Clinico-pathological phenotypes of systemic sclerosis-associated myopathy: analysis of a large multicentre cohort.

OBJECTIVE: The objective of this study was to analyse the clinico-serological and histological phenotypes of patients with SSc with associated myopathy. METHODS: From November 2002 to September 2020, 52 patients with SSc underwent a muscle biopsy for suspected myopathy. We established two subgroups according to the histological findings based on the presence of isolated fibrosis or fibrosis together with significant inflammation. These patterns were designated as fibrosing and inflammatory, respectively. Clinical data, antibody profile, electrophysiologic studies, muscle biopsy findings and data regarding treatment, mortality and survival were compared between the two groups. RESULTS: Fourteen biopsies had a fibrosing pattern, whereas 26 showed an inflammatory pattern that could be classified (according to the predominant pattern) into DM (n = 7), necrotizing myopathy (n = 4) and non-specific myositis (n = 15). Additionally, 12 muscle biopsies were reported as neurogenic atrophy (n = 2), or normal muscle or minimal changes (n = 10). Compared with the inflammatory group, SSc patients with the fibrosing pattern presented a higher prevalence of ischaemic heart disease (38.5% vs 3.8%, P = 0.011), conduction abnormalities or arrhythmias (61.5% vs 26.9%, P = 0.036), anti-topo I antibodies (42.9% vs 11.5%, P = 0.044), greater median ESR (53.5 mm/h vs 32.5 mm/h, P = 0.013), with poor response to treatment and a higher mortality (42.9% vs 3.8%, P = 0.004) and lower cumulative survival (P = 0.035). CONCLUSIONS: Patients with SSc-associated myopathy require a comprehensive approach that encompasses clinical, serological and histopathological aspects, given their outcome predictive capacity. At least two different phenotypes can be drawn, considering clinico-pathological features. Significant differences are delineated between both a fibrotic and an inflammatory phenotype.

Myositis-specific antibodies and clinical characteristics in patients with autoimmune inflammatory myopathies: reported by the Argentine Registry of Inflammatory Myopathies of the Argentine Society of Rheumatology.

INTRODUCTION/OBJECTIVES: To describe clinical features in patients with inflammatory myopathies (IMs) from the Argentine Registry of Inflammatory Myopathies, and their relationship with myositis-specific antibodies (MSAs). METHODS: This cross-sectional study included 360 adult patients with dermatomyositis (DM), polymyositis (PM), and inclusion body myositis. Demographics, clinical, and serological characteristics were retrospectively recorded (2016-2019). MSAs were determined by immunoblotting. Patients who were positive for anti-Jo-1, Mi-2, and MDA5 were compared against a group of patients, taken as reference group, who were negative for all MSAs. RESULTS: Women 72%, median age at diagnosis was 47.3 years (18-82). The most frequent subtypes were DM (43.9%) followed by PM (30%).The most frequent MSAs were anti-Jo-1 (51/317), 16.1%; MDA5 (12/111), 10.8%, and Mi-2 (23/226), 10.2%. Anti-Jo-1 was associated (p < 0.05) with a higher frequency of chronic disease course, interstitial lung disease (ILD), arthritis, and mechanic's hands. Anti-Mi-2 was found in patients who had higher frequency of skin manifestations and higher CK values (p < 0.001). Patients with anti-MDA5 had normal or low CK levels. Anti-MDA5 was associated (p < 0.05) with skin manifestations, arthritis, and ILD. The rest of MSAs had frequencies lower than 8%. Anti-TIF1ϒ was found in eight DM patients and one had cancer. Anti-SRP was found in seven patients who had PM and elevated CK. CONCLUSION: Anti-Jo-1 was the most frequent MSA, and was associated with ILD; MDA5 was associated with CADM and ILD, and Mi-2, with classical DM. Despite the different prevalence with respect to other cohorts, the clinical characteristics for each MSA group were similar to the data reported in other studies. Key Points • This study describes the prevalence of MSAs in the Argentine Registry of IMs. • Anti-Jo-1 and anti-MDA5 were associated with ILD. • Anti-Mi-2 was the third most frequent MSA, associated with classical DM.

Características del empleo y la remuneración económica en el ejercicio de la Reumatología en diferentes distritos de Argentina / Characteristics of employment and economic remuneration in the practice of Rheumatology in different districts of Argentina

Resumen Introducción: la Reumatología es una especialidad clínica con elevada demanda y gran desigualdad en su ejercicio en Latinoamérica y el país. Objetivos: describir el estado del ejercicio de la Reumatología en nuestro país. Materiales y métodos: estudio observacional de corte transversal que recabó información a través de una encuesta online difundida por e-mail y de un código QR en el 54º Congreso Argentino de Reumatología. Resultados: se recibieron 127 respuestas; 86 (67,7%) fueron del sexo femenino, con una mediana de edad de 45 años. Ciento trece (113; 89%) encuestados eran especialistas en Reumatología, con 20 años (RIC 14-28) de ejercicio de la medicina y 13 años (RIC 6,5-20) de la especialidad. El 55,5% ejercía tanto en el ámbito público como privado, el 38,6% solo en el privado y el 7,6% únicamente en el público. Del total, el 84,3% trabajaba en dos o más lugares y el 21,2% en cuatro o más. Sesenta de los encuestados (60; 59,8%) recibía una remuneración mensual de entre 100.000 a 249.000 pesos (U$S: 501,68-1249,18) y 27 (23,1%) de menos de 100.000 pesos (U$S 501,68). Conclusiones: nuestro estudio muestra un alto porcentaje de profesionales que se desempeña en múltiples lugares de trabajo. Una quinta parte de ellos tenía remuneración baja.

Abstract Introduction: Rheumatology is a clinical specialty with high demand and great inequality in its practice throughout Latin America and our country. Objectives: to describe the state of the practice of Rheumatology in our country. Materials and methods: cross-sectional observational study that collected information through an online survey distributed by e-mail and through a QR code in the 54th Argentine Congress of Rheumatology. Results: 127 responses were received. Eighty-six (67.7%) were women, with a median age of 45 years. One hundred thirteen (89%) were specialists in Rheumatology, with 20 years (IQR 14-28) in the practice of medicine and 13 years (IQR 6.5-20) in the specialty. The 55.5% worked in both the public and private spheres, 38.6% only in the private sphere and 7.6% only in the public sphere. Of the total, 84.3% worked in 2 or more locations, with 21.2% working in 4 or more. In 60 (59.8%), the monthly remuneration was between 100,000 to 249,000 pesos (U$S: 501.68-1249.18) and in 27 (23.1%) it was less than 100,000 pesos (US$ 501.68). Conclusions: Our study shows that a high percentage of professionals who work in multiple places. One-fifth of respondents had low pay.

Características del empleo y la remuneración económica en el ejercicio de la Reumatología en diferentes distritos de Argentina / Characteristics of employment and economic remuneration in the practice of Rheumatology in different districts of Argentina

Introducción: la Reumatología es una especialidad clínica con elevada de-manda y gran desigualdad en su ejercicio en Latinoamérica y el país. Objetivos: describir el estado del ejercicio de la Reumatología en nuestro país. Materiales y métodos: estudio observacional de corte transversal que recabó información a través de una encuesta online difundida por e-mail y de un código QR en el 54º Congreso Argentino de Reumatología. Resultados: se recibieron 127 respuestas; 86 (67,7%) fueron del sexo femenino, con una mediana de edad de 45 años. Ciento trece (113; 89%) encuestados eran especialistas en Reumatología, con 20 años (RIC 14-28) de ejercicio de la medicina y 13 años (RIC 6,5-20) de la especialidad. El 55,5% ejercía tanto en el ámbito público como privado, el 38,6% solo en el privado y el 7,6% únicamente en el público. Del total, el 84,3% trabajaba en dos o más lugares y el 21,2% en cuatro o más. Sesenta de los encuestados (60; 59,8%) recibía una remuneración mensual de entre 100.000 a 249.000 pesos (U$S: 501,68-1249,18) y 27 (23,1%) de menos de 100.000 pesos (U$S 501,68). Conclusiones: nuestro estudio muestra un alto porcentaje de profesionales que se desempeña en múltiples lugares de trabajo. Una quinta parte de ellos tenía remuneración baja.

Introduction: Rheumatology is a clinical specialty with high demand and great inequality in its practice throughout Latin America and our country. Objectives: to describe the state of the practice of Rheumatology in our country. Materials and methods: cross-sectional observational study that collected information through an online survey distributed by email and through a QR code in the 54thArgentine Congress of Rheumatology. Results: 127 responses were received. Eighty-six (67.7%) were women, with a median age of 45 years. One hundred thirteen (89%) were specialists in Rheumatology, with 20 years (IQR 14-28) in the practice of medicine and 13 years (IQR 6.5-20) in the specialty.The 55.5% worked in both the public and private spheres, 38.6% only in the private sphere and 7.6% only in the public sphere. Of the total, 84.3% worked in 2 or more locations, with 21.2% working in 4 or more. In 60 (59.8%), the monthly remuneration was between 100,000 to 249,000 pesos (U$S: 501.68-1249.18) and in 27 (23.1%) it was less than 100,000 pesos (US$ 501.68). Conclusions: Our study shows that a high percentage of professionals who work in multiple places. One-fifth of respondents had low pay

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.

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.