RESUMO

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.

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El síndrome antisintetasa es una miopatía inflamatoria idiopática (MII) de origen autoinmune, poco frecuente, que se caracteriza por la presencia de autoanticuerpos antisintetasa ARNt (generalmente anti-Jo1), asociado frecuentemente a miositis, enfermedad pulmonar intersticial, poliartritis, manos de mecánico y fenómeno de Raynaud. Se reporta el caso de una mujer de 45 años de edad que presenta este síndrome con características fenotípicas de dermatomiositis y responde de forma favorable luego de la administración del tratamiento con glucocorticoides asociado a metotrexato.

Anti-synthetase syndrome is a rare autoimmune inflammatory myopathy characterized by autoantibodies against tRNA synthetases (most commonly anti-Jo1) with clinical features that include myositis, interstitial lung disease, polyarthritis, mechanic's hands and Raynaud's phenomenon. We report a 45-year-old woman who presented with dermatomyositis phenotypical features and a significant improvement with corticosteroids and metotrexate treatment.

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RESUMO

El síndrome antisintetasa es una miopatía inflamatoria idiopática (MII) de origen autoinmune, poco frecuente, que se caracteriza por la presencia de autoanticuerpos antisintetasa ARNt (generalmente anti-Jo1), asociado frecuentemente a miositis, enfermedad pulmonar intersticial, poliartritis, manos de mecánico y fenómeno de Raynaud. Se reporta el caso de una mujer de 45 años de edad que presenta este síndrome con características fenotípicas de dermatomiositis y responde de forma favorable luego de la administración del tratamiento con glucocorticoides asociado a metotrexato.

Anti-synthetase syndrome is a rare autoimmune inflammatory myopathy characterized by autoantibodies against tRNA synthetases (most commonly anti-Jo1) with clinical features that include myositis, interstitial lung disease, polyarthritis, mechanic's hands and Raynaud's phenomenon. We report a 45-year-old woman who presented with dermatomyositis phenotypical features and a significant improvement with corticosteroids and metotrexate treatment.

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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)

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RESUMEN 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 daño de órganos en pacientes con miopatías inflamatorias idiopáticas. Método: 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 % (α=0,05) para relacionar la presencia de anticuerpos y el tipo de miopatía, así como la presencia de manifestaciones clínicas de miopatías inflamatorias idiopáticas. Resultados: Del total de pacientes estudiadas, 80,8 % fueron mujeres, 61,5 % de color de piel negra, 86,5 % 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, 80,8 % estaban en remisión, 50 % tenía anticuerpos específicos. La hipertensión arterial se encontró en 28,8 % de los pacientes y 23,1 % presentó neumonía intersticial. La artritis estuvo presente en 96,2 %, 26,9 % presentaron anticuerpos específicos Jo1 y 21,2 % Ro 52. Conclusiones: Predominaron los pacientes del sexo femenino, en la cuarta década de la vida, de procedencia urbana. Los anticuerpos específicos encontrado con más frecuencia fue el anti Jo-1, que se asoció a la presencia de neumopatía intersticial.

ABSTRACT 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 organ damage in patients with idiopathic inflammatory myopathies. Method: An observational, descriptive, cross-sectional study was carried out in 52 patients with diagnosis of idiopathic inflammatory myopathy, followed up in the protocolized service of Rheumatology at Hermanos Ameijeiras Clinical Surgical Hospital from January 2016 to January 2017. The qualitative variables were calculated with the percentages in each group. Pearson's Chi-square (Fisher's exact statistic) (95% 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 idiopathic inflammatory myopathies. Results: 80.8% were women of the total patients studied, 61.5% non-white skin color, 86.5% 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. 80.8% were in remission, 50% had specific antibodies. Hypertension was found in 28.8% of the patients and 23.1% had interstitial pneumonia. Arthritis was present in 96.2%. We found 26.9% had specific Jo1 antibodies and 21.2% Ro 52. Conclusions: Urban origin female patients predominated, in their fourth decade of life, the more frequent specific antibodies found was anti Jo-1, which was associated with the presence of interstitial lung disease.

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Antisynthetase syndrome (ASSD) is a rare multisystemic connective tissue disease affecting the skin, joints, muscles, and lungs, characterized by anti-aminoacyl transfer-RNA-synthetases (anti-tRNA) autoantibodies production, being anti-Jo1 the most frequent. We included one-hundred twenty-one ASSD patients and 340 healthy subjects (HS), and also, we divided the case group into anti-Jo1 and non-anti-Jo1. Two single nucleotide polymorphisms (SNPs) in the IL17A gene were evaluated. Anti-Jo1 was the most common anti-tRNA antibody in our cohort, and the most frequent tomographic pattern was non-specific interstitial pneumonia (NSIP). Anti-Jo1 ASSD patients had higher levels of creatine phosphokinase than the non-anti-Jo1 group. Significant differences in genotype frequencies with rs8193036/CC between anti-Jo1 vs. non-anti-Jo1 ASSD patients (p < 0.001), maintaining the association after Bonferroni correction (p = 0.002). Additionally, in the anti-Jo1 group vs. HS comparison, we found a statistically significant difference with the same SNP (p = 0.018, OR = 2.91, 95% CI = 1.15-7.35), maintaining the association after Bonferroni correction (p = 0.036). The rs8193036/CC genotype in IL17A is associated with ASSD patients with anti-Jo1. Also, anti-Jo1 and non-anti-Jo1 patients display differences in genotype frequencies.

RESUMO

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.

RESUMO

The antisynthetase syndrome (ASSD) is an autoimmune disorder characterized by myositis, arthritis, mechanic's hands, fever, Raynaud phenomenon, and interstitial lung disease (ILD). We aimed to evaluate single-nucleotide polymorphisms in the interleukin 1B (IL1B) gene and their association between ILD with antisynthetase autoantibodies, as well as IL-1ß serum levels. The most frequent antisynthetase autoantibody was anti-Jo1. The most frequent tomographic pattern was non-specific interstitial pneumonia, whereas in the anti-Jo1 subjects, it was organized pneumonia. Anti-Jo1 patients tend to have more significant arthritis, and Raynaud phenomenon have higher levels of creatinine phosphokinase. In the IL1B gene, the GG genotype and G allele of rs1143634 [odds ratio (OR) = 2.21 and OR = 2.60, respectively, p < 0.05] are associated with an increased risk, as well as with the dominant and recessive models (p < 0.05). This finding is maintained after logistic regression analysis adjusting for potential confounding variables (p < 0.05). Subjects with the rs16944/AG heterozygous genotype had higher serum levels of IL-1ß compared to homozygous (p < 0.05). In conclusion, rs1143634 is associated with a higher risk of ASSD. Also, the GA genotype is associated with higher levels of IL-1ß in ASSD patients.

RESUMO

Las Miopatías Inflamatorias Idiopáticas (MII) son un grupo heterogéneo de enfermedades que se caracterizan por debilidad muscular e inflamación subyacente en la biopsia muscular. Los principales órganos afectados son el músculo, la piel y también puede afectarse el pulmón. Se distinguen dentro de los subtipos clínicos como Polimiositis (PM), Dermatomiositis (DM), DM con la variante Dermatomiositis Clínicamente Amiopática (DMCA), el Síndrome Antisintetasa (SAS), la Miositis Necrotizante Inmunomediada, la Miositis por Cuerpos de Inclusión (MCI) y la Miositis Asociada a Neoplasia. La presencia de ciertos anticuerpos específicos y asociados predispone al desarrollo de manifestaciones clínicas, determinando el pronóstico de la enfermedad. Se presentan 4 pacientes del Registro de MII de la Sociedad Argentina de Reumatología (SAR) con estas características: un paciente con PM y anti Jo-1 positivo y tres pacientes con DM (uno con DMCA y anti-RO 52 y dos pacientes con anti-PL7 y anti-TIF1γ respectivamente).

Idiopathic Inflammatory Myopathies (MII) are a heterogeneous group of diseases characterized by muscle weakness and inflammation underlying muscle biopsy. The main organs affected are muscle, skin and the lung can also be affected. They are distinguished within clinical subtypes such as Polymyositis (PM), Dermatomyositis (DM), DM with the variant Clinically Amiopathic Dermatomyositis (DMCA), the Syndrome Antisynthetase (SAS), Immune-mediated Necrotizing Myositis, Body Myositis Inclusion (MCI) and Neoplasia-Associated Myositis. The presence of certain specific and associated antibodies predisposes to the development of clinical manifestations, determining the disease prognosis. 4 patients from the Registry of MII of the Argentine Society of Rheumatology (SAR) are presented with these characteristics: one patient with PM and anti Jo-1 positive and three patients with DM (one with DMCA and anti-RO 52 and two patients with anti-PL7 and anti-TIF1γ respectively).

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Las Miopatías Inflamatorias Idiopáticas (MII) son un grupo heterogéneo de enfermedades que se caracterizan por debilidad muscular e inflamación subyacente en la biopsia muscular. Los principales órganos afectados son el músculo, la piel y también puede afectarse el pulmón. Se distinguen dentro de los subtipos clínicos como Polimiositis (PM), Dermatomiositis (DM), DM con la variante Dermatomiositis Clínicamente Amiopática (DMCA), el Síndrome Antisintetasa (SAS), la Miositis Necrotizante Inmunomediada, la Miositis por Cuerpos de Inclusión (MCI) y la Miositis Asociada a Neoplasia. La presencia de ciertos anticuerpos específicos y asociados predispone al desarrollo de manifestaciones clínicas, determinando el pronóstico de la enfermedad. Se presentan 4 pacientes del Registro de MII de la Sociedad Argentina de Reumatología (SAR) con estas características: un paciente con PM y anti Jo-1 positivo y tres pacientes con DM (uno con DMCA y anti- RO 52 y dos pacientes con anti-PL7 y anti-TIF1γ respectivamente).

Idiopathic Inflammatory Myopathies (MII) are a heterogeneous group of diseases characterized by muscle weakness and inflammation underlying muscle biopsy. The main organs affected are muscle, skin and the lung can also be affected. They are distinguished within clinical subtypes such as Polymyositis (PM), Dermatomyositis (DM), DM with the variant Clinically Amiopathic Dermatomyositis (DMCA), the Syndrome Antisynthetase (SAS), Immune-mediated Necrotizing Myositis, Body Myositis Inclusion (MCI) and Neoplasia-Associated Myositis. The presence of certain specific and associated antibodies predisposes to the development of clinical manifestations, determining the disease prognosis. 4 patients from the Registry of MII of the Argentine Society of Rheumatology (SAR) are presented with these characteristics: one patient with PM and anti Jo-1 positive and three patients with DM (one with DMCA and anti-RO 52 and two patients with anti-PL7 and anti-TIF1γ respectively).

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Abstract Anti-tumor necrosis factor drugs are frequently preferred in the treatment of rheumatologic diseases and other inflammatory diseases. The development of myositis after using anti-tumor necrosis factor drugs is a rare clinical condition. Here we aimed to report cases who developed myositis after using anti-tumor necrosis factor drugs and review the current literature. We report two cases of rheumatoid arthritis and a case of ankylosing spondylitis developed idiopathic inflammatory myopathy following anti-tumor necrosis factor therapy. In conclusion, myositis could develop during anti-tumor necrosis factor therapy, so these patients should be evaluated carefully initially for myositis and should be closely monitored due to the potential for developing myositis in treatment process.

Resumo Os fármacos antifator de necrose tumoral (anti-TNF) são frequentemente preferidos no tratamento de doenças reumatológicas e outras doenças inflamatórias. O desenvolvimento de miosite após o uso de anti-FNT é uma condição clínica rara. Este estudo objetivou descrever casos de pacientes que desenvolveram miosite após o uso de anti-TNF e fazer uma revisão da literatura atual. Descrevem-se dois casos de artrite reumatoide (AR) e um caso de espondilite anquilosante (EA) que desenvolveram miopatia inflamatória idiopática após o tratamento com anti-TNF. Em conclusão, pode haver desenvolvimento de miosite durante o tratamento com anti-TNF, de modo que esses pacientes devem ser cuidadosamente avaliados inicialmente à procura de miosite e devem ser cuidadosamente monitorados em razão do potencial de desenvolvimento de miosite no processo de tratamento

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Anti-tumor necrosis factor drugs are frequently preferred in the treatment of rheumatologic diseases and other inflammatory diseases. The development of myositis after using anti-tumor necrosis factor drugs is a rare clinical condition. Here we aimed to report cases who developed myositis after using anti-tumor necrosis factor drugs and review the current literature. We report two cases of rheumatoid arthritis and a case of ankylosing spondylitis developed idiopathic inflammatory myopathy following anti-tumor necrosis factor therapy. In conclusion, myositis could develop during anti-tumor necrosis factor therapy, so these patients should be evaluated carefully initially for myositis and should be closely monitored due to the potential for developing myositis in treatment process.

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OBJETIVO: Determinar la relación entre satisfacción laboral y clima organizacional de docentes y administrativos de una institución de educación superior de la comuna de Chillán-Chile. MÉTODO: Estudio cuantitativo, correlacional, en 166 trabajadores. Se aplicó el Cuestionario de Satisfacción Laboral S20/23, el Instrumento para Clima Organizacional y un cuestionario para identificar antecedentes sociodemográficos y laborales de los participantes. RESULTADOS: Un 95% de los docentes y un 90,6% de los administrativos refirieron sentirse satisfechos labo ralmente. Un 80% de los docentes y un 72,7% de los administrativos manifestaron percibir un alto nivel de clima organizacional o ambiente de trabajo. Los funcionarios más satisfechos y que perciben un más alto nivel de clima organizacional son aquellos que tienen entre 15 a 29 años de antigüedad en la universidad y los que traba jan menos de 40 horas semanales. Los que tienen contrato de titular se encuentran más satisfechos laboralmente y los a contrata perciben un nivel de clima organizacional más alto. La correlación entre los puntajes globales de clima organizacional y satisfacción laboral fue estadísticamente significativa, tanto en docentes (r = 0,523; p < 0,001) y administrativos (r = 0,468; p < 0,001). CONCLUSIÓN: La percepción de un clima organizacional alto se asocia a un mayor nivel de satisfac ción laboral de docentes y administrativos.

OBJECTIVE: To determine the relationship between job satisfaction and the organizational climate of teachers and administrators of an insti tution of higher education in the commune of Chillán-Chile. METHOD: A quantitative, correlational study of 166 workers. The Labor Satisfaction Questionnaire S20 / 23, the Organizational Climate Instrument and a questionnaire were used to identify the sociodemographic and labor background of the participants. RESULTS: 95% of the teachers and 90,6% of the administrators said they felt satisfied with their work. 80% of the teachers and 72,7% of the administrative staff showed a high level of organizational climate or work environment. The most satisfied and highest-perceived employees are those who are between 15 and 29 years of age at university and those who work less than 40 hours a week. Those who have a contract are more satisfied and the contractor perceives a higher level of organizational climate. The correlation between the overall organizational climate and work satisfaction scores was statistically significant in both teachers (r = 0,523, p <0,001) and administrative (r = 0,468, p <0,001). CONCLUSION: The perception of a high organizational climate is associated to a higher level of job sat isfaction of teachers and administrators.

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Anti-tumor necrosis factor (anti-TNF) drugs are frequently preferred in the treatment of rheumatologic diseases and other inflammatory diseases. The development of myositis after using anti-TNF is a rare clinical condition. Here we aimed to report cases who developed myositis after using anti-TNF and review the current literature. We report two cases of rheumatoid arthritis (RA) and a case of ankylosing spondylitis (AS) developed idiopathic inflammatory myopathy following anti-TNF therapy. In conclusion, myositis could develop during anti-TNF therapy, so these patients should be evaluated carefully initially for myositis and should be closely monitored due to the potential for developing myositis in treatment process.

RESUMO

Antissintetase Syndrome (ASS) is characterized by myositis, Raunaud's phenomenon, fever, intertitial lung disease, mechanic's hands and arthropathy associated with the presence of antibodies against tRNA synthetase, especially anti-Jo-1. This article aims to review the literature on ASS and report two cases where the first is a patient with polymyositis who developed subluxation on the proximal interphalangeal joint of bilateral first right finger after a few years of the disease, associated with pulmonary manifestations and positive anti-JO-1. In the second case, we present a patient with dermatomyositis, who developed a subluxation of the two first fingers, anti-Jo1 positive and chest CT changes, but without clinical evidence of pulmonary involvement. These cases reveal the importance of performing early diagnosis. The authors describe two cases of this rare syndrome, emphasizing the severity of interstitial lung disease and arthritis.

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A Síndrome Antissintetase (SAS) é caracterizada por miosite, fenômeno de Raynaud, febre, doença pulmonar intersticial, artropatia e mãos de mecânico associados à presença de anticorpos contra a sintetase do RNAt especialmente anti-Jo-1. Este artigo tem como objetivo revisar a literatura sobre SAS e relatar dois casos, sendo o caso 1 de uma paciente com Polimiosite que desenvolveu, após alguns anos de doença, subluxação da articulação interfalangeana proximal do primeiro quirododáctilo direito, associada a manifestações pulmonares e anti-Jo-1 positivo. O caso 2 é de uma paciente com Dermatomiosite que evoluiu com subluxação dos dois primeiros quirodáctilos, anti-Jo-1 positivo e alterações pulmonares intersticiais na TC de tórax, porém assintomática. Esses casos demonstram a importância do diagnóstico precoce. Os autores descrevem dois casos dessa síndrome rara, enfatizando a sua gravidade do ponto de vista pulmonar e articular.

Antisynthetase Syndrome (ASS) is characterized by myositis, Raynaud's phenomenon, fever, interstitial lung disease, mechanic's hands and arthropathy associated with the presence of antibodies against tRNA synthetase, especially anti-Jo-1. This article aims to review the literature on ASS and report two cases where the first is a patient with polymyositis who developed subluxation on the proximal interphalangeal joint of bilateral first right finger after a few years of the disease, associated with pulmonary manifestations and positive anti-JO-1. In the second case, we present a patient with dermatomyositis, who developed a subluxation of the two first fingers, anti-Jo1 positive and chest CT changes, but without clinical evidence of pulmonary involvement. These cases reveal the importance of performing early diagnosis. The authors describe two cases of this rare syndrome, emphasizing the severity of interstitial lung disease and arthritis.

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El Síndrome Antisintetasa, es una enfermedad poco frecuente perteneciente al grupo de las miopatías inflamatorias de origen inmunológico. Su caracterización inmunológica es muy variable y de allí las distintas manifestaciones clínicas de su presentación y su difícil diagnóstico. Se presenta un paciente femenino de 42 años de edad, con diagnóstico de Artritis Reumatoide (AR), con 9/10 puntos por puntaje para AR por EULAR 2011, anti-CCP +, RF -, desde octubre de 2011, en tratamiento con Prednisona y Metotrexate. Acude en febrero de 2012 por presentar disnea progresiva y tos con expectoración verdosa. Recibe antibioticoterapia sin respuesta. Se realiza TC de Tórax dónde se evidencia fibrosis pulmonar y bronquiectasias por tracción, con imágenes en panal de abeja a predominio de segmentos inferiores y posteriores. Durante su estancia hospitalaria presenta debilidad muscular proximal con elevación de CK a 4.969 U/L. , se realiza biopsia de músculo, que reporta miopatía inflamatoria; electromiografía, con patrón característico de miopatía inflamatoria y perfil inmunológico, obteniéndose Anti-Jo1 positivo, 0,885 (Negativo < 0,250). En el contexto de un síndrome poliarticular inflamatorio, una enfermedad pulmonar intersticial y una miopatía inflamatoria proximal con Anti Jo-1 (+); se realiza diagnóstico definitivo de Síndrome Antisintetasa.

This is a rare disease, member of the inflammatory myopathies of immunological origin. It has a very variable immunologic profile which makes the diagnosis difficult. We present a female patient 42 years- old, with the diagnosis of Rheumatoid Arthritis (RA), with 9/10 points of EULAR 2011 Score for RA diagnosis and RF -, Anti CCP +, in October of 2011; her treatment at that time was prednisone and methotrexate. In February of 2012, she consulted to the emergency with dyspnea and productive cough. She received antibiotics with no response. The chest Computerized Tomography evidenced pulmonary fibrosis, traction bronchiectasis and honeycombe images in posterior and inferior segments of both lungs. During her hospitalization, she presented proximal muscular weakness with CK elevation to 4.969 U/L. The muscle biopsy, which concluded inflammatory myopathy, the electromyography had the characteristic pattern of inflammatory myopathy and the immunologic profile, with a positive Anti-Jo1 0,885 (Negative <0,250). In the context of a polyarticular syndrome, an interstitial lung disease and a proximal inflammatory myopathy with a positive Anti-Jo1, we made the final diagnosis of Antisynthetase syndrome.

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RESUMO

O presente artigo investiga, no processo de formação de um grupo, as relações que seus componentes estabelecem com o que a psicanálise freudiana intitula ideal do eu, articulando tal conceito às noções antropológicas de diferença e alteridade tal como trabalhadas por Baudrillard. As discussões são feitas a partir de dados obtidos em trabalho de campo realizado com adolescentes de uma escola pública da periferia do Distrito Federal, os quais participaram, durante um ano, de oficinas práticas de fotografia, texto literário e conversação livre, que foram compreendidas como oficinas de trocas simbólicas, em referência ao trabalho da linguagem que as atravessava. Durante os trabalhos das oficinas, procurou-se observar o processo de transformação do que era, de início, um mero amontoado de adolescentes, em um grupo constituído como tal. Busca-se, aqui, apresentar os resultados a que chegaram os jogos de rivalidade e de solidariedade que se estabeleceram durante as oficinas, dando ênfase aos acontecimentos que trouxeram à tona as tensões entre indivíduos e entre sub-grupos que se formaram em oposição um ao outro e que se relacionam ao narcisismo das pequenas diferenças e à recusa da alteridade. Tais oposições aparecem em várias dimensões das relações intergrupais, principalmente aquelas que dizem respeito à dimensão econômica e à dimensão etária.

This article investigates - in the process of group formation - the relationships that their members establish with the ideal self, linking this concept, created by Freudian psychoanalysis, to the anthropological notion of difference and otherness as proposed by Baudrillard. Discussions are made based on data obtained in a fieldwork with ten adolescents from a public school in the outskirts of the Federal District, who participated for one year, of practical workshops on photography, literary text and free conversations. Such practical workshops have been called workshops of symbolic exchanges, in reference to the language work that they contain. During the workshop sessions, we tried to observe the transformation of what was initially just a bunch of teenagers in a group constituted as such. We intend to present herein the results reached by the games of rivalry and solidarity established during the workshops, with emphasis on events that have stirred tensions between individuals and between sub-groups formed in opposition to each other. Such oppositions can be related to the so called narcissism of small differences and the refusal of otherness, because they appear in several dimensions of intergroup relations, especially those concerning the economic dimension and age of group members.

Este artículo investiga, en el proceso de formar un grupo, las relaciones que sus miembros establezcan con lo que el psicoanálisis freudiano del ideal de jo que, articulando un concepto a nociones antropológicas de la diferencia y la otredad como trabajado por Baudrillard. Las discusiones se hacen de los datos obtenidos en trabajo de campo realizado con adolescentes de una escuela pública en las afueras del Distrito Federal, que participó, durante un año de talleres prácticos de fotografía, texto literario y conversación libre, que se entendía como talleres de intercambio simbólico, en referencia a la labor de la lengua que la travesía. Durante los talleres, trató de observar que el proceso de transformación de lo que era al principio un mero revoltijo de adolescentes en un grupo constituido como tal. Búsqueda, aquí presentamos los resultados alcanzados por los juegos de rivalidad y de solidaridad que se establecieron durante los talleres, centrándose en los eventos que trajeron a las superficie las tensiones entre personas y entre subgrupos que han formado en oposición a los demás y que se relacionan con la negativa de la alteridad y el narcisismo de las pequeñas diferencias. Tales oposiciones aparecen en diversas dimensiones de las relaciones intergrupales, especialmente aquellos que se relacionan con la dimensión económica y soporte de tamaño.

Cet article examine, en train de constituer un groupe, des relations que ses membres établir avec ce que la psychanalyse freudienne est l'idéal de je, articuler cette notion aux notions anthropologiques de la différence et l'altérité comme travaillé par Baudrillard. Les discussions sont fabriquées à partir des données obtenues dans le travail de terrain effectué avec des adolescents d'une école publique à la périphérie de Distrito Federal, qui a participé, pour une année d'ateliers pratiques de la photographie, le texte littéraire et la conversation libre, qui ont été interprétées comme un ateliers d'échange symbolique, en ce qui concerne le travail de la langue que le passage à niveau. Au cours des ateliers, a cherché à observer que le processus de transformation de ce qui fut d'abord un simple fouillis d'adolescents dans un groupe constitué en tant que tel. Recherche, ici, présenter les résultats atteints par les jeux de rivalité et de la solidarité qui se sont installés au cours des ateliers, en mettant l'accent sur les événements qui a amené les tensions de surface entre individus et entre les sous-groupes qui ont formé opposition à l'autre et qui concernent le narcissisme des petites différences et le refus de l'altérité. Ces oppositions apparaissent sous divers aspects des relations intergroupes, notamment celles qui ont trait à la dimension économique et le support de la taille.

RESUMO

O presente artigo investiga, no processo de formação de um grupo, as relações que seus componentes estabelecem com o que a psicanálise freudiana intitula ideal do eu, articulando tal conceito às noções antropológicas de diferença e alteridade tal como trabalhadas por Baudrillard. As discussões são feitas a partir de dados obtidos em trabalho de campo realizado com adolescentes de uma escola pública da periferia do Distrito Federal, os quais participaram, durante um ano, de oficinas práticas de fotografia, texto literário e conversação livre, que foram compreendidas como oficinas de trocas simbólicas, em referência ao trabalho da linguagem que as atravessava. Durante os trabalhos das oficinas, procurou-se observar o processo de transformação do que era, de início, um mero amontoado de adolescentes, em um grupo constituído como tal. Busca-se, aqui, apresentar os resultados a que chegaram os jogos de rivalidade e de solidariedade que se estabeleceram durante as oficinas, dando ênfase aos acontecimentos que trouxeram à tona as tensões entre indivíduos e entre sub-grupos que se formaram em oposição um ao outro e que se relacionam ao narcisismo das pequenas diferenças e à recusa da alteridade. Tais oposições aparecem em várias dimensões das relações intergrupais, principalmente aquelas que dizem respeito à dimensão econômica e à dimensão etária.(AU)

This article investigates - in the process of group formation - the relationships that their members establish with the ideal self, linking this concept, created by Freudian psychoanalysis, to the anthropological notion of difference and otherness as proposed by Baudrillard. Discussions are made based on data obtained in a fieldwork with ten adolescents from a public school in the outskirts of the Federal District, who participated for one year, of practical workshops on photography, literary text and free conversations. Such practical workshops have been called workshops of symbolic exchanges, in reference to the language work that they contain. During the workshop sessions, we tried to observe the transformation of what was initially just a bunch of teenagers in a group constituted as such. We intend to present herein the results reached by the games of rivalry and solidarity established during the workshops, with emphasis on events that have stirred tensions between individuals and between sub-groups formed in opposition to each other. Such oppositions can be related to the so called narcissism of small differences and the refusal of otherness, because they appear in several dimensions of intergroup relations, especially those concerning the economic dimension and age of group members.(AU)

Este artículo investiga, en el proceso de formar un grupo, las relaciones que sus miembros establezcan con lo que el psicoanálisis freudiano del ideal de jo que, articulando un concepto a nociones antropológicas de la diferencia y la otredad como trabajado por Baudrillard. Las discusiones se hacen de los datos obtenidos en trabajo de campo realizado con adolescentes de una escuela pública en las afueras del Distrito Federal, que participó, durante un año de talleres prácticos de fotografía, texto literario y conversación libre, que se entendía como talleres de intercambio simbólico, en referencia a la labor de la lengua que la travesía. Durante los talleres, trató de observar que el proceso de transformación de lo que era al principio un mero revoltijo de adolescentes en un grupo constituido como tal. Búsqueda, aquí presentamos los resultados alcanzados por los juegos de rivalidad y de solidaridad que se establecieron durante los talleres, centrándose en los eventos que trajeron a las superficie las tensiones entre personas y entre subgrupos que han formado en oposición a los demás y que se relacionan con la negativa de la alteridad y el narcisismo de las pequeñas diferencias. Tales oposiciones aparecen en diversas dimensiones de las relaciones intergrupales, especialmente aquellos que se relacionan con la dimensión económica y soporte de tamaño.(AU)

Cet article examine, en train de constituer un groupe, des relations que ses membres établir avec ce que la psychanalyse freudienne est l'idéal de je, articuler cette notion aux notions anthropologiques de la différence et l'altérité comme travaillé par Baudrillard. Les discussions sont fabriquées à partir des données obtenues dans le travail de terrain effectué avec des adolescents d'une école publique à la périphérie de Distrito Federal, qui a participé, pour une année d'ateliers pratiques de la photographie, le texte littéraire et la conversation libre, qui ont été interprétées comme un ateliers d'échange symbolique, en ce qui concerne le travail de la langue que le passage à niveau. Au cours des ateliers, a cherché à observer que le processus de transformation de ce qui fut d'abord un simple fouillis d'adolescents dans un groupe constitué en tant que tel. Recherche, ici, présenter les résultats atteints par les jeux de rivalité et de la solidarité qui se sont installés au cours des ateliers, en mettant l'accent sur les événements qui a amené les tensions de surface entre individus et entre les sous-groupes qui ont formé opposition à l'autre et qui concernent le narcissisme des petites différences et le refus de l'altérité. Ces oppositions apparaissent sous divers aspects des relations intergroupes, notamment celles qui ont trait à la dimension économique et le support de la taille.(AU)

Assuntos

RESUMO

Complicações do Sistema Nervoso Central (SNC) raramente são descritas em miopatias inflamatórias idiopáticas. Os autores relatam o caso de uma paciente de 48 anos com diagnóstico de polimiosite com autoanticorpo anti-Jo-1 positivo que, após cinco anos de evolução, apresentou extensa lesão desmielinizante do SNC associada à arterite linfocítica.

Central Nervous System (CNS) complications in idiopathic inflammatory myopathies are seldom reported. The authors describe the case of a 48-year old female with polymyositis and positive anti-Jo-1 autoantibody who, after five years of evolution, developed extensive CNS demyelinating injury associated with lymphocytic arteritis.

Assuntos