Calidad de vida en pacientes con esclerosis sistémica en confinamiento por pandemia de COVID-19 en Argentina / Quality of life in patients with systemic sclerosis in confinement due to the COVID-19 pandemic in Argentina

Introducción: los pacientes con enfermedades reumáticas tienen una calidad de vida significativamente deteriorada. La pandemia por COVID-19 tuvo un notable impacto sobre la población y los sistemas de salud de todo el mundo. Objetivos: en este trabajo nos proponemos conocer el impacto de la pandemia en la calidad de vida de los pacientes con esclerosis sistémica (ES) y cómo fue el acceso a la atención médica. Materiales y métodos: mediante encuestas anónimas y digitales a pacientes durante julio y agosto de 2020 se evaluó la calidad de vida utilizando el cuestionario de calidad de vida de la esclerosis sistémica (SScQoL). Además, se realizaron preguntas para evaluar el acceso al sistema de salud durante ese período. Resultados: se encuestaron 300 pacientes con ES. La mediana de afectación de la calidad de vida según el cuestionario utilizado fue de 17 (9,25-22) y el dolor fue el dominio más afectado. El 29,33% no hizo los controles médicos. El 74,33% refirió haber tenido estudios médicos pendientes al inicio de la cuarentena y solo el 25% pudo realizarlos. Conclusiones: los pacientes con ES presentaron compromiso de la calidad de vida durante la pandemia y mostraron dificultades en el acceso al sistema de salud.

Introduction: patients with rheumatic diseases have a significantly impaired quality of life. The COVID-19 pandemic has had a significant impact on the population and health systems around the world. Objectives: to analyze the impact of the pandemic on the quality of life and access to medical care of patients with systemic sclerosis (SS). Materials and methods: through anonymous and digital surveys of patients during July and August 2020, quality of life was assessed using the Systemic Sclerosis Quality of Life Questionnaire (SScQoL). In addition, questions were asked to assess access to the health system during that period. Results: 300 patients with SS were surveyed. The median quality of life affectation according to the questionnaire used was 17 (9.25-22), with pain being the most affected domain. Twenty-nine percent did not attend their medical appointments, 74.33% reported having pending medical studies at the beginning of the quarantine, and only 25% could carry them out. Conclusions: patients with SS presented compromised quality of life during the pandemic and showed difficulties in accessing the health system.

Taller de concordancia en la evaluación de imágenes capilaroscópicas / Reliability of nailfold videocapillaroscopy workshop

Introducción: la capilaroscopia es un método no invasivo que permite observar la microvasculatura en el área periungueal. Los resultados informados pueden ser altamente variables entre distintos observadores. A lo largo del tiempo surgieron métodos cuantitativos y semicuantitativos para mejorar la reproducibilidad. Objetivos: conocer el nivel de acuerdo intra e interobservador al informar los diferentes patrones capilaroscópicos en individuos con diferente nivel de entrenamiento. Materiales y métodos: estudio de corte transversal. Participaron médicos reumatólogos especialistas y en formación que habían realizado previamente un curso virtual de capacitación en capilaroscopia. Recibieron 40 imágenes capilaroscópicas proyectadas en una presentación de PowerPoint y debían responder a través de un cuestionario digital. Se evaluó la concordancia de respuestas intra e interobservador. Resultados: se encontró un alto nivel de concordancia global con un kappa 0,66 IC 95% (0,63-0,70) p<0,0000. También en otros grupos como reumatólogos en formación: kappa 0,65 IC 95% (0,60-0,71) p=0,0000, y médicos reumatólogos: kappa 0,67 IC 95% (0,62-0,72) p=0,0000. Conclusiones: el nivel de concordancia encontrado fue globalmente alto, independientemente del nivel de entrenamiento de los profesionales, y de ser o no reumatólogo. La concordancia fue superior cuando se comparó a quienes tenían más de 4 años de experiencia en la realización de videocapilaroscopia.

Introduction: videoapillaroscopy is a non-invasive method that allows the observation of the microvasculature in the periungual area. Reported results can be highly variable between different observers. Over time, quantitative and semi-quantitative methods emerged to improve reproducibility. Objetives: to know the level of intra and interobserver agreement when reporting the different capillaroscopic patterns in individuals with different levels of training. Materials and methods: cross section study. Specialist rheumatologists and those in training who had previously completed a virtual capillaroscopy training course participated. They received 40 capillaroscopic images projected in a PowerPoint presentation and had to issue their response through a digital questionnaire. Concordance of intra and interobserver responses was evaluated. Results: a high level of global agreement was found with a kappa 0.66 CI 95% (0.63-0.70) p<0.0000, also in other groups such as rheumatologists in training: kappa 0.65 CI 95% (0.60-0.71) p=0.0000, physicians rheumatologists: kappa 0.67 95% CI (0.62-0.72) p=0.0000. Conclusions: the level of agreement found was globally high, regardless of the level of training of the professionals, and whether or not they were a rheumatologist. Concordance was higher when compared to those who had more than 4 years of experience performing videocapillaroscopy.

Factor reumatoide en vasculitis ANCA asociadas / Rheumatoid factor in ANCA associated vasculitis

Introducción: las pruebas de factor reumatoide (FR) se utilizan principalmente para el diagnóstico de artritis reumatoidea (AR), sin embargo, también pueden estar presentes en otras enfermedades. Su significado clínico en contexto de vasculitis ANCA asociadas (VAA) todavía es incierto. El objetivo de este estudio fue establecer el significado clínico y pronóstico del FR en pacientes con VAA. Materiales y métodos: se realizó un estudio observacional, retrospectivo. Se evaluaron pacientes con diagnóstico de VAA (granulomatosis con poliangitis -GPA-, poliangitis microscópica -PAM- y granulomatosis eosinofílica con poliangitis -GEPA-), mayores de 18 años, con FR solicitado antes del inicio del tratamiento. Se recolectaron los siguientes datos: edad, sexo, tipo de VAA, duración de la enfermedad, tratamiento, órganos afectados, Birmingham Vasculitis Activity Score (BVAS), Five Factor Score (FFS), eritrosedimentación, proteína C reactiva, requerimiento de diálisis, asistencia respiratoria mecánica, mortalidad y causa de muerte. Se calculó el odds ratio (OR) y su intervalo de confianza del 95% (IC 95%) para determinar la fuerza de asociación entre las variables. Resultados: incluimos 32 pacientes en los que encontramos 31,2% de FR positivo. Comparados con aquellos con FR negativo, no hubo diferencias significativas con respecto a manifestaciones clínicas, ni actividad de la enfermedad medida por el BVAS y reactantes de fase aguda. La presencia de factor reumatoide se asoció a menor ingreso a diálisis (OR 0.14 IC 0,02-0,8; p=0,02) y mortalidad (p=0,04). No hubo diferencias significativas con respecto al FFS. Conclusiones: en nuestro estudio la presencia de FR en pacientes con vasculitis ANCA se asoció a menor requerimiento de diálisis y menor mortalidad. Estos hallazgos no se correlacionaron estadísticamente con el FFS. No se encontró asociación entre la presencia de FR y el compromiso de órgano, actividad de la enfermedad, ni requerimiento de asistencia respiratoria mecánica.

Introduction: rheumatoid factor tests are mainly used for the diagnosis of rheumatoid arthritis (RA); however, it may also be present in other diseases. Its clinical significance in the context of ANCA associated vasculitis (AAV) is still uncertain. The objective was to establish the clinical and prognostic significance of RF in patients with AAV. Materials and methods: a retrospective observational study was carried out. Patients with a diagnosis of AAV (GPA, MAP and AGEP) over 18 years of age with RF requested before the start of treatment were evaluated. The following data were collected: age, sex, type of AAV, disease duration, treatment, affected organs, Birmingham Vasculitis Activity Score (BVAS), Five Factor Score (FFS), erythrocyte sedimentation rate, C-reactive protein, dialysis requirement, attendance mechanical ventilation, mortality and cause of death. The odds ratio (OR) and its 95% confidence interval (95%CI) were calculated to determine the strength of association between variables. Results: we included 32 patients in whom we found 31.2% positive RF. Compared to those with negative RF, there were no significant differences with respect to clinical manifestations, nor disease activity measured by BVAS and acute phase reactants. The presence of the rheumatoid factor was associated with lower admission to dialysis (OR 0.14 CI 0.02-0.8 p=0.02) and mortality (p=0.04). There were no significant differences with respect to FFS. Conclusions: in our study, the presence of RF in patients with ANCA vasculitis was associated with a lower requirement for dialysis and lower mortality. These findings were not statistically correlated with the FFS. No association was found between the presence of RF and organ involvement, disease activity, or mechanical ventilation requirement.

Vacunación contra SARS-CoV-2 en pacientes con esclerosis sistémica en Argentina: preferencias, acceso y adherencia al plan de vacunación / Vaccination against SARS-CoV-2 in patients with systemic sclerosis in Argentina: preferences, access and adherence to the vaccination plan

Introducción: en pacientes con enfermedades reumatológicas autoinmunes se recomienda la aplicación sistemática y secuencial de una serie de vacunas para la prevención de enfermedades transmisibles. El objetivo de este estudio fue estimar la proporción de pacientes con esclerosis sistémica (ES) que recibieron vacunación contra el coronavirus (SARS-CoV-2). Materiales y métodos: se envió una encuesta anónima por correo electrónico o contacto por WhatsApp desde mayo a septiembre de 2021, con preguntas para evaluar la adherencia al esquema de vacunación recomendado en pacientes con enfermedades reumatológicas, así como temores, preferencias y adherencia al esquema de vacunación contra el SARS-CoV-2. Resultados: se incluyeron 295 pacientes con ES. El 68,81% estaba vacunado contra el SARS-CoV-2 con al menos una dosis, de los cuales el 48,7% tenía dos dosis. El 84,75% refirió conversar con su médico sobre su esquema de vacunación general. Solo el 5,4% tenía las cuatro vacunas. El 93,56% manifestó voluntad de vacunarse contra el SARS-CoV-2; el 56,27% prefirió la vacuna Sputnik V. El 7,46% manifestó su voluntad de no vacunarse. Los factores que influyeron en la adherencia a la vacunación, con mayor frecuencia, fueron el miedo a contraer la infección por SARS-CoV-2 (86,1%) y las reacciones adversas (23,05%). Conclusiones: destacamos el hecho de que solo 6 meses después de que se dispusiera la vacunación contra el SARS-CoV-2, la mitad de los pacientes con ES tenía el esquema recomendado completo.

Introduction: in patients with autoimmune rheumatic diseases, the systematic and sequential application of a series of vaccines is recommended for the prevention of communicable diseases. The objective was to estimate the proportion of patients with systemic sclerosis (SSc) who received vaccination against coronavirus (SARS-CoV-2). Materials and methods: since may to september 2021, an anonymous survey was sent by email or messaging app, containing questions to assess adherence to the recommended vaccination schedule in patients with rheumatic diseases, as well as fears, preferences and adherence to vaccination schedule against SARS-CoV-2. Results: 295 patients with SSc were included. 68.81% were vaccinated for SARS-CoV-2 with at least one dose, 48.7% of this group had two doses. 84.75% reported talking to their doctor about their general vaccination schedule. Only 5.4% had all four vaccines. 93.56% expressed willingness to be vaccinated against SARS-CoV-2, 56.27% preferred the Sputnik V vaccine. 7.46% expressed their willingness to not be vaccinated. The factors that most frequently influenced adherence to vaccination were fear of contracting SARS-CoV-2 infection (86.1%) and adverse reactions (23.05%). Conclusions: we highlight the fact that only 6 months after vaccination against SARS-CoV-2 became available, half of the patients with SSc had the full recommended schedule.

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.

Lupus eritematoso sistémico y parvovirus. ¿Relación causal?: Reporte de un caso / Systemic lupus erythematosus and parvovirus. Causal relationship?: Case report

El lupus eritematoso sistémico (LES) es una enfermedad autoinmune de etiología aún desconocida, afecta a individuos genéticamente predispuestos. Es desencadenado por factores hormonales, estrogénicos, ambientales y virales como hepatitis B/C, citomegalovirus (CMV), herpes o parvovirus B19 (PVB19). El PVB19 puede afectar diferentes órganos y presentar manifestaciones clínicas e inmunológicas, similares a las que se observan en pacientes con LES. Se han descripto alteraciones inmunológicas hasta en 79% de los pacientes con infección asociada por PVB19, principalmente positivización de anticuerpos antinucleares (ANA).

Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown etiology that affects genetically predisposed individuals. It is triggered by hormonal, estrogenic, environmental and viral factors such as hepatitis B/C, cytomegalovirus (CMV), herpes or parvovirus B19 (PVB19). PVB19 can affect different organs and present clinical and immunological diseases, as well as those observed in patients with SLE. Immunological alterations have been described in up to 79% of patients with infection associated with PVB19, mainly positivization of antinuclear antibodies (ANA).

Immune diffuse alveolar hemorrhage: Clinical presentation and outcome.

BACKGROUND: Diffuse alveolar haemorrhage (DAH) is a manifestation of several immune and nonimmune diseases. OBJECTIVE: The objective of this study was to characterize the clinical characteristics and short-term outcomes of patients with immune-mediated DAH requiring hospital admission. METHODS: A retrospective study from December 2010 to December 2015, was conducted by analyzing the clinical records of 39 patients with DAH with a proven immunological origin. The diagnosis of individual collagen vascular diseases (CVD) was made according to the criteria of the corresponding societies. RESULTS: Thirty-nine patients were included (median age 44.8 years, range 16-76). The main causes of DAH were ANCA-related vasculitis (74.3%) mainly granulomatosis with polyangiitis (n = 14) and microscopic polyangiitis (n = 13). Thirty patients (76.9%) had hemoptysis. An alveolar airspace filling pattern was found in most of patients (59%). All the patients had a drop in hemoglobin level that ranged from 1.0 to 3.0 g/dL. BAL fluid was macroscopically bloody in 43.6% of patients (n = 17) and showed siderophagic alveolitis on BAL cytology in 100%. All patients received high doses of corticosteroids. Other additional treatments were antibiotics (53,8%, n = 21), intravenous cyclophosphamide (87.2%, n = 34), plasma exchange (35.9%, n = 14); intravenous immunoglobulin (12.8%, n = 5) and rituximab in 5 patients (12.8%). Mortality rate was higher amongst patients who required dialysis (50.0 vs 15.4%, p = 0.045), with SaO2 <90% at admission (50.0 vs 5.3%, p = 0.003) or those who required mechanical ventilation (76.9 vs 6.8%, p = < 0.001). CONCLUSION: DAH may present without hemoptysis and requires an early bronchoscopy to confirm the diagnosis and exclude infection. Other characteristics could be included in the abstract which are relevant to the paper. (relation between mortality, dialysis, ventilation, etc.).