Considering the limitations of this study, we concluded that the 2017 EULAR/ACR criteria for IIM classification are sensitive and specific for classifying patients with IIM in the Mexican population. Additionally, the addition of antibodies other than anti-Jo1 may improve performance in certain populations.(AU)

Objetivo: Este estudio retrospectivo tuvo como objetivo realizar la primera validación externa de los criterios de clasificación ACR/EULAR para miopatía inflamatoria (MII) en una cohorte dinámica de pacientes mexicanos que fueron evaluados en consulta y con muestras de laboratorio. Como objetivos secundarios presentamos las características clínicas de los pacientes e incluimos anticuerpos distintos al anti-Jo1 para evaluar su impacto en nuestra población. Metodología: Este estudio incluyó a 70 pacientes con MII y 70 pacientes con diagnóstico diferencial de MII, según la puntuación absoluta de los criterios de clasificación. Obtuvimos la sensibilidad y la especificidad en la modalidad sin biopsia, y como análisis exploratorio añadimos otros anticuerpos del panel extendido de miositis. Analizamos el área bajo la curva (AUC) de tres modelos: puntuación sin anticuerpos, con anti-Jo1 y con cualquier otro anticuerpo. Resultados: Los criterios ACR/EULAR mostraron una mayor especificidad y una sensibilidad, al menos similar a la de la cohorte original (85% de sensibilidad y 92% de especificidad), con un punto de cohorte de >55%. Cuando clasificamos a los pacientes en las categorías de definitiva, probable, posible y sin MII, al agregar el panel ampliado de miopatía, 6 de los 10 pacientes clasificados inicialmente como «Sin MII» cambiaron su clasificación a «Probable MII» y 4 a «MII Definitiva»; de los 16 pacientes clasificados como «Probable MII», 15 cambiaron su clasificación a «MII Definitiva». Conclusión: Considerando las limitaciones de este estudio, concluimos que los criterios de 2017 de la EULAR/ACR para la clasificación de la MII son sensibles y específicos para clasificar a los pacientes con MII en la población mexicana. Además, la adición de anticuerpos que no sean anti-Jo1 puede mejorar la estadificación en ciertas poblaciones.(AU)

Clinical risk factors in patients with interstitial lung disease associated with anti-MDA5 autoantibodies / Factores de riesgo clínicos en pacientes con enfermedad pulmonar intersticial con anticuerpos anti-MDA5

Introduction The anti-MDA5-associated autoimmune disease represents a poorly understood entity. The study's objectives were to describe a cohort of interstitial lung disease (ILD) patients who were positive for anti-MDA5 autoantibody and identify clinical risk factors associated with survival. Methods This single-center cohort study included ILD patients positive for anti-MDA5 autoantibody. Baseline clinical features were registered, and survival analysis was performed to identify risk factors associated with worse survival. Results Fifty-three ILD-MDA5 positive patients were included; twelve died during follow-up due to rapidly progressive interstitial lung disease (RP-ILD). Dermatological signs of anti-MDA5 (Gottron papules, Gottron sign, palmar papules, V-neck sign, facial dermatomyositis rashes, and skin ulcers) were strongly associated with death secondary to RP-ILD (HR: 3.7, 95% CI: 1.02–13.35). Patients with dermatological signs were younger, had higher anti-MDA5 autoantibodies titers, more frequent inflammatory patterns in HRCT evaluation, and less fibrosis extent in HRCT. Conclusion Dermatological manifestation in ILD patients to anti-MDA5 autoantibodies are associated with RP-ILD and short-term fatal outcomes. Dermatological signs may identify a subgroup of ILD-positive to anti-MDA5 patients with a high risk of RP-ILD (AU)

Introducción La enfermedad autoinmune asociada a los anticuerpos anti-MDA5 es una entidad poco estudiada. Los objetivos de este estudio son describir una cohorte de sujetos con enfermedad pulmonar intersticial (EPI) positivos al anticuerpo anti-MDA5 e identificar los factores clínicos de riesgo asociados con la supervivencia. Métodos Estudio de cohorte de un solo centro de pacientes con EPI y positivos al anticuerpo anti-MDA5. Se registraron las características clínicas basales y se realizó un análisis de supervivencia para identificar los factores de riesgo asociados con la supervivencia. Resultados Se incluyeron 53 pacientes con EPI y positivos a anti-MDA5; 12 pacientes fallecieron por una enfermedad intersticial rápidamente progresiva (EPI-RP). Los signos dermatológicos asociados a anti-MDA5 (pápulas de Gottron, signo de Gottron, pápulas palmares, signo de la V del escote, eritema facial de dermatomiositis y úlceras cutáneas) se asociaron fuertemente con la EPI-RP (HR: 3,7, IC 95%: 1,02-13,35). Los pacientes con manifestaciones dermatológicas eran más jóvenes, tenían mayores títulos de anticuerpos anti-MDA5, tenían mayor frecuencia de patrones inflamatorios en la tomografía de tórax de alta resolución y menor extensión de la fibrosis en la TCAR. Conclusión Las manifestaciones dermatológicas en los pacientes con EPI positivos a anticuerpos anti-MDA5 están asociados a EPI-RP y a desenlaces fatales al corto plazo. Los signos dermatológicos pueden identificar un subgrupo de pacientes positivos a anti-MDA5 con mayor riesgo de EPI-RP (AU)

Arritmias en pacientes con miopatía miotubular ligada al cromosoma X / Arrhythmias in patients with X-linked myotubular myopathy

Introducción: La miopatía miotubular es una enfermedad muscular congénita causada por una mutación en el gen de la miotubularina (MTM1). La miopatía miotubular ligada al cromosoma X (XLMTM) afecta a los hombres con síntomas de aparición temprana como debilidad muscular, hipotonía y dificultad respiratoria. Hasta donde sabemos, la afectación cardíaca en estos pacientes no se ha descrito previamente, a diferencia de otros tipos de miopatías congénitas, como la miopatía nemalínica o la miopatía con cores. Casos clínicos: Presentamos dos casos clínicos de XLMTM que comenzaron con bradicardia sinusal grave o bloqueo auriculoventricular desde los primeros días de vida, con Holter patológico en ambos casos. Se descartó una afectación cardíaca primaria por estudios electrofisiológicos y se recuperó la frecuencia cardíaca normal con soporte respiratorio adecuado. Conclusión: Estos casos con bradicardia grave en una patología bien conocida, como la XLMTM, suponen un matiz en el diagnóstico diferencial habitual de las miopatías congénitas.(AU)

Introduction: Myotubular myopathy is a congenital muscle disease caused by a mutation in the myotubularin (MTM1) gene. The X-linked myotubular myopathy (XLMTM) affects males with early-onset symptoms such as muscle weakness, hypotonia, and respiratory distress. To our knowledge, cardiac involvement has not been previously described in this condition, in contrast to other types of congenital myopathies such as nemaline myopathy or core myopathy. Case reports: We report two clinical cases of XLMTM that started with severe sinus bradycardia or auriculoventricular block from the first days of life, with pathologic 24-hours Holter monitoring in both cases. A primary cardiac affection was excluded by electrophysiological studies and normal heart rate was recovered with proper respiratory support. Discussion: These cases with sever bradyarrhythmia in a well know pathology such the XLMTM represents a nuance on the usual differential diagnostics of congenital myopathies.(AU)

Clinical risk factors in patients with interstitial lung disease associated with anti-MDA5 autoantibodies.

INTRODUCTION: The anti-MDA5-associated autoimmune disease represents a poorly understood entity. The study's objectives were to describe a cohort of interstitial lung disease (ILD) patients who were positive for anti-MDA5 autoantibody and identify clinical risk factors associated with survival. METHODS: This single-center cohort study included ILD patients positive for anti-MDA5 autoantibody. Baseline clinical features were registered, and survival analysis was performed to identify risk factors associated with worse survival. RESULTS: Fifty-three ILD-MDA5 positive patients were included; twelve died during follow-up due to rapidly progressive interstitial lung disease (RP-ILD). Dermatological signs of anti-MDA5 (Gottron papules, Gottron sign, palmar papules, V-neck sign, facial dermatomyositis rashes, and skin ulcers) were strongly associated with death secondary to RP-ILD (HR: 3.7, 95% CI: 1.02-13.35). Patients with dermatological signs were younger, had higher anti-MDA5 autoantibodies titers, more frequent inflammatory patterns in HRCT evaluation, and less fibrosis extent in HRCT. CONCLUSION: Dermatological manifestation in ILD patients to anti-MDA5 autoantibodies are associated with RP-ILD and short-term fatal outcomes. Dermatological signs may identify a subgroup of ILD-positive to anti-MDA5 patients with a high risk of RP-ILD.

Characterization of patients with dermatomyositis in a university hospital in Colombia

Introduction: Dermatomyositis is an idiopathic inflammatory myopathy characterized by the presence of skin lesions; it is considered a heterogeneous disease, due to its clinical presentation, course, and prognosis. In Colombia there are few records that describe the clinical characteristics of these patients. Methods: Cross-sectional study. Medical records of patients who consulted a university hospital in Colombia between January 2004 and December 2019 were reviewed. The records were obtained using databases from the dermatology, rheumatology, dermatopathology, and electrophysiology units, and CIE10 diagnostic codes. Results: Seventy patients with a dermatomyositis diagnosis were found, 63 (90%) fulfilled the Bohan and Peter diagnostic criteria and 7 (10%) had amyopathic dermatomyositis, with an average age of 43 years (SD ± 15.3). Forty-eight were women (68.5%). The most frequent clinical signs were Gottron's papules 80%, periorbital violaceous (heliotrope) erythema with edema 78.5% (n = 55) and poikiloderma 75.7% (n = 53). The most frequently found systemic manifestations were dysphagia (21.4%, n = 15), interstitial lung disease (11.4%, n = 8), and pulmonary hypertension (8.5%, n = 6). Cancer was documented in 8.5% (n = 6) of patients. Conclusion: We showed clinical information of patients with dermatomyositis in a referral hospital in Colombia. The data obtained is consistent with information from other case series worldwide.

Introducción: La dermatomiositis es una miopatía inflamatoria idiopática que se caracteriza por presentar lesiones en la piel; por su presentación clínica, su curso y su pronóstico, se la considera una enfermedad heterogénea. En Colombia existen pocos registros que describan las características clínicas de los pacientes afectados por esta enfermedad. Métodos: Estudio descriptivo de corte transversal, se revisaron las historias clínicas de pacientes que consultaron a un hospital universitario en Colombia entre enero del 2004 y diciembre del 2019. Los registros se obtuvieron utilizando bases de datos de las unidades de dermatología, reumatología, dermatopatología, electrofisiología y códigos diagnósticos CIE10 asociados con dermatomiositis. Resultados: Se obtuvieron 70 pacientes con diagnóstico de dermatomiositis, 63 (90%) de los cuales cumplían criterios de clasificación de Bohan y Peter, en tanto que 7 (10%) presentaban dermatomiositis amiopática. El promedio de edad fue de 43 arios (DS ± 15,3); 48 fueron mujeres (68,5%); los signos clínicos más frecuentes fueron: pápulas de Gottron (80%, n = 56), eritema heliotropo (78,5%, n = 55) y poiquilodermia (75,7%, n = 53). Las manifestaciones sistêmicas más comúnmente encontradas fueron: disfagia (21,4%, n = 15), enfermedad pulmonar intersticial (11,4%, n = 8) e hipertensión pulmonar (8,5%, n = 6). Se documentó cáncer en el 8,5% (n = 6) de los pacientes. Conclusión: Se presenta información clínica de pacientes con dermatomiositis en un centro hospitalario de referencia en Colombia; los datos obtenidos concuerdan con la información de otros estudios de series de casos a escala mundial.

Myositis-associated Interstitial Lung Disease: Clinical Characteristics and Factors Related to Pulmonary Function Improvement: A Latin-American Multicenter Cohort Study.

BACKGROUND AND OBJECTIVES: ILD patients can be positive to highly specific autoantibodies of connective tissue diseases (CTD). Among them stand out myositis-specific and associated autoantibodies (MSA/MAA). There is limited knowledge about treatment response and prognosis of ILD patients positive to MSA/MAA (MSA/MAA-ILD). Our aim was to describe clinical, radiological and pulmonary function (PF) of MSA/MAA-ILD Latin-American patients and risk factors associated to PF at onset and long term follow up. METHODS: Multicentric retrospective study of MSA/MAA-ILD patients evaluated between 2016 and 2018 in 3 ILD clinics in Latin America. Clinical, functional and tomographic variables were described. Variables associated with poor baseline PF and associated with functional improvement (FI) were analyzed in a multivariate logistic regression model. RESULTS: We included 211 patients, 77.4% female, mean age 57 years old. Most frequent MSA/MAA were Ro-52 and Jo-1. Poor baseline PF was associated to ILD as initial diagnosis and NSIP/OP HRCT pattern. 121 patients were included in the follow up PF analysis: 48.8% remained stable and 33% had a significant FI. In multivariate analysis, OP pattern on HRCT was associated with FI. Systemic symptoms from the beginning and the absence of sclerodactyly showed a trend to be associated with FI. CONCLUSIONS: Worse baseline PF could be related to the absence of extra-thoracic symptoms and "classic" antibodies in CTD (ANA), which causes delay in diagnosis and treatment. In contrast, FI could be related to the presence of extra-thoracic signs that allow timely diagnosis and therapy, and more acute and subacute forms of ILD, such as OP pattern.

Myositis-associated Interstitial Lung Disease: Clinical Characteristics and Factors Related to Pulmonary Function Improvement: A Latin-American Multicenter Cohort Study / Enfermedad pulmonar intersticial asociada con miositis: Características clínicas y factores relacionados con la mejoría de la función pulmonar. Estudio latinoamericano multicéntrico de cohorte

Background and objectives: ILD patients can be positive to highly specific autoantibodies of connective tissue diseases (CTD). Among them stand out myositis-specific and associated autoantibodies (MSA/MAA). There is limited knowledge about treatment response and prognosis of ILD patients positive to MSA/MAA (MSA/MAA-ILD). Our aim was to describe clinical, radiological and pulmonary function (PF) of MSA/MAA-ILD Latin-American patients and risk factors associated to PF at onset and long term follow up. Methods: Multicentric retrospective study of MSA/MAA-ILD patients evaluated between 2016 and 2018 in 3 ILD clinics in Latin America. Clinical, functional and tomographic variables were described. Variables associated with poor baseline PF and associated with functional improvement (FI) were analyzed in a multivariate logistic regression model. Results: We included 211 patients, 77.4% female, mean age 57 years old. Most frequent MSA/MAA were Ro-52 and Jo-1. Poor baseline PF was associated to ILD as initial diagnosis and NSIP/OP HRCT pattern. 121 patients were included in the follow up PF analysis: 48.8% remained stable and 33% had a significant FI. In multivariate analysis, OP pattern on HRCT was associated with FI. Systemic symptoms from the beginning and the absence of sclerodactyly showed a trend to be associated with FI. Conclusions: Worse baseline PF could be related to the absence of extra-thoracic symptoms and “classic” antibodies in CTD (ANA), which causes delay in diagnosis and treatment. In contrast, FI could be related to the presence of extra-thoracic signs that allow timely diagnosis and therapy, and more acute and subacute forms of ILD, such as OP pattern.(AU)

Antecedentes y objetivos: Los pacientes con enfermedad pulmonar intersticial (EPI) pueden presentar positividad para autoanticuerpos específicos de enfermedades autoinmunes, como los anticuerpos mioespecíficos (AME) o mioasociados (AMA). Existe escasa información disponible sobre pronóstico y respuesta al tratamiento de estos pacientes. Nuestro objetivo fue describir las características clínicas, radiológicas, funcionales y pronósticas de una cohorte latinoamericana de pacientes con EPI y AME/AMA. Métodos: Estudio retrospectivo multicéntrico de una cohorte pacientes con EPI y AME/AMA, evaluados en tres centros latinoamericanos entre 2016 y 2018. Describimos variables clínicas, tomográficas y funcionales. Analizamos variables asociadas con valores funcionales bajos al inicio y mejoría funcional mediante regresión logística. Resultados: Incluimos 211 pacientes, 77,4% mujeres, con una media de edad de 57 años. Los anticuerpos más frecuentes fueron Ro-52 y Jo-1. Valores funcionales bajos al inicio se asociaron con la presencia de EPI desde el debut y con un patrón de neumonía intersticial no específica (NINE)/neumonía organizativa (NO) en la tomografía computarizada de alta resolución (TACAR). Se analizó la evolución funcional en 121 pacientes: 48% permanecieron estables y 33% presentaron mejoría. El patrón de NO en TACAR, se asoció significativamente con mejoría funcional, mientras que la presencia de EPI desde el debut y la ausencia de esclerodactilia mostraron una tendencia en el análisis multivariado. Conclusiones: Valores funcionales bajos al debut podrían asociarse con la ausencia de síntomas extratorácicos al inicio, por llevar a un diagnóstico y tratamiento tardíos. Por el contrario, la presencia de síntomas sistémicos al debut, junto con formas más agudas de EPI como NINE/NO podrían asociarse con mejoría funcional por permitir un diagnóstico y tratamiento precoz.(AU)

Rendimiento diagnóstico de las biopsias musculares en la población pediátrica: experiencia de un centro terciario / Diagnostic yield of muscle biopsies in pediatric population: a tertiary center experience

Introducción: La biopsia muscular es un examen importante en la investigación de enfermedades neuromusculares, aunque su rendimiento diagnóstico puede ser decepcionante.Objetivo: Analizar el rendimiento diagnóstico de las biopsias musculares en la población pediátrica. Pacientes y métodos: Se analizó retrospectivamente una base de datos de un laboratorio terciario de neuropatología para identificar a pacientes (mayores de 18 años) sometidos a biopsia muscular entre enero de 2015 y agosto de 2019. Se evaluaron los datos demográficos, la presentación clínica, el diagnóstico, el tratamiento y el seguimiento. Resultados: Se incluyó a 106 pacientes, de los que el 52,8% (n = 56) eran varones. La mediana de edad fue de 6 años (rango intercuartílico: 10 años). Los pacientes se dividieron en ocho grupos, según sospecha diagnóstica clínica: miopatías mitocondriales (n = 29), miopatías congénitas (n = 9), miopatías inflamatorias (n = 8), distrofias musculares (n = 7), valores elevados de creatincinasa en el suero (n = 7), miopatías metabólicas (n = 5), otros síntomas neuromusculares (n = 30) y múltiples sospechas clínicas (n = 11). La biopsia fue normal en 50 pacientes. De los restantes, 27 mostraron características diagnósticas específicas, y el 88,9% (n = 24) permitió un diagnóstico definitivo: distrofias musculares (n = 7), miopatías metabólicas (n = 5), miopatías congénitas (n = 4), miopatías inflamatorias (n = 4), miopatías mitocondriales (n = 3) y atrofia muscular espinal (n = 1). La histología llevó a un cambio de tratamiento en cuatro pacientes. La mediana de seguimiento fue de un año (rango intercuartílico: 2 años). Conclusiones: El rendimiento diagnóstico de biopsia fue del 22,6% y fue útil en la orientación diagnóstica o terapéutica en el 35,8%...(AU)

Introduction: Background and aim. Muscle biopsy is still an important exam on the investigation of neuromuscular diseases although data regarding its diagnostic yield can be disappointing. We aimed to analyze the diagnostic yield of muscle biopsies in the pediatric population. Patients and methods: We retrospectively analyzed a tertiary Neuropathology laboratory database to identify patients (<18 years old), submitted to muscle biopsy between January 2015 and August 2019. Demographics, clinical presentation, diagnosis, treatment, and follow-up were evaluated. Descriptive statistical analysis was performed. Results: One-hundred and six patients were included, 52,8% (n = 56) were male. Median age at biopsy was 6 years (IQR 10 years). Patients were divided into 8 groups, according to clinical diagnostic suspicion: mitochondrial myopathies (n = 29), congenital myopathies (n = 9), inflammatory myopathies (n = 8), muscular dystrophies (n = 7), raised CK values in serum (n = 7), metabolic myopathies (n = 5), weakness /other neuromuscular symptoms (n = 30) and multiple clinical suspicions (n = 11). Biopsy was normal in 50 patients. Of the remaining, 27 displayed specific diagnostic features, with 88,9% (n = 24) allowing a definite diagnosis: muscular dystrophies (n = 7), metabolic myopathies (n = 5), congenital myopathies (n = 4), inflammatory myopathies (n = 4), mitochondrial myopathies (n = 3) and spinal muscular atrophy (n = 1). Histology led to a change of treatment in 4 patients, all diagnosed with inflammatory myopathies. Median length of follow-up was 1 year (IQR 2 years). Conclusion: Biopsy diagnostic yield was 22,6%, and it was useful either in diagnostic or therapeutic approaches in 35,8%. Although advances of molecular techniques led to a decrease in muscle biopsy indications, it remains an important tool on the diagnosis of neuromuscular diseases.(AU)

Myositis-associated Interstitial Lung Disease: Clinical Characteristics and Factors Related to Pulmonary Function Improvement: A Latin-American Multicenter Cohort Study.

BACKGROUND AND OBJECTIVES: ILD patients can be positive to highly specific autoantibodies of connective tissue diseases (CTD). Among them stand out myositis-specific and associated autoantibodies (MSA/MAA). There is limited knowledge about treatment response and prognosis of ILD patients positive to MSA/MAA (MSA/MAA-ILD). Our aim was to describe clinical, radiological and pulmonary function (PF) of MSA/MAA-ILD Latin-American patients and risk factors associated to PF at onset and long term follow up. METHODS: Multicentric retrospective study of MSA/MAA-ILD patients evaluated between 2016 and 2018 in 3 ILD clinics in Latin America. Clinical, functional and tomographic variables were described. Variables associated with poor baseline PF and associated with functional improvement (FI) were analyzed in a multivariate logistic regression model. RESULTS: We included 211 patients, 77.4% female, mean age 57 years old. Most frequent MSA/MAA were Ro-52 and Jo-1. Poor baseline PF was associated to ILD as initial diagnosis and NSIP/OP HRCT pattern. 121 patients were included in the follow up PF analysis: 48.8% remained stable and 33% had a significant FI. In multivariate analysis, OP pattern on HRCT was associated with FI. Systemic symptoms from the beginning and the absence of sclerodactyly showed a trend to be associated with FI. CONCLUSIONS: Worse baseline PF could be related to the absence of extra-thoracic symptoms and "classic" antibodies in CTD (ANA), which causes delay in diagnosis and treatment. In contrast, FI could be related to the presence of extra-thoracic signs that allow timely diagnosis and therapy, and more acute and subacute forms of ILD, such as OP pattern.

Wooden breast and white striping: comparative occurrence in three poultry companies

ABSTRACT: To increase the amount of meat produced, researchers have promoted intensive genetic selection for growth rate and muscling and have improved nutrition and management conditions. However, there has been an increase in the number of reports of breast muscle myopathies observed in poultry processing plants, including white striping (WS) and wooden breast (WB). This study aimed to evaluate and to compare the occurrence of WS and WB myopathies in three poultry processing plants and to perform an anatomopathological characterization, including macroscopic and microscopic analyses. A total of 408,334 carcasses were condemned or downgraded due to the presence of WB or WS, which represents 0.73% of the total number of slaughtered animals during the evaluated period. WB was more frequent than WS, but the occurrence of each myopathy varied significantly according to each establishment. WB was more frequent in the establishment which includes only male flocks, an average age of 45 days, and an average live weight of 2775g (B). WS was more frequent in establishment with male, female and mixed flocks, average ages ranging from 41 to 44 days, and average live weight 1731g-2830g (A). It is probably related with specific condition of each poultry company, including genetics, age, nutrition and management conditions. Macroscopically, WB and WS lesions are characterized by hypertrophy and stiffness of the pectoralis major muscle. Under microscopy, the myopathies showed similarities regarding the detected histological abnormalities, characterized by a process of myodegeneration, although the connective tissue infiltrate was more severe in the breasts with WB than in those with WS myopathy. The results found in this study demonstrate that the rates of condemnation for these myopathies are high, vary significantly among the analyzed companies and may cause major economic losses for the productive sector in the region.

RESUMO: Para melhorar a quantidade de carne produzida, os pesquisadores têm promovido ao longo dos anos uma seleção genética intensiva para a taxa de crescimento e desenvolvimento de músculos, além de melhorias nas condições nutricionais, sanitárias e de manejo. No entanto, houve um aumento no número de relatos de miopatias dos músculos do peito observados em abatedouros-frigoríficos de aves, incluindo white striping (WS) e wooden breast (WB). O objetivo deste estudo foi avaliar a ocorrência de WB e WS em três abatedouros-frigoríficos de aves e realizar uma caracterização anatomopatológica, incluindo análises macroscópicas e microscópicas. Um total de 408.334 carcaças foi condenado (total ou parcialmente) devido à presença WB ou WS, o que representou 0,73% do número total de animais abatidos durante o período avaliado. Apesar de a ocorrência de WB ter sido maior do que a de WS, a frequência de cada uma variou significativamente de acordo com o estabelecimento. WB foi mais frequente no estabelecimento que incluía apenas lotes de machos, com média de 45 dias de idade e peso médio ao abate de 2775g (B). WS foi mais frequente no estabelecimento com lotes de machos, fêmeas e mistos, com idade entre 41 e 44 dias e peso médio ao abate entre 1731g-2830g (A). Isto pode ser justificado por condições específicas de cada empresa, incluindo genética, idade das aves, nutrição e condições de manejo. Macroscopicamente, as lesões de WB e de WS foram caracterizadas principalmente por hipertrofia e rigidez do músculo pectoralis major. À microscopia, ambas as miopatias mostraram semelhanças em relação às anormalidades histológicas detectadas, caracterizadas principalmente por um processo de miodegeneração. Nos músculos apresentando WB, o infiltrado de tecido conjuntivo foi mais intenso quando comparado àqueles com WS. Os resultados encontrados neste trabalho demonstram que os índices de condenação por essas miopatias são altos, variam significativamente entre as empresas analisadas e podem causar grandes perdas econômicas para o setor produtivo da região.

Miopatia mitocondrial e deficiência auditiva neurossensorial: estudo de caso / Mitochondrial myopathy and sensorineural hearing loss: case study

Resumo A miopatia mitocondrial é causada pela ausência e/ou insuficiência de uma enzina quaternária, L-carnitina, responsável por transportar ácidos graxos livres para a parte interna da mitocôndria. A função da mitocôndria é produzir energia, contribuindo para o bom funcionamento das células. A Lipidose Muscular é uma doença que provoca anomalias em enzimas que metabolizam gordura e por consequência causa acúmulo de toxinas de subprodutos com gordura nos tecidos. O objetivo deste trabalho é apresentar o estudo de caso da paciente B.D., 37 anos, diagnosticada com Lipidose Muscular aos seis anos, com deficiência de L-Carnitina e relatar o acompanhamento fonoaudiológico realizado em um serviço de saúde auditiva. A abertura de prontuário da paciente foi realizada em 05/03/1989. Foi prescrito pelo neurologista o uso contínuo de 2g/dia de L-carnitina. A mãe relatou que B.D. apresentava dificuldades em ouvir, pois era muito desatenta, o que foi mais evidente quando começou a frequentar a escola. Em 1988, a paciente foi diagnosticada com perda auditiva neurossensorial de grau moderado bilateral e começou a fazer uso de aparelhos de amplificação sonora individual retroauriculares em 1989. O desempenho escolar e comunicação melhoraram. Em 1998, passou a utilizar aparelhos tipo micro canal, o que a favoreceu esteticamente, promoveu melhora da localização sonora e maior ganho em altas frequências. Os limiares de audibilidade apresentaram uma leve piora e a paciente atualmente é pós-graduada e trabalha em uma grande instituição financeira. Conclui-se que o diagnostico neurológico e a intervenção fonoaudiológica precoces possibilitaram o adequado desenvolvimento de linguagem da paciente.

Abstract Mitochondrial myopathy is caused by the absence and/or insufficiency of L-carnitine, a quaternary enzyme responsible for transporting free fatty acids into the mitochondria. The primary function of the mitochondria is to produce energy, contributing to proper cell functioning. Muscular lipidosis causes abnormalities in enzymes that metabolize fat, resulting in the accumulation of harmful amounts of fats in tissues. The aim of this study was to present the case study of patient B.D., a 37-year-old woman diagnosed with muscular lipidosis with L-carnitine deficiency at 6 years old, and describe the speech-language follow-up performed at a hearing care clinic. The first entry in the patient's medical chart was on 03/05/1989, with continuous use of 2g/day of L-carnitine prescribed by a neurologist. The mother reported that B.D. had difficulty hearing and was inattentive, which became more evident when she started school. In 1988 the patient was diagnosed with moderate bilateral sensorineural hearing loss and began using behind-the-ear (BTE) hearing aids in 1989, after which her academic performance and communication improved. In 1998 she switched to Completely in Canal (CIC) hearing aids, which are more discreet, provided better sound localization and greater high frequency gain, although her hearing thresholds worsened slightly. She completed her graduate studies and currently works at a large financial institution. It was concluded that early neurological diagnosis and speech-language intervention enabled adequate language development in the patient.

Wooden breast and white striping: comparative occurrence in three poultry companies / Wooden breast e white striping: ocorrência em três empresas avícolas

To increase the amount of meat produced, researchers have promoted intensive genetic selection for growth rate and muscling and have improved nutrition and management conditions. However, there has been an increase in the number of reports of breast muscle myopathies observed in poultry processing plants, including white striping (WS) and wooden breast (WB). This study aimed to evaluate and to compare the occurrence of WS and WB myopathies in three poultry processing plants and to perform an anatomopathological characterization, including macroscopic and microscopic analyses. A total of 408,334 carcasses were condemned or downgraded due to the presence of WB or WS, which represents 0.73% of the total number of slaughtered animals during the evaluated period. WB was more frequent than WS, but the occurrence of each myopathy varied significantly according to each establishment. WB was more frequent in the establishment which includes only male flocks, an average age of 45 days, and an average live weight of 2775g (B). WS was more frequent in establishment with male, female and mixed flocks, average ages ranging from 41 to 44 days, and average live weight 1731g-2830g (A). It is probably related with specific condition of each poultry company, including genetics, age, nutrition and management conditions. Macroscopically, WB and WS lesions are characterized by hypertrophy and stiffness of the pectoralis major muscle. Under microscopy, the myopathies showed similarities regarding the detected histological abnormalities, characterized by a process of myodegeneration, although the connective tissue infiltrate was more severe in the breasts with WB than in those with WS myopathy. The results found in this study demonstrate that the rates of condemnation for these myopathies are high, vary significantly among the analyzed companies and may cause major economic losses for the productive sector in the region.(AU)

Para melhorar a quantidade de carne produzida, os pesquisadores têm promovido ao longo dos anos uma seleção genética intensiva para a taxa de crescimento e desenvolvimento de músculos, além de melhorias nas condições nutricionais, sanitárias e de manejo. No entanto, houve um aumento no número de relatos de miopatias dos músculos do peito observados em abatedouros-frigoríficos de aves, incluindo white striping (WS) e wooden breast (WB). O objetivo deste estudo foi avaliar a ocorrência de WB e WS em três abatedouros-frigoríficos de aves e realizar uma caracterização anatomopatológica, incluindo análises macroscópicas e microscópicas. Um total de 408.334 carcaças foi condenado (total ou parcialmente) devido à presença WB ou WS, o que representou 0,73% do número total de animais abatidos durante o período avaliado. Apesar de a ocorrência de WB ter sido maior do que a de WS, a frequência de cada uma variou significativamente de acordo com o estabelecimento. WB foi mais frequente no estabelecimento que incluía apenas lotes de machos, com média de 45 dias de idade e peso médio ao abate de 2775g (B). WS foi mais frequente no estabelecimento com lotes de machos, fêmeas e mistos, com idade entre 41 e 44 dias e peso médio ao abate entre 1731g-2830g (A). Isto pode ser justificado por condições específicas de cada empresa, incluindo genética, idade das aves, nutrição e condições de manejo. Macroscopicamente, as lesões de WB e de WS foram caracterizadas principalmente por hipertrofia e rigidez do músculo pectoralis major. À microscopia, ambas as miopatias mostraram semelhanças em relação às anormalidades histológicas detectadas, caracterizadas principalmente por um processo de miodegeneração. Nos músculos apresentando WB, o infiltrado de tecido conjuntivo foi mais intenso quando comparado àqueles com WS. Os resultados encontrados neste trabalho demonstram que os índices de condenação por essas miopatias são altos, variam significativamente entre as empresas analisadas e podem causar grandes perdas econômicas para o setor produtivo da região.(AU)

Inicio de dermatomiositis juvenil en un escolar de 7 años de edad / Debut of juvenile dermatomyositis in a 7-year-old schoolboy

La dermatomiositis es una enfermedad que afecta con mayor frecuencia a pacientes mayores de 60 años y preferiblemente del sexo femenino. Sin embargo, en algunas ocasiones aparece en la edad infantil. Se exponen los elementos medulares que permiten el diagnóstico de esta enfermedad. Se presenta el caso de una escolar de 7 años de edad que acude al servicio de emergencia con manifestaciones clínicas sugerentes de una dermatomiositis juvenil. Las manifestaciones clínicas y los resultados de los exámenes complementarios permitieron llegar al diagnóstico definitivo de la enfermedad. Fue necesario hacer el diagnóstico diferencial con otras afecciones que cursan con síntomas similares. La paciente comenzó a mostrar una evolución favorable, lo que motivó el alta hospitalaria con tratamiento para el hogar y seguimiento por consulta externa con la especialidad de Reumatología. Conclusiones: A pesar de presentar un patrón epidemiológico predominante en adultos mayores, la dermatomiositis puede aparecer en edades más tempranas. Aunque las manifestaciones clínicas orientan hacia su diagnóstico, este se confirma con la ayuda de determinados exámenes complementarios(AU)

Dermatomyositis is a disease that occurs more frequently in patients older than 60 years and preferably female; however, sometimes it occurs in children. To publicize the core elements that allow the diagnosis of this disease. The case of a 7-year-old schoolgirl who comes to the emergency service with clinical manifestations suggesting a juvenile dermatomyositis is presented. The clinical manifestations and the results of the complementary examinations allowed to reach the definitive diagnosis of the disease, it was necessary to make the differential diagnosis with other conditions that present with similar symptomatology. Conclusions: despite presenting a predominant epidemiological pattern in older adults, dermatomyositis may occur at earlier ages; although the clinical manifestations are oriented towards its diagnosis, it is confirmed with the help of certain complementary tests(AU)

Avances en el empleo del ultrasonido muscular en miopatías inflamatorias / Advances in the use of muscle ultrasound in inflammatory myopathies

Aunque los métodos de imágenes aún no se han introducido en los criterios de clasificación de la miositis, el uso de imágenes musculares en la evaluación de las miopatías inflamatorias idiopáticas (MII) ha crecido a lo largo de los años. Las diferentes técnicas de imagen han demostrado ser útiles, pero la RM sigue siendo el estándar de oro para la imagen muscular. Sin embargo, el alto costo y las contraindicaciones en algunos pacientes, hacen considerar otros métodos como el ultrasonido muscular. Esta revisión tiene el objetivo de ofrecer una visión general de las diferentes técnicas de ultrasonido que se han estudiado y proporcionar información a los reumatólogos sobre el papel actual del ultrasonido para diagnosticar las MII. Existen diversos factores que pueden influir en la medición de los parámetros musculares: estandarizar la configuración de la máquina, el plano de visión y la posición del paciente, ya que pueden afectar la medición de la intensidad del eco y el grosor muscular. En el caso de las miopatías inflamatorias influyen otros factores en los resultados de la imagen: sexo, edad, escala de grises de la imagen, cambios en la calidad muscular, intensidad del eco, grosor, tamaño y ecogenicidad muscular. La ecografía muscular es una herramienta próxima en la evaluación de los trastornos neuromusculares y las miopatías inflamatorias. Es fácilmente aplicable en diversos entornos clínicos, no tiene contraindicaciones y proporciona una alternativa rentable a otras modalidades de imágenes como la resonancia magnética(AU)

Although imaging methods have not yet been introduced into the myositis classification criteria, the use of muscle imaging in the evaluation of IIMs has grown over the years. Different imaging techniques have proven helpful, but MRI remains the gold standard for muscle imaging. In this review, the objective is to provide an overview of the different ultrasound techniques that have been studied and to provide information to rheumatologists about the current role of ultrasound in the field of IIM. Development: There are multiple factors that can influence the measurement of muscle parameters that must be considered. First, it is important to standardize the machine configuration, the plane of vision and the position of the patient, as these can affect the measurement of echo intensity and muscle thickness. Conventionally, a linear ultrasound probe is used with sufficient frequency (at least 6-12 MHz) to obtain images of the peripheral skeletal muscle. Magnetic resonance imaging remains the gold standard for muscle imaging. However, the role as a diagnostic tool in the field of IIMs has grown over the years, and the promising results of new advanced imaging techniques suggest that it has not yet reached its full potential(AU)

Métodos de diagnóstico en el estudio de las Miopatías Inflamatorias Autoinmunes: Datos del Registro Argentino de Miopatías Inflamatorias de la Sociedad Argentina de Reumatología / Diagnostic methods in the study of Inflammatory Myopathies. Data from the Argentine Register: Argentine Rheumatology Society

Las Miopatías Inflamatorias Autoinmunes (MI) comprenden un grupo de enfermedades heterogéneas con presentación y características clínicas variables. Se distinguen subtipos clínicos como Polimiositis (PM), Dermatomiositis (DM), Miositis por cuerpos de Inclusión (MCI), Miopatía Necrotizante Inmunomediada (MNIM), Miositis de los Síndromes de Superposición, formas juveniles de MI (DMJ), Síndrome Antisintetasa (SAS) y Miopatía Asociada a Cáncer (MAC). La presencia de anticuerpos séricos y el infiltrado inflamatorio en la biopsia de músculo sugiere que se trata de una condición autoinmune. Realizar el diagnóstico de las MI suele ser un desafío y las herramientas diagnósticas no siempre están disponibles en la práctica diaria. Se obtuvo información sobre la disponibilidad de estos métodos del Registro Argentino de Miopatías Inflamatorias. El estudio de enzimas musculares, Anticuerpos Antinucleares (ANA), anticuerpo anti-Jo-1 y la tomografía computada de tórax, estuvieron disponibles para la mayoría de los pacientes mientras que la Resonancia Magnética de musculo (RM), el estudio de difusión de monóxido de carbono (DLco) y la biopsia muscular se realizaron en menos del 50% de los casos. La determinación de otros anticuerpos específicos de miositis, de importancia en el diagnóstico y pronóstico de la enfermedad se realizó, en mayor parte, a través de un subsidio de la SAR.

The Idiopathic Inflammatory Myopathies (IIM) comprise a heterogeneous group of acquired muscle diseases classified as polymyositis (PM), dermatomyositis (DM), Inclusion Body Myositis (IBM), Immuno Mediated Necrotizing Myopathies (IMNM), Overlap Myositis (OM), juvenile myositis, Antisynthethase Syndrome (ASS) and cancer related myositis (CAM). The presence of myositis specific antibodies in the serum and autoantibodies against target antigens and inflammatory infiltrates in muscle tissue suggests the autoimmune condition of the disease. The diagnosis of inflammatory myopathies is often a challenge and the disposal of diagnostic tools are not always available in daily practice. Information on the accessibility of these methods was obtained from the Argentine Register of Myopathies. The study of muscle enzymes, ANA, anti-Jo-1 antibodies and chest tomography were easy to get to most patients while muscle MRI, lung diffusion capacity for carbon monoxide (DLco) and muscle biopsy were performed in less than 50% of cases. Other myositis specific antibodies, necessary for disease diagnosis and prognosis, were mostly done through a subsidy from the Argentine Rheumatology Society.

Métodos de diagnóstico en el estudio de las Miopatías Inflamatorias Autoinmunes: Datos del Registro Argentino de Miopatías Inflamatorias de la Sociedad Argentina de Reumatología / Diagnostic methods in the study of myopathies Inflammatory Autoimmune: Data from the Argentine Registry of Inflammatory Myopathies of the Argentine Society of Rheumatology

Las Miopatías Inflamatorias Autoinmunes (MI) comprenden un grupo de enfermedades heterogéneas con presentación y características clínicas variables. Se distinguen subtipos clínicos como Polimiositis (PM), Dermatomiositis (DM), Miositis por cuerpos de Inclusión (MCI), Miopatía Necrotizante Inmunomediada (MNIM), Miositis de los Síndromes de Superposición, formas juveniles de MI (DMJ), Síndrome Antisintetasa (SAS) y Miopatía Asociada a Cáncer (MAC).La presencia de anticuerpos séricos y el infiltrado inflamatorio en la biopsia de músculo sugiere que se trata de una condición autoinmune. Realizar el diagnóstico de las MI suele ser un desafío y las herramientas diagnósticas no siempre están disponibles en la práctica diaria. Se obtuvo información sobre la disponibilidad de estos métodos del Registro Argentino de Miopatías Inflamatorias. El estudio de enzimas musculares, Anticuerpos Antinucleares (ANA), anticuerpo anti-Jo-1 y la tomografía computada de tórax, estuvieron disponibles para la mayoría de los pacientes mientras que la Resonancia Magnética de musculo (RM), el estudio de difusión de monóxido de carbono (DLco) y la biopsia muscular se realizaron en menos del 50% de los casos. La determinación de otros anticuerpos específicos de miositis, de importancia en el diagnóstico y pronóstico de la enfermedad se realizó, en mayor parte, a través de un subsidio de la SAR.

The Idiopathic Inflammatory Myopathies (IIM) comprise a heterogeneous group of acquired muscle diseases classified as polymyositis (PM), dermatomyositis (DM), Inclusion Body Myositis(IBM), ImmunoMediated Necrotizing Myopathies, (IMNM), Overlap Myositis(OM), juvenile myositis, Antisynthethase Syndrome (ASS) and cancer related myositis(CAM).The presence of myositis specific antibodies in the serum and autoantibodies against target antigens and inflammatory infiltrates in muscle tissue suggests the autoimmune condition of the disease. The diagnosis of inflammatory myopathies is often a challenge and the disposal of diagnostic tools are not always available in daily practice. Information on the accessibility of these methods was obtained from the Argentine Register of Myopathies. The study of muscle enzymes, ANA, anti-Jo-1 antibodies and chest tomography were easy to get to most patients while muscle MRI, lung diffusion capacity for carbon monoxide (DLco%) and muscle biopsy were performed in less than 50% of cases. Other myositis specific antibodies, necessary for disease diagnosis and prognosis, were mostly done through a subsidy from the Argentine Rheumatology Society.

Neumopatía crónica secundaria al trastorno de la deglución en un paciente con miopatía mitocondrial / Chronic pneumopathy secondary to swallowing disorder in a patient with mitochondrial myopathy

RESUMEN Introducción: la enfermedad pulmonar crónica secundaria a la disfagia es una complicación frecuente en los pacientes con enfermedades neuromusculares. Las miopatías mitocondriales son un conjunto de enfermedades que pueden conducir a daño pulmonar progresivo, secundario al síndrome aspirativo crónico. Caso clínico: niño de 7 años con signos clínicos y radiológicos de enfermedad pulmonar crónica; además, con desnutrición crónica, debilidad muscular, disfonía y oculoparesia externa crónica multiplanar. Su padre tuvo síntomas similares desde la infancia y requirió alimentación con dieta espesa por trastorno de la deglución. Se confirma en el paciente la presencia de disfagia como la causa de la neumopatía crónica y se sospecha miopatía congénita hereditaria. En consecuencia, se realiza el diagnóstico de enfermedad mitocondrial con oculoparesia externa crónica, mediante la secuenciación del gen polimerasa gamma del ADN mitocondrial (POLG). Conclusiones: en los pacientes con neumopatía crónica se deben considerar las enfermedades neuromusculares en el diagnóstico diferencial. La miopatía mitocondrial con oculoparesia externa crónica progresiva, se asocia con trastorno de la deglución hasta en un 50 % de los casos. El diagnóstico temprano es importante para retardar el deterioro de la función pulmonar.

SUMMARY Introduction: Chronic lung disease secondary to dysphagia is a frequent complication in patients with neuromuscular diseases. Mitochondrial myopathies could lead to progressive lung damage due to chronic aspiration syndrome. Clinical case: Seven-year-old male with clinical and radiological signs of chronic lung disease, as well as low weight, weakness, dysphonia and multiplanar external oculoparesis. His father had similar symptoms during infancy and needed thickened liquid diet due to swallowing disorder. Dysphagia was confirmed as the cause of chronic lung disease and, therefore, hereditary congenital myopathy was suspected. Mitochondrial disease with chronic external oculoparesis was confirmed by molecular sequencing of the mitochondrial DNA gamma polymerase gene (POLG). Conclusion: Neuromuscular disorders may cause chronic lung disease. Mitochondrial myopathy with progressive chronic external oculoparesis is associated with swallowing disorder in 50 % of the cases. Early diagnosis is important to slow decline in lung function.

Sporadic inclusion body myositis: Diagnostic value of p62 immunostaining.

BACKGROUND AND OBJECTIVES: Sporadic inclusion body myositis (sIBM) diagnosis is frequently delayed or confused with another class of disorders, and misdiagnosis is common. Sometimes, we have problems diagnosing an sIBM in the early stages or predicting when a PM is going to become an sIBM. In this sense, we believe that p62 immunostaining could help clinicians. CASE REPORT: We report the case of a 61-year-old patient with sIBM who six years earlier had been diagnosed with polymyositis (PM). After muscle biopsies analyses, we showed the natural history of sIBM by p62 expression. RESULTS: When we looked for p62 aggregates retrospectively we could see small dotted p62 aggregates in the muscle fibres of the first muscle biopsy. Six years later, the patient presented with the typical clinical picture of sIBM, also the muscle biopsy was characteristic, with large p62 aggregates. CONCLUSIONS: Probably p62 immunostaining could help to distinguish PM patients that are going to become sIBM, but to date there has been no systematic study to clarify p62 utility in myositis.

Coexistence of anti-Jo1 and anti-signal recognition particle antibodies in a polymyositis patient. / Coexistencia de anticuerpos anti-histidil-tRNA-sintetasa y anti-partícula de reconocimiento de la señal en un paciente con polimiositis.

Idiopathic inflammatory myopathies are a heterogeneous group of potentially treatable myopathies. They are classified, on the basis of clinical and histopathological features, into four subtypes: dermatomyositis, polymyositis, necrotizing autoimmune myositis and inclusion-body myositis. Myositis-associated antibodies and myositis-specific autoantibodies are frequently found in patients with idiopathic inflammatory myopathies, and are useful in the diagnosis and classification. Anti-histidyl transfer RNA synthetase antibody is the most widely prevalent and is highly specific for polymyositis. Signal recognition particle antibody is also a specific autoantibody for polymyositis, but it is infrequent and rarely found in patients having other myositis-specific autoantibodies. We present a man with polymyositis who had both antibodies in serum, which is considered an extremely rare clinical situation. Here we analyze the clinical course and findings, and examine the effect of the coexistence and possible interaction on prognosis.