Síndrome antisintetasa: reporte de un caso / Antisynthetase syndrome: a case repor

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.

Síndrome antisintetasa: reporte de un caso / Antisynthetase syndrome: a case report

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.

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

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

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

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

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.

The anti-synthetase syndrome in a type 1 diabetes

Anti-synthetase syndrome is an autoimmune disease associated with interstitial lung disease (ILD), dermatomyositis and polymyositis. It has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with dermatomyositis. 37-year-old male, known case of type 1 diabetes mellitus, came with complaints of: generalized anasarca, pain in both knees, ankles, wrist and small joints of fingers. He also had dyspnoea on exertion, chronic non-productive cough, and fever off and on, all for 1 month. Initially all involvement was attributed to diabetes. For joint pain an antinuclear antibodies (ANA) was sent. He turned out to be anti-Jo1, antibody positive. Rash on hands was diagnosed by dermatologist as, mechanic’s hand, hence diagnosed as an inflammatory myopathy with dermatomyositis anti-synthetase syndrome. Patient was successfully treated with immunosuppressants and supportive treatment and responded to tablet Prednisolone and Mycophenolate mofetil. The patient had one major and 2 minor criteria-ILD, arthritis and Mechanic’s hand and anti-Jo 1 antibody positive. Thus, diagnosed as anti-synthetase syndrome with type 1 diabetes mellitus.

Serie de casos clínicos de pacientes con Miopatía inflamatoria idiopática y Enfermedad pulmonar intersticial del registro de Miopatías inflamatorias idiopáticas de la Sociedad Argentina de Reumatología / Clinical case series of patients with idiopathic inflammatory myopathy and interstitial lung disease from the SAR's Registry of Idiopathic Inflammatory Myopathies

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).

Serie de casos clínicos de pacientes con Miopatía inflamatoria idiopática y Enfermedad pulmonar intersticial del registro de Miopatías inflamatorias idiopáticas de la Sociedad Argentina de Reumatología / Series of clinical cases of patients with idiopathic inflammatory myopathy and interstitial lung disease from the registry of idiopathic inflammatory myopathies of the Argentine Society of Rheumatology

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).

Cardiovascular risk factors among dermatomyositis patients with positive anti-Jo-1 antibody / 中国免疫学杂志

Objective:To assess the association between anti-Jo-1 antibody and cardiovascular risk factors among patients with dermatomyositis (DM).Methods:87 patients with DM were included from 2006 to 2013.Serum anti-Jo-1 antibody was measured by ELISA,and cardiovascular risk factors were evaluated.Multiple linear regression was used to assess the association between anti-Jo-1 antibody and cardiovascular risk factors in these patients.Results:The prevalence of hypertension,diabetes,hyperuricemia,and dyslipidemia were 16.09%,22.99%,10.34% and 67.82%,respectively.16.09% of DM patients had a positive anti-Jo-1 antibody and these patients had higher frequency of arthralgia/Raynaud's phenomenon/interstitial lung disease,and higher level of leukocyte and C reactive protein,while had lower level of serum uric acid (P<0.05).Multiple linear regression demonstrated that anti-Jo-1 antibody was closely associated with the level of C reactive protein and serum uric acid.Conclusion:Positive anti-Jo-1 antibody is associated with inflammation marker;however,the detailed mechanism remains further research.

Three cases of anti-TNF induced myositis and literature review / Três casos de miosite induzida pelo anti-TNF e revisão da literatura

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

Clinical features and imaging findings of interstitial lung disease in antisynthetase syndrome / 中华放射学杂志

Objective To investigate the clinical features and imaging findings of interstitial lung disease in antisynthetase syndrome(AS-ILD)and to compare the characteristics among AS specificities. Methods A total of 59 cases with AS-ILD at our hospital during the last 5 years were retrospectively reviewed, including anti-Jo1 positive in 37 cases and anti-PL7 positive in 6 cases and anti-PL12 positive in 6 cases and anti EJ positive in 10 cases. There were 14 males and 45 females aged (51 ± 12) years. The clinical features including myositis, arthritis, fever,"mechanic's hands", rash, proximal dysphagia, raynaud phenomenon were identified. Two radiologists evaluated the pattern, distribution and the ILD pattern of the lung abnormalities on HRCT findings. Based on the anti synthetase antibody positive subtype, 59 patients could be divided into four groups;then the differences of clinical features and HRCT findings between different subtypes were analyzed. X2 test was performed for the comparison of the differences between anti-Jo1 antibody positive and the other 3 group. Results (1)Myositis and arthritis were the most common AS manifestations, which were 61.02%(36/59)and 50.85%(30/59) respectively.(2)The lung abnormalities were predominantly basal(91.53%, 54/59) and peripheral(59.32%, 35/59). Ground-grass opacities(79.66%, 47/59)and reticulations(76.27%, 45/59) were found most frequently. Non-specific interstitial pneumonia (NSIP) was the most common HRCT pattern(64.41%, 38/59).(3)As compared with anti-Jo1 positive AS patients, anti-PL12 positive AS patients showed a high rate of traction bronchiectasis at diagnosis(83.33%, 5/6), while the difference was statistically significant(χ2=7.206, P=0.015). The prevalence of pericardial effusion(40.00%, 4/10) was significantly higher in the group of the anti-EJ positive AS patients than that with anti-Jo1 antibody AS(χ2=6.317, P=0.044). Conclusions Myositis and arthritis are the predominant clinical features. NSIP is the most common HRCT pattern in AS-ILD patients. There are some differences of signs among various subtypes, indicating that the difference of fibrosis in the lung and inflammatory reaction in the body being correlated with the AS specificities.

Clinical features and imaging findings of interstitial lung disease in antisynthetase syndrome / 中华放射学杂志

Objective To investigate the clinical features and imaging findings of interstitial lung disease in antisynthetase syndrome(AS-ILD)and to compare the characteristics among AS specificities. Methods A total of 59 cases with AS-ILD at our hospital during the last 5 years were retrospectively reviewed, including anti-Jo1 positive in 37 cases and anti-PL7 positive in 6 cases and anti-PL12 positive in 6 cases and anti EJ positive in 10 cases. There were 14 males and 45 females aged (51 ± 12) years. The clinical features including myositis, arthritis, fever,"mechanic's hands", rash, proximal dysphagia, raynaud phenomenon were identified. Two radiologists evaluated the pattern, distribution and the ILD pattern of the lung abnormalities on HRCT findings. Based on the anti synthetase antibody positive subtype, 59 patients could be divided into four groups;then the differences of clinical features and HRCT findings between different subtypes were analyzed. X2 test was performed for the comparison of the differences between anti-Jo1 antibody positive and the other 3 group. Results (1)Myositis and arthritis were the most common AS manifestations, which were 61.02%(36/59)and 50.85%(30/59) respectively.(2)The lung abnormalities were predominantly basal(91.53%, 54/59) and peripheral(59.32%, 35/59). Ground-grass opacities(79.66%, 47/59)and reticulations(76.27%, 45/59) were found most frequently. Non-specific interstitial pneumonia (NSIP) was the most common HRCT pattern(64.41%, 38/59).(3)As compared with anti-Jo1 positive AS patients, anti-PL12 positive AS patients showed a high rate of traction bronchiectasis at diagnosis(83.33%, 5/6), while the difference was statistically significant(χ2=7.206, P=0.015). The prevalence of pericardial effusion(40.00%, 4/10) was significantly higher in the group of the anti-EJ positive AS patients than that with anti-Jo1 antibody AS(χ2=6.317, P=0.044). Conclusions Myositis and arthritis are the predominant clinical features. NSIP is the most common HRCT pattern in AS-ILD patients. There are some differences of signs among various subtypes, indicating that the difference of fibrosis in the lung and inflammatory reaction in the body being correlated with the AS specificities.

Síndrome Antissintetase: relato de dois casos e revisão da literatura / Antisynthetase Syndrome: two case reports and literature review

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.

Síndrome antisintetasa / Antisynthetase syndrome

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.

The levels and clinical significance of serum B cell activating factor in Chinese patients with polymyositis or dermatomyositis / 中华内科杂志

Objectives To investigate serum levels of B cell activating factor(BAFF)in Chinese patients with polymyositis(PM)or dermatomyositis(DM),and analyze the correlation of BAFF with autoantibodies and clinical phenotypes.Methods Serum BAFF levels of 28 PM patients and 30 DM patients(study group),and 25 matched healthy controls(control group)were measured by ELISA.Serum anti-Jo-1 antibody levels were also measured by ELISA in all the subjects.The results of the two groups were compared by unpaired t test and the relevance was analyzed by Pearson's correlation analysis.Results Serum levels of BAFF in PM/DM patients were significantly higher compared to healthy controls(P =0.000),but there was no statistically significant difference between the PM and DM patients(P ＞ 0.05).Patients with interstitial lung disease(ILD)had significantly higher serum BAFF level than the patients without ILD(P =0.000)or the controls(P =0.000).Serum BAFF levels of patients with positive antinuclear antibody(ANA)were significantly higher than those with negative ANA(P =0.003).For patients with anti-Jo-1 antibodies,the serum BAFF levels were correlated with the serum concentration of anti-Jo-1 antibodies(r =0.799,P =0.006).Conclusions Serum levels of BAFF are increased in Chinese PM/DM patients.These findings indicate that BAFF may be possibly enrolled in the pathogenesis of PM/DM.Detecting serum BAFF levels could have some implication for the diagnosis and treatment of PM/DM.

Serum anti-Jo-1 antibody level correlates with disease activity in polymyositis and dermatomyositis / 中华风湿病学杂志

ObjectiveTo evaluate the correlation between serum anti-Jo-1 antibody level and myositis disease activity in polymyositis/dermatomyositis.MethodsAnti-Jo-1 antibody levels in serum from 148 polymyositis/dermatomyositis patients and 130 healthy controls were measured by both ELISA and immunoblot assay.Disease activity of the anti-Jo-1 antibody positive patients was assessed by the global myositis disease activity score (visual analogue score,VAS) established by the International Myositis Assessment and Clinical Studies(IMACS) Group.The correlation between disease activity and level of serum antiJo-1 antibody was assessed.Comparisons between groups were performed by x2 test or t test.ResultsThe positive rate of ELISA and EUROLINE was 24.3％(36/148) and 27.0％(40/148) respectively.Fever,ILD,arthritis/arthralgia were found to be more predominant in anti-Jo-1 antibody positive patients than those who were anti-Jo-1 antibody negative patients.There was significant positive correlation between the global myositis disease activity score (VAS) and serum level of anti-Jo-1 antibody (r=0.874,P=9.000).Serum antiJo-1 antibody levels,together with global disease activity,were significantly decreased in the 7 patients after treatment.ConclusionAnti-Jo-1 antibody level measured by ELISA is associated with disease activity of PM/DM,and could be a marker of disease activity.

Overlap Syndrome of Antisynthetase Syndrome and Rheumatoid Arthritis: A Case Report / 대한내과학회지

The antisynthetase syndrome is characterized by anti-Jo-1 antibody production, interstitial lung disease, inflammatory muscle disease, and, in many cases, fever, polyarthritis, Raynaud's phenomenon, and mechanic's hands. Joint signs and symptoms occur in up to 90% of patients with antisynthetase syndrome, occasionally as the initial manifestations. Although visual inspection of the hands shows changes that are highly suggestive of rheumatoid arthritis, notable differences exist. Antisynthetase syndrome is a predominantly nonerosive arthropathy with subluxations of the distal interphalangeal joints. It manifests as overlap syndrome with other connective tissue diseases. However, overlap syndrome of antisynthetase syndrome and rheumatoid arthritis is rare. We treated a 51-year-old male patient with overlap syndrome of antisynthetase syndrome and rheumatoid arthritis, and report the case with a review of the literature.

Polimiosite associada à arterite linfocítica do sistema nervoso central / Polymyositis associated with lymphocytic arteritis of the central nervous system

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.

A Case of Dermatomyositis with Secondary Organizing Pneumonia / 영남의대학술지

Dermatomyositis is characterized by progressive, symmetric, proximal muscle weakness and a nonsuppurative inflammatory myopathy of unknown etiology involving predominantly skeletal muscles. It is also characterized by typical skin lesions. Interstitial lung disease has a poor prognosis when it is associated with dermatomyositis. Organizing pneumonia is a disease in which granulation tissue fills the lumina of terminal and respiratory bronchioles and extends into the distal airspaces. The cryptogenic nature of the process is appreciated in that organizing pneumonia patterns of injury can be seen in secondary forms of the disease (secondary organizing pneumonia). Organizing pneumonia has been reported to occur in 5~10% in dermatomyositis-polymyositis patients. Anti-histidyl tRNA synthetase antibody (anti-Jo-1) is a predictive disease marker that is reported to occur in up to 70% of patients. We describe a 49-year-old male dermatomyositis patient who presented with organizing pneumonia and was found to have negative anti-Jo-1 antibody.

Overlap Syndrome of Amyopathic Antisynthetase Syndrome and Systemic Lupus Erythematosus: A Case Report / 대한류마티스학회지

Interstitial lung disease (ILD) with autoantibodies against antisynthetase may occur in the absence of clinically apparent myositis. We report 58-year-old woman who initially presented with dyspnea. The patient showed positive immunologic markers reflecting systemic lupus erythematosus, destructive polyarthritis, Raynaud's phenomenon, and ILD. There is no proximal muscle weakness in her symptoms. This syndrome was confirmed by positive antibodies to Jo-1. For the early diagnosis of this syndrome, the possibility of this syndrome including other connective tissue disease should be considered in patients presenting with symptoms related to ILD and positive anti-nuclear antibody.

Extraction and purification of Jo-1 antigen / 基础医学与临床

Objective To improve the purifying method of Jo-1 antigen from rabbit thymus used for detection of anti-Jo-1 antibody by dot-blotting immunoassay(DB).Methods The rabbit thymus glands were cut into pieces,homogenized and extracted by PBS.Total protein was precipitated by acetone to get acetone powder(RTAP).The RTAP was solved in PBS and separated by an by anti-Jo-1 IgG affinity column.Results 5～7 g RTAP was obtained from 100g rabbit thymus glands.There was 19%～24% of protein in RTAP.Jo-1 antigen was enriched around 1900 folds through affinity chromatography,with 2.5% recovery of antigenic activity.In this preparation,there were several bands on SDS-PAGE,but only one band about 50 ku,reacted with anti-Jo-1 antisera on immunoblotting.Dot-blotting also showed that the antigen only reacted with Jo-1 antisera.The purified Jo-1 antigen was not stable for long time,but the antigenic activity could maintain for a long time when there was MgCl2 in the solution.Conclusion Affinity chromatography was a simple and easy method for purifying Jo-1 antigen from rabbit thymus.The antigen purified by affinity chromatography could meet the requirement for detecting Jo-1 antibody bydot-blotting.