Description of a single centre cohort of patients with systemic sclerosis from the University Hospital of Buenos Aires and factors associated with lung function deterioration. A retrospective study.

INTRODUCTION: Given the paucity of data in Latin America and especially in Argentina regarding the epidemiology of SSc, the prevalence of ILD, its course, and particularly the response to treatment, our objective was to evaluate a cohort of SSc patients evaluated in a single University Hospital in Buenos Aires. PATIENTS/METHODS: We included 152 patients with SSc, followed from disease onset to last pulmonary function test and with at least two PFT and up to 30 months between each. RESULTS: Sixty-one percent had diffuse SSc (DSSc) and 32% limited SSc (LSSc). The only significant clinical differences between these groups were a higher initial mRodnan score and prevalence of ILD in the DSSc. These also had significantly more anti Scl-70 (Topoisomerase 1) antibodies compared to the LSSC group who had significantly more anti centromere antibodies. The DSSc group also had significantly more extensive damage on HRCT with no differences in terms of imaging patterns. Comparing patients with and without ILD by HRCT, those with ILD had significantly more extensive damage, significantly more anti Scl-70 antibodies, and significantly fewer anti centromere antibodies than those without ILD. Patients whose ILD progressed had a smoking history (OR 4.97) and prior immunosuppressive treatment (OR 15.6) (multivariate analysis). Overall disease duration was significantly shorter in those who progressed. CONCLUSIONS: Our SSc population had similar characteristics to those described elsewhere as well as prevalence of ILD and its progression. We found a shorter disease duration, smoking, and prior immunosuppressive treatment to be associated with ILD progression.

Description of a single centre cohort of patients with systemic sclerosis from the University Hospital of Buenos Aires and factors associated with lung function deterioration. A retrospective study / Descripción de una cohorte de pacientes con esclerosis sistémica del Hospital de la Universidad de Buenos Aires y factores asociados al deterioro de la función pulmonar. Un estudio retrospectivo

Introduction: Given the paucity of data in Latin America and especially in Argentina regarding the epidemiology of SSc, the prevalence of ILD, its course, and particularly the response to treatment, our objective was to evaluate a cohort of SSc patients evaluated in a single University Hospital in Buenos Aires. Patients/Methods: We included 152 patients with SSc, followed from disease onset to last pulmonary function test and with at least two PFT and up to 30 months between each. Results: Sixty-one percent had diffuse SSc (DSSc) and 32% limited SSc (LSSc). The only significant clinical differences between these groups were a higher initial mRodnan score and prevalence of ILD in the DSSc. These also had significantly more anti Scl-70 (Topoisomerase 1) antibodies compared to the LSSC group who had significantly more anti centromere antibodies. The DSSc group also had significantly more extensive damage on HRCT with no differences in terms of imaging patterns. Comparing patients with and without ILD by HRCT, those with ILD had significantly more extensive damage, significantly more anti Scl-70 antibodies, and significantly fewer anti centromere antibodies than those without ILD. Patients whose ILD progressed had a smoking history (OR 4.97) and prior immunosuppressive treatment (OR 15.6) (multivariate analysis). Overall disease duration was significantly shorter in those who progressed. Conclusions: Our SSc population had similar characteristics to those described elsewhere as well as prevalence of ILD and its progression. We found a shorter disease duration, smoking, and prior immunosuppressive treatment to be associated with ILD progression.(AU)

Introducción: La escasez de datos en Latinoamérica, y especialmente en Argentina, sobre la epidemiología de la esclerosis sistémica (SSc), la prevalencia de enfermedad pulmonar intersticial (EPID) y su progresión, llevó a evaluar una cohorte de pacientes con SSc atendidos en un hospital universitario de Buenos Aires, Argentina. Pacientes/Métodos: Incluimos 152 pacientes con SSc, seguidos desde el inicio de la enfermedad hasta el último examen funcional respiratorio (EFR) y con por lo menos dos EFR separados por un mínimo de 30 meses. Resultados: El 61% tenían enfermedad difusa (DSSc) y el 32%, limitada (LSSc). Aquellos con DSSc tuvieron significativamente un mayor índice modificado de Rodnan y prevalencia de EPID. Estos también tuvieron significativamente más anticuerpos anti-Scl-70 (topoisomerasa 1) comparados con LSSc, quienes tuvieron significativamente más anticuerpos anti-centrómero. Aquellos con DSSc mostraron significativamente más daño en la tomografía computada de alta resolución (TACAR), pero sin diferencias respecto a patrón de imágenes. Aquellos con EPID por TACAR tuvieron significativamente más daño, más anticuerpos anti Scl-70 y menos anticuerpos anti-centrómero que aquellos sin EPID. La progresión de EPID (análisis multivariado) se relacionó con consumo de tabaco (OR: 4,97) y uso previo de inmunosupresores (OR: 15,6). La duración de la enfermedad fue menor en los que progresaron. Conclusiones:Nuestra población de SSc tuvo características similares a lo descripto en el resto del mundo, así como la prevalencia y la progresión de EPID. Encontramos una menor duración de enfermedad, el consumo de tabaco y el uso previo de inmunosupresores asociados a la progresión de EPID.(AU)

[Factors associated with the development of interstitial lung disease in patients with systemic sclerosis]. / Factores asociados al desarrollo de enfermedad pulmonar intersticial en esclerosis sistémica.

INTRODUCTION: interstitial lung involvement occurs in 80% of chest CT scans of patients with systemic sclerosis (SS) and has a great impact on morbidity and mortality. The aim of the study was to describe factors associated with the development of interstitial lung disease (ILD) in patients diagnosed with SS in our division. METHODS: a retrospective case-control study of patients followed up between 2005-2021 who met the classification criteria for SS was performed. ILD was defined as the finding of interstitial manifestations on high-resolution chest tomography (HRCT): non-specific interstitial pneumonia pattern (NSIP) or usual interstitial pneumonia (UIP), and/or findings on pulmonary function tests (FVC less than 80% and DLCO less than 80%). Patients with ILD (cases) and without it (controls) were identified. Demographic, clinical and serological variables were analyzed. Percentage, mean (SD) and median (IQR) measurements were calculated for each variable. A univariate and multivariate analysis was performed using logistic regression to establish its association with ILD. RESULTS: Seventy nine patients with SS were included, 31 with ILD. Univariate analysis showed that sclerosis subtype (according to Le Roy classification), lung function measures, and anticentromere antibody positivity were factors associated with ILD in a statistically significant way. In the multivariate analysis, only the presence of anti-centromere antibodies was statistically significant. DISCUSSION: the analysis of risk factors to determine the development and progression of lung damage is of vital importance for an early implementation of treatment, which would impact the mortality rate of patients with SS.

Introducción: el compromiso pulmonar intersticial se presenta en 80% de las tomografías de tórax de pacientes con esclerosis sistémica (ES) y tiene gran impacto en la morbimortalidad. El objetivo de este trabajo fue describir factores asociados al desarrollo de enfermedad pulmonar intersticial (EPI) en pacientes con diagnóstico de ES de nuestra división. Métodos: Se realizó un estudio retrospectivo, casos y controles, de pacientes seguidos entre 2005-2021 que cumplían criterios de ES. Se definió EPI al hallazgo de manifestaciones intersticiales en tomografía de tórax con cortes de alta resolución (TACAR): patrón neumonía intersticial no específica (NINE) o neumonía intersticial usual (NIU), y/o hallazgos en pruebas de función pulmonar (CVF menor al 80% y DLCO menor al 80%). Se identificaron pacientes con EPI (casos) y sin ella (controles). Se analizaron variables demográficas, clínicas y serológicas. Se calcularon medidas de porcentaje, media (DS) y mediana (RIQ) en cada variable. Se efectuó análisis univariado y multivariado, mediante regresión logística para establecer su asociación con EPI. Resultados: Se incluyeron 79 pacientes con ES, 31 con EPI. El análisis univariado demostró que el subtipo de esclerosis (según clasific ación Le Roy), las medidas de función pulmonar y positividad del anticuerpo anticentrómero fueron factores asociados en forma estadísticamente significativa con EPI. En el análisis multivariado solo la presencia de anticuerpos anti-centrómero fue estadísticamente significativa. Discusión: el análisis de los factores de riesgo para determinar desarrollo y progresión de daño pulmonar tiene vital importancia para una implementación temprana del tratamiento, lo que impactaría en la tasa de mortalidad de los pacientes con ES.

Factores asociados al desarrollo de enfermedad pulmonar intersticial en esclerosis sistémica / Factors associated with the development of interstitial lung disease in patients with systemic sclerosis

Resumen Introducción: el compromiso pulmonar intersticial se presenta en 80% de las tomografías de tórax de pacientes con esclerosis sistémica (ES) y tiene gran impacto en la morbimortalidad. El objeti vo de este trabajo fue describir factores asociados al desarrollo de enfermedad pulmonar intersticial (EPI) en pacientes con diagnóstico de ES de nuestra división. Métodos: Se realizó un estudio retrospectivo, casos y controles, de pacientes seguidos entre 2005-2021 que cumplían criterios de ES. Se definió EPI al hallazgo de manifestaciones intersticiales en tomografía de tórax con cortes de alta resolución (TACAR): patrón neumonía intersticial no específica (NINE) o neumonía intersticial usual (NIU), y/o hallazgos en pruebas de función pulmonar (CVF menor al 80% y DLCO menor al 80%). Se identificaron pacientes con EPI (casos) y sin ella (controles). Se analizaron variables demográficas, clínicas y serológicas. Se calcularon medidas de porcentaje, media (DS) y mediana (RIQ) en cada variable. Se efectuó análisis univariado y multivariado, mediante regresión logística para establecer su asociación con EPI. Resultados: Se incluyeron 79 pacientes con ES, 31 con EPI. El análisis univariado demostró que el subtipo de esclerosis (según clasific ación Le Roy), las medidas de función pulmonar y positividad del anticuerpo anticentrómero fueron factores asociados en forma estadísticamente significativa con EPI. En el análisis multivariado solo la presencia de anticuerpos anti-centrómero fue estadísticamente significativa. Discusión: el análisis de los factores de riesgo para determinar desarrollo y progresión de daño pulmonar tiene vital importancia para una implementación temprana del tratamiento, lo que impactaría en la tasa de mortalidad de los pacientes con ES.

Abstract Introduction: interstitial lung involvement occurs in 80% of chest CT scans of patients with systemic sclerosis (SS) and has a great impact on morbidity and mortality. The aim of the study was to describe factors associated with the development of interstitial lung disease (ILD) in patients diagnosed with SS in our division. Methods: a retrospective case-control study of patients followed up between 2005-2021 who met the classification criteria for SS was performed. ILD was defined as the finding of interstitial manifestations on high-resolution chest tomography (HRCT): non-specific interstitial pneumonia pattern (NSIP) or usual interstitial pneumonia (UIP), and/or findings on pulmonary function tests (FVC less than 80% and DLCO less than 80%). Patients with ILD (cases) and without it (controls) were identified. Demographic, clinical and serological variables were analyzed. Percentage, mean (SD) and median (IQR) measurements were calculated for each variable. A univariate and multivariate analysis was performed using logistic regression to establish its association with ILD. Results: Seventy nine patients with SS were included, 31 with ILD. Univariate analysis showed that sclerosis subtype (according to Le Roy classification), lung function measures, and anticentromere antibody positivity were factors associated with ILD in a statistically significant way. In the multivariate analysis, only the presence of anti-centromere antibodies was statistically significant. Discussion: the analysis of risk factors to determine the development and progression of lung damage is of vital importance for an early implementation of treatment, which would impact the mortality rate of patients with SS.

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.

Vasculitis reumatoidea como manifestación extraarticular en dos casos / Rheumatoid vasculitis as an extra articular manifestation in two cases

La vasculitis reumatoidea es un proceso inflamatorio poco frecuente que confiere una morbilidad y mortalidad significativa en pacientes con artritis reumatoidea (AR). Presenta una incidencia de 0,7 a 5,4% de los casos de AR y hasta un 40% de mortalidad a 5 años, siendo la manifestación extraarticular de mayor gravedad1,2. Se caracteriza por el desarrollo de vasculitis necrotizante; ocurre típicamente en pacientes masculinos con AR seropositiva de larga data, nodular y erosiva, tabaquistas y, en general, tiene mal pronóstico. Se presenta una serie de casos, paciente masculino y femenino con AR y manifestaciones extraarticulares, necrosis digital y compromiso multiorgánico, interpretados como cuadros de vasculitis reumatoidea, realizando tratamiento de referencia con inmunosupresores.

Rheumatoid vasculitis is a rare inflammatory process that confers significant morbidity and mortality in patients with rheumatoid arthritis (RA); it has an incidence of 0.7 to 5.4% of RA cases and up to 40% mortality at five years, making it the most serious of all the extra-articular manifestations of RA1,2. It is characterized by the development of necrotizing vasculitis, it typically occurs in male patients with long-standing seropositive RA, erosive nodular, smokers and generally has a poor prognosis. A series of cases is presented, male and female with RA and extra-articular manifestations, digital necrosis and multiorgan system involvement, interpreted as rheumatoid vasculitis, undergoing treatment with immunosuppressants.

Reasons for hospitalisation in patients with systemic lupus erythematosus in a university hospital in Buenos Aires.

OBJECTIVE: To determine the reasons for hospitalisation in patients with systemic lupus erythematosus (SLE) admitted to the Hospital de Clínicas "José de San Martín" Buenos Aires, Argentina. METHODS: We retrospectively analysed the clinical histories of SLE patients (SLICC 2012) during or prior to hospitalisation, from 1-2014 to 12-2017. Demographic data, reasons for hospitalisation, treatments, SLEDAI-2 K and comorbid conditions. RESULTS: 121 hospitalisations corresponding to 72 patients, 25 patients (34.7%) were hospitalised more than once. Females (83.3%), the median length of disease until admission was 5 years. There was more than one reason for hospitalisation in 32, a total of 164 reasons. The main reason was relapse of disease (52.4%). The most frequent manifestations were renal involvement, serositis and pulmonary involvement. Infections were the second reason for hospitalisation (26.8%); urinary tract, pneumonia and soft tissue. Cardiovascular involvement 4.9%. Hospitalisation in a closed unit 14.05%, mortality 2.48%. CONCLUSION: Relapse of disease and infections were the main reasons for hospitalisation; admission secondary to infection was higher than that reported in other series.

Causas de internación en pacientes con lupus eritematoso sistémico en un hospital universitario de Buenos Aires / Reasons for Hospitalisation in Patients with Systemic Lupus Erythematosus in a University Hospital in Buenos Aires

Objetivo: Determinar causas de hospitalización en pacientes con lupus eritematoso sistémico (LES) internados en Hospital de Clínicas «José de San Martín», Buenos Aires, Argentina. Métodos: Se analizaron retrospectivamente historias clínicas de pacientes LES (SLICC 2012) durante o previo a la internación, desde 1-2014 hasta 12-2017. Datos demográficos, motivos de hospitalización, tratamientos, SLEDAI 2 K y condiciones comórbidas. Resultados: 121 hospitalizaciones correspondientes a 72 pacientes, 25 pacientes (34,7%) tuvieron más de una hospitalización. Mujeres (83,3%), mediana de duración de la enfermedad hasta la admisión 5 años. Más de una causa de internación en 32, total de 164 motivos. La principal causa fue recaída de la enfermedad (52,4%). La manifestación más frecuente fue compromiso renal, serositis y compromiso pulmonar. Las infecciones fueron la segunda causa de hospitalización (26,8%); tracto urinario, neumonía y partes blandas. Compromiso cardiovascular 4,9%. Hospitalización en unidad cerrada 14,05%, mortalidad 2,48%. Conclusión: Recaída de la enfermedad e infecciones, fueron las principales causas de hospitalización; la admisión secundaria a infección fue superior a la reportada en otras series.(AU)

Objective: To determine the reasons for hospitalisation in patients with systemic lupus erythematosus (SLE) admitted to the Hospital de Clínicas «José de San Martín» Buenos Aires, Argentina. Methods: We retrospectively analysed the clinical histories of SLE patients (SLICC 2012) during or prior to hospitalisation, from 1-2014 to 12-2017. Demographic data, reasons for hospitalisation, treatments, SLEDAI-2K and comorbid conditions. Results: 121 hospitalisations corresponding to 72 patients, 25 patients (34.7%) were hospitalised more than once. Females (83.3%), the median length of disease until admission was 5 years. There was more than one reason for hospitalisation in 32, a total of 164 reasons. The main reason was relapse of disease (52.4%). The most frequent manifestations were renal involvement, serositis and pulmonary involvement. Infections were the second reason for hospitalisation (26.8%); urinary tract, pneumonia and soft tissue. Cardiovascular involvement 4.9%. Hospitalisation in a closed unit 14.05%, mortality 2.48%. Conclusion: Relapse of disease and infections were the main reasons for hospitalisation; admission secondary to infection was higher than that reported in other series.(AU)

Reasons for Hospitalisation in Patients with Systemic Lupus Erythematosus in a University Hospital in Buenos Aires. / Causas de internación en pacientes con lupus eritematoso sistémico en un hospital universitario de Buenos Aires.

OBJECTIVE: To determine the reasons for hospitalisation in patients with systemic lupus erythematosus (SLE) admitted to the Hospital de Clínicas «José de San Martín¼ Buenos Aires, Argentina. METHODS: We retrospectively analysed the clinical histories of SLE patients (SLICC 2012) during or prior to hospitalisation, from 1-2014 to 12-2017. Demographic data, reasons for hospitalisation, treatments, SLEDAI-2K and comorbid conditions. RESULTS: 121 hospitalisations corresponding to 72 patients, 25 patients (34.7%) were hospitalised more than once. Females (83.3%), the median length of disease until admission was 5 years. There was more than one reason for hospitalisation in 32, a total of 164 reasons. The main reason was relapse of disease (52.4%). The most frequent manifestations were renal involvement, serositis and pulmonary involvement. Infections were the second reason for hospitalisation (26.8%); urinary tract, pneumonia and soft tissue. Cardiovascular involvement 4.9%. Hospitalisation in a closed unit 14.05%, mortality 2.48%. CONCLUSION: Relapse of disease and infections were the main reasons for hospitalisation; admission secondary to infection was higher than that reported in other series.

[Hematopoietic stem cell transplantation in autoimmune diseases]. / Trasplante de células progenitoras hematopoyéticas en enfermedades autoinmunes.

Transplantation of hematopoietic stem cells, which are capable of self renewal and reconstitution of all types of blood cells, can be a treatment for numerous potential lethal diseases, including leukemias and lymphomas. It may now be applicable for the treatment of severe autoimmune diseases, such as therapy-resistant multiple sclerosis, lupus and systemic sclerosis. Studies in animal models show that the transfer of hematopoietic stem cells can reverse autoimmunity. The outcome of ongoing clinical trials, as well as of studies in patients and animal models, will help to determine the role that stem-cell transplantation can play in the treatment of autoimmune diseases.