Impact of mediterranean diet promotion on environmental sustainability: a longitudinal analysis

[EN]This article aims to estimate the differences in environmental impact (greenhouse gas [GHG] emissions, land use, energy used, acidification and potential eutrophication) after one year of promoting a Mediterranean diet (MD). Methods Baseline and 1-year follow-up data from 5800 participants in the PREDIMED-Plus study were used. Each participant's food intake was estimated using validated semi-quantitative food frequency questionnaires, and the adherence to MD using the Dietary Score. The influence of diet on environmental impact was assessed through the EAT-Lancet Commission tables. The influence of diet on environmental impact was assessed through the EAT-Lancet Commission tables. The association between MD adherence and its environmental impact was calculated using adjusted multivariate linear regression models.SIPublicación en abierto financiada por el Consorcio de Bibliotecas Universitarias de Castilla y León (BUCLE), con cargo al Programa Operativo 2014ES16RFOP009 FEDER 2014-2020 DE CASTILLA Y LEÓN, Actuación:20007-CL - Apoyo Consorcio BUCL

Standardized incidence ratios and risk factors for cancer in patients with systemic sclerosis: Data from the Spanish Scleroderma Registry (RESCLE)

Aim: Patients with systemic sclerosis (SSc) are at increased risk of cancer, a growing cause of non-SSc-related death among these patients. We analyzed the increased cancer risk among Spanish patients with SSc using standardized incidence ratios (SIRs) and identified independent cancer risk factors in this population. Material and methods: Spanish Scleroderma Registry data were analyzed to determine the demographic characteristics of patients with SSc, and logistic regression was used to identify cancer risk factors. SIRs with 95% confidence intervals (CIs) relative to the general Spanish population were calculated. Results: Of 1930 patients with SSc, 206 had cancer, most commonly breast, lung, hematological, and colorectal cancers. Patients with SSc had increased risks of overall cancer (SIR 1.48, 95% CI 1.36-1.60; P < 0.001), and of lung (SIR 2.22, 95% CI 1.77-2.73; P < 0.001), breast (SIR 1.31, 95% CI 1.10-1.54; P = 0.003), and hematological (SIR 2.03, 95% CI 1.52-2.62; P < 0.001) cancers. Cancer was associated with older age at SSc onset (odds ratio [OR] 1.22, 95% CI 1.01-1.03; P < 0.001), the presence of primary biliary cholangitis (OR 2.35, 95% CI 1.18-4.68; P = 0.015) and forced vital capacity <70% (OR 1.8, 95% CI 1.24-2.70; P = 0.002). The presence of anticentromere antibodies lowered the risk of cancer (OR 0.66, 95% CI 0.45-0.97; P = 0.036). Conclusions: Spanish patients with SSc had an increased cancer risk compared with the general population. Some characteristics, including specific autoantibodies, may be related to this increased risk

Molecular Characterization of Monocyte Subsets Reveals Specific and Distinctive Molecular Signatures Associated With Cardiovascular Disease in Rheumatoid Arthritis

Objectives: This study, developed within the Innovative Medicines Initiative Joint Undertaking project PRECISESADS framework, aimed at functionally characterize the monocyte subsets in RA patients, and analyze their involvement in the increased CV risk associated with RA.Methods: The frequencies of monocyte subpopulations in the peripheral blood of 140 RA patients and 145 healthy donors (HDs) included in the PRECISESADS study were determined by flow cytometry. A second cohort of 50 RA patients and 30 HDs was included, of which CD14+ and CD16+ monocyte subpopulations were isolated using immuno-magnetic selection. Their transcriptomic profiles (mRNA and microRNA), proinflammatory patterns and activated pathways were evaluated and related to clinical features and CV risk. Mechanistic in vitro analyses were further performed.Results: CD14++CD16+ intermediate monocytes were extended in both cohorts of RA patients. Their increased frequency was associated with the positivity for autoantibodies, disease duration, inflammation, endothelial dysfunction and the presence of atheroma plaques, as well as with the CV risk score. CD14+ and CD16+ monocyte subsets showed distinctive and specific mRNA and microRNA profiles, along with specific intracellular signaling activation, indicating different functionalities. Moreover, that specific molecular profiles were interrelated and associated to atherosclerosis development and increased CV risk in RA patients. In vitro, RA serum promoted differentiation of CD14+CD16− to CD14++CD16+ monocytes. Co-culture with RA-isolated monocyte subsets induced differential activation of endothelial cells.Conclusions: Our overall data suggest that the generation of inflammatory monocytes is associated to the autoimmune/inflammatory response that mediates RA. These monocyte subsets, -which display specific and distinctive molecular signatures- might promote endothelial dysfunction and in turn, the progression of atherosclerosis through a finely regulated process driving CVD development in RA

VIII Encuentro de Docentes e Investigadores en Historia del Diseño, la Arquitectura y la Ciudad

Acta de congresoLa conmemoración de los cien años de la Reforma Universitaria de 1918 se presentó como una ocasión propicia para debatir el rol de la historia, la teoría y la crítica en la formación y en la práctica profesional de diseñadores, arquitectos y urbanistas. En ese marco el VIII Encuentro de Docentes e Investigadores en Historia del Diseño, la Arquitectura y la Ciudad constituyó un espacio de intercambio y reflexión cuya realización ha sido posible gracias a la colaboración entre Facultades de Arquitectura, Urbanismo y Diseño de la Universidad Nacional y la Facultad de Arquitectura de la Universidad Católica de Córdoba, contando además con la activa participación de mayoría de las Facultades, Centros e Institutos de Historia de la Arquitectura del país y la región. Orientado en su convocatoria tanto a docentes como a estudiantes de Arquitectura y Diseño Industrial de todos los niveles de la FAUD-UNC promovió el debate de ideas a partir de experiencias concretas en instancias tales como mesas temáticas de carácter interdisciplinario, que adoptaron la modalidad de presentación de ponencias, entre otras actividades. En el ámbito de VIII Encuentro, desarrollado en la sede Ciudad Universitaria de Córdoba, se desplegaron numerosas posiciones sobre la enseñanza, la investigación y la formación en historia, teoría y crítica del diseño, la arquitectura y la ciudad; sumándose el aporte realizado a través de sus respectivas conferencias de Ana Clarisa Agüero, Bibiana Cicutti, Fernando Aliata y Alberto Petrina. El conjunto de ponencias que se publican en este Repositorio de la UNC son el resultado de dos intensas jornadas de exposiciones, cuyos contenidos han posibilitado actualizar viejos dilemas y promover nuevos debates. El evento recibió el apoyo de las autoridades de la FAUD-UNC, en especial de la Secretaría de Investigación y de la Biblioteca de nuestra casa, como así también de la Facultad de Arquitectura de la UCC; va para todos ellos un especial agradecimiento

Prevalence, associated factors and outcomes of pressure injuries in adult intensive care unit patients: the DecubICUs study

Funder: European Society of Intensive Care Medicine; doi: http://dx.doi.org/10.13039/501100013347Funder: Flemish Society for Critical Care NursesAbstract: Purpose: Intensive care unit (ICU) patients are particularly susceptible to developing pressure injuries. Epidemiologic data is however unavailable. We aimed to provide an international picture of the extent of pressure injuries and factors associated with ICU-acquired pressure injuries in adult ICU patients. Methods: International 1-day point-prevalence study; follow-up for outcome assessment until hospital discharge (maximum 12 weeks). Factors associated with ICU-acquired pressure injury and hospital mortality were assessed by generalised linear mixed-effects regression analysis. Results: Data from 13,254 patients in 1117 ICUs (90 countries) revealed 6747 pressure injuries; 3997 (59.2%) were ICU-acquired. Overall prevalence was 26.6% (95% confidence interval [CI] 25.9–27.3). ICU-acquired prevalence was 16.2% (95% CI 15.6–16.8). Sacrum (37%) and heels (19.5%) were most affected. Factors independently associated with ICU-acquired pressure injuries were older age, male sex, being underweight, emergency surgery, higher Simplified Acute Physiology Score II, Braden score 3 days, comorbidities (chronic obstructive pulmonary disease, immunodeficiency), organ support (renal replacement, mechanical ventilation on ICU admission), and being in a low or lower-middle income-economy. Gradually increasing associations with mortality were identified for increasing severity of pressure injury: stage I (odds ratio [OR] 1.5; 95% CI 1.2–1.8), stage II (OR 1.6; 95% CI 1.4–1.9), and stage III or worse (OR 2.8; 95% CI 2.3–3.3). Conclusion: Pressure injuries are common in adult ICU patients. ICU-acquired pressure injuries are associated with mainly intrinsic factors and mortality. Optimal care standards, increased awareness, appropriate resource allocation, and further research into optimal prevention are pivotal to tackle this important patient safety threat

Prevalence, associated factors and outcomes of pressure injuries in adult intensive care unit patients: the DecubICUs study

Funder: European Society of Intensive Care Medicine; doi: http://dx.doi.org/10.13039/501100013347Funder: Flemish Society for Critical Care NursesAbstract: Purpose: Intensive care unit (ICU) patients are particularly susceptible to developing pressure injuries. Epidemiologic data is however unavailable. We aimed to provide an international picture of the extent of pressure injuries and factors associated with ICU-acquired pressure injuries in adult ICU patients. Methods: International 1-day point-prevalence study; follow-up for outcome assessment until hospital discharge (maximum 12 weeks). Factors associated with ICU-acquired pressure injury and hospital mortality were assessed by generalised linear mixed-effects regression analysis. Results: Data from 13,254 patients in 1117 ICUs (90 countries) revealed 6747 pressure injuries; 3997 (59.2%) were ICU-acquired. Overall prevalence was 26.6% (95% confidence interval [CI] 25.9–27.3). ICU-acquired prevalence was 16.2% (95% CI 15.6–16.8). Sacrum (37%) and heels (19.5%) were most affected. Factors independently associated with ICU-acquired pressure injuries were older age, male sex, being underweight, emergency surgery, higher Simplified Acute Physiology Score II, Braden score 3 days, comorbidities (chronic obstructive pulmonary disease, immunodeficiency), organ support (renal replacement, mechanical ventilation on ICU admission), and being in a low or lower-middle income-economy. Gradually increasing associations with mortality were identified for increasing severity of pressure injury: stage I (odds ratio [OR] 1.5; 95% CI 1.2–1.8), stage II (OR 1.6; 95% CI 1.4–1.9), and stage III or worse (OR 2.8; 95% CI 2.3–3.3). Conclusion: Pressure injuries are common in adult ICU patients. ICU-acquired pressure injuries are associated with mainly intrinsic factors and mortality. Optimal care standards, increased awareness, appropriate resource allocation, and further research into optimal prevention are pivotal to tackle this important patient safety threat

Correction to: Prevalence, associated factors and outcomes of pressure injuries in adult intensive care unit patients: the DecubICUs study

The original version of this article unfortunately contained a mistake

Anticuerpos antinucleares específicos de la esclerodermia como determinantes del pronóstico y de los diferentes patrones clínicos de la enfermedad

La esclerodermia (ES) es una enfermedad autoinmune con expresividad clínica y pronóstico muy variables. El objetivo de este estudio es establecer la asociación de algunos anticuerpos específicos y asociados a la ES con perfiles clínico-epidemiológicos determinados, y demostrar la importancia del patrón inmunológico para predecir el curso y pronóstico de la enfermedad. MÉTODOS: Se estudió una amplia cohorte de pacientes con ES visitados en la unidad de Enfermedades autoinmunes del Hospital Vall d’Hebron (Barcelona). Todos cumplían los criterios de clasificación propuestos por Leroy y Medsger. Se recogieron datos epidemiológicos, clínicos e inmunológicos de acuerdo con un protocolo estándar. Se realizaron 3 estudios independientes centrados en los tres anticuerpos más específicos de la enfermedad (anticentrómero (ACA), anti-Topoisomerasa (ATA-I) y anti-RNA polimerasa (RNAp)), y en el anticuerpo antiSSa-Ro52. En el primero se incluyeron 319 pacientes, se compararon los grupos de pacientes con ACA y ATA-I con los grupos con ES limitada y difusa; en el segundo se seleccionaron 175 pacientes con determinación para RNAp, y en el tercero 132 pacientes con determinación para antiSSa/Ro52. RESULTADOS: Primer estudio, de los 319 pacientes, 288 (90.3%) eran mujeres. La edad media al diagnóstico fue de 51.5±15.1 años y al inicio de la enfermedad 43±15.9 años. ACA fue el anticuerpo más frecuente (39.5%), seguido por ATA-I (16.5%). La afección esofágica, enfermedad pulmonar intersticial (EPI), EPI grave, crisis renal esclerodérmica (CRE), úlceras digitales (UD), clínica osteoarticular y el patrón activo en la capilaroscopia, fueron más frecuentes en el grupo ATA-I positivo. El fenómeno de Raynaud (FR) en general y como primera manifestación, y el patrón lento capilaroscópico fueron más prevalentes en el grupo ACA positivo. La distribución por subtipos cutáneos fue: ES limitada (58.3%), ES difusa (20%), ES sine ES (14.7%) y pre-ES (6.9%). La frecuencia de manifestaciones clínicas en los pacientes con ES limitada y difusa fueron muy similares a las identificadas en los pacientes clasificados por anticuerpo, ACA y ATA-I, respectivamente. En el análisis univariable se encontraron las siguientes diferencias entre ambas categorizaciones: ATA-I presentó más afección esofágica que la ES difusa; la ES limitada más hipertensión arterial pulmonar (HAP) aislada que el anticuerpo ACA; y la ES difusa más muertes por EPI que ATA-I. Hubo una mayor tendencia a presentar UD en el grupo ES difusa que en el ATA-I positivo. El análisis multivariable mostró que la contribución del perfil inmunológico sobre las principales complicaciones orgánicas es similar a la del perfil cutáneo. La ES difusa tiene más peso sobre la EPI y CRE, y el ACA es un factor protector independiente para la afección esofágica y EPI. En el segundo estudio, de los 175 pacientes seleccionados, 24 presentaban RNAp. En el grupo RNAp positivo fue más prevalente la ES difusa, los pacientes cumplieron más frecuentemente los criterios diagnósticos ACR/EULAR 2013, presentaron con menor frecuencia FR como primera manifestación de la enfermedad y desarrollaron más CRE. Hubo mayor tendencia a presentar UD y cáncer en el grupo con RNAp, sin llegar a la significación estadística. En el tercer estudio, de los 132 pacientes, 87.1% eran mujeres; 51.5% presentaban ES limitada. La prevalencia del antiSSa/Ro52 fue 35.6%. No se encontró ninguna asociación entre antiSSa/Ro52 y manifestaciones clínicas. Un alto porcentaje de pacientes presentó ACA concomitante con antiSSa/Ro52. CONCLUSIONES: -El perfil inmunológico (ATA-I y ACA) tiene un poder similar al subtipo cutáneo para predecir el curso de la enfermedad en ES. -Los anticuerpos RNAp se asocian con el subtipo cutáneo difusa, CRE y formas de inicio de la enfermedad diferentes al FR. -Los anticuerpos antiSSa/Ro52 son frecuentes en pacientes con ES y no se asociaron con manifestaciones clínicas determinadas.Systemic Sclerosis (SSc) is an autoimmune disease with a high clinical and prognostic variability. The aims of these studies are to identify the associations between specific SSc-related antibodies and specific clinical and epidemiological profiles, and to prove the importance of the immunological pattern to predict the course and prognosis of the disease. METHODS A large cohort of SSc patients diagnosed in the Systemic Diseases Unit of Vall d'Hebron Hospital (Barcelone) was studied, all of whom fulfilled the classification criteria proposed by LeRoy and Medsger. Epidemiological, clinical and immunological data were collected according to a standard protocol. Three independent studies were performed focusing on the 3 most specific SSc-related antibodies (anticentromere [ACA], antitopoisomerase I [ATA-I] and anti-RNA polymerase [RNAp]), and on the antiSSa/Ro52 antibodies. In the first study 319 patients were recruited; both autoantibody subsets, ACA and ATA-I positive, were compared with SSc subsets (lcSSc and dcSSc). The second study comprised 175 patients with analysis of RNAp and the third study, 132 patients with analysis of antiSSa/Ro52. RESULTS: First study: Of 319 patients, 288 (90.3%) were women. Mean age at diagnosis was 51.5±15.1 years and mean age at disease onset was 43±15.9 years. ACA was the most frequent autoantibody (39.5%) followed by ATA-I (16.5%). Patients with ACA had a higher prevalence of esophageal involvement, interstitial lung disease (ILD), severe ILD, scleroderma renal crisis (SRC), digital ulcers (DU), joint involvement and of an active pattern in nailfold capillaroscopy. The incidence of Raynaud phenomenon (RP), RP as the first disease symptom, and a slow pattern in nailfold capillaroscopy were higher in patients with ACA. Cutaneous subsets distribution was as follows: limited cutaneous Ssc (lcSSc) 58.3%, diffuse cutaneous SSc (dSSc) 20%, SSc sine scleroderma (ssSSc) 14.7% and "prescleroderma" (pre-SSc) 6.9%. The frequency of the clinical manifestations according to the cutaneous subsets, lcSSc and dcSSc, was similar to the frequency according to the particular autoantibody status (ACA or ATA-I). Significant differences in disease presentation found on univariate analysis were as follows: ATA-I positivity, rather than dcSSc, was associated with higher frequency of esophageal involvement; instead, patients with lcSSc had a higher prevalence of isolated pulmonary arterial hypertension (PAH) compared with those with ACA; and dcSSc, rather than ATA-I positivity had more scleroderma-related deaths due to ILD. Patients with dcSSc had a trend to develop DU in comparison with patients with ATA-I. The multivariate studies showed that the contributory effect of the antibody status for the disease manifestations was very similar to that of the clinical categorization into lcSSc and dcSSc. dcSSc was an independent predictor factor for ILD and SRC, and ACA antibodies were an independent protector factor for esophageal involvement and ILD. Second study: Among the 175 selected patients 24 had RNAp. RNAp patients presented with higher incidence of dcSSc and met the ACR / EULAR 2013 diagnostic criteria more frequently than negative RNAp patients; they also had a lower incidence of RP as the first manifestation of the disease and a higher incidence of SCR. RNAp patients tended to develop DU and cancer compared with negative RNAp patients, without reaching statistical significance. Third study: of 132 patients, 87.1% were women; 51.5% had lcSSc. Prevalence of antiSSa/Ro52 was 35.6%. No association between antiSSa/Ro52 and clinical manifestations was found. A high percentage of patients presented ACA concomitantly with antiSSa/Ro52. CONCLUSIONS: -The classification according to immunological pattern (ATA-I and ACA) was as strongly associated with the course of SE as the categorization into cutaneous subtypes. -RNAp antibodies are associated with dcSSc, SRC and other symptoms different from RP at the onset of the disease. -AntiSSa/Ro52 antibodies are often found in SSc patients. No clinical manifestations, including inflammatory myopathy, were related with antiSSa/Ro52 antibodies

Anticuerpos antinucleares específicos de la esclerodermia como determinantes del pronóstico y de los diferentes patrones clínicos de la enfermedad

La esclerodermia (ES) es una enfermedad autoinmune con expresividad clínica y pronóstico muy variables. El objetivo de este estudio es establecer la asociación de algunos anticuerpos específicos y asociados a la ES con perfiles clínico-epidemiológicos determinados, y demostrar la importancia del patrón inmunológico para predecir el curso y pronóstico de la enfermedad. MÉTODOS: Se estudió una amplia cohorte de pacientes con ES visitados en la unidad de Enfermedades autoinmunes del Hospital Vall d’Hebron (Barcelona). Todos cumplían los criterios de clasificación propuestos por Leroy y Medsger. Se recogieron datos epidemiológicos, clínicos e inmunológicos de acuerdo con un protocolo estándar. Se realizaron 3 estudios independientes centrados en los tres anticuerpos más específicos de la enfermedad (anticentrómero (ACA), anti-Topoisomerasa (ATA-I) y anti-RNA polimerasa (RNAp)), y en el anticuerpo antiSSa-Ro52. En el primero se incluyeron 319 pacientes, se compararon los grupos de pacientes con ACA y ATA-I con los grupos con ES limitada y difusa; en el segundo se seleccionaron 175 pacientes con determinación para RNAp, y en el tercero 132 pacientes con determinación para antiSSa/Ro52. RESULTADOS: Primer estudio, de los 319 pacientes, 288 (90.3%) eran mujeres. La edad media al diagnóstico fue de 51.5±15.1 años y al inicio de la enfermedad 43±15.9 años. ACA fue el anticuerpo más frecuente (39.5%), seguido por ATA-I (16.5%). La afección esofágica, enfermedad pulmonar intersticial (EPI), EPI grave, crisis renal esclerodérmica (CRE), úlceras digitales (UD), clínica osteoarticular y el patrón activo en la capilaroscopia, fueron más frecuentes en el grupo ATA-I positivo. El fenómeno de Raynaud (FR) en general y como primera manifestación, y el patrón lento capilaroscópico fueron más prevalentes en el grupo ACA positivo. La distribución por subtipos cutáneos fue: ES limitada (58.3%), ES difusa (20%), ES sine ES (14.7%) y pre-ES (6.9%). La frecuencia de manifestaciones clínicas en los pacientes con ES limitada y difusa fueron muy similares a las identificadas en los pacientes clasificados por anticuerpo, ACA y ATA-I, respectivamente. En el análisis univariable se encontraron las siguientes diferencias entre ambas categorizaciones: ATA-I presentó más afección esofágica que la ES difusa; la ES limitada más hipertensión arterial pulmonar (HAP) aislada que el anticuerpo ACA; y la ES difusa más muertes por EPI que ATA-I. Hubo una mayor tendencia a presentar UD en el grupo ES difusa que en el ATA-I positivo. El análisis multivariable mostró que la contribución del perfil inmunológico sobre las principales complicaciones orgánicas es similar a la del perfil cutáneo. La ES difusa tiene más peso sobre la EPI y CRE, y el ACA es un factor protector independiente para la afección esofágica y EPI. En el segundo estudio, de los 175 pacientes seleccionados, 24 presentaban RNAp. En el grupo RNAp positivo fue más prevalente la ES difusa, los pacientes cumplieron más frecuentemente los criterios diagnósticos ACR/EULAR 2013, presentaron con menor frecuencia FR como primera manifestación de la enfermedad y desarrollaron más CRE. Hubo mayor tendencia a presentar UD y cáncer en el grupo con RNAp, sin llegar a la significación estadística. En el tercer estudio, de los 132 pacientes, 87.1% eran mujeres; 51.5% presentaban ES limitada. La prevalencia del antiSSa/Ro52 fue 35.6%. No se encontró ninguna asociación entre antiSSa/Ro52 y manifestaciones clínicas. Un alto porcentaje de pacientes presentó ACA concomitante con antiSSa/Ro52. CONCLUSIONES: -El perfil inmunológico (ATA-I y ACA) tiene un poder similar al subtipo cutáneo para predecir el curso de la enfermedad en ES. -Los anticuerpos RNAp se asocian con el subtipo cutáneo difusa, CRE y formas de inicio de la enfermedad diferentes al FR. -Los anticuerpos antiSSa/Ro52 son frecuentes en pacientes con ES y no se asociaron con manifestaciones clínicas determinadas.Systemic Sclerosis (SSc) is an autoimmune disease with a high clinical and prognostic variability. The aims of these studies are to identify the associations between specific SSc-related antibodies and specific clinical and epidemiological profiles, and to prove the importance of the immunological pattern to predict the course and prognosis of the disease. METHODS A large cohort of SSc patients diagnosed in the Systemic Diseases Unit of Vall d'Hebron Hospital (Barcelone) was studied, all of whom fulfilled the classification criteria proposed by LeRoy and Medsger. Epidemiological, clinical and immunological data were collected according to a standard protocol. Three independent studies were performed focusing on the 3 most specific SSc-related antibodies (anticentromere [ACA], antitopoisomerase I [ATA-I] and anti-RNA polymerase [RNAp]), and on the antiSSa/Ro52 antibodies. In the first study 319 patients were recruited; both autoantibody subsets, ACA and ATA-I positive, were compared with SSc subsets (lcSSc and dcSSc). The second study comprised 175 patients with analysis of RNAp and the third study, 132 patients with analysis of antiSSa/Ro52. RESULTS: First study: Of 319 patients, 288 (90.3%) were women. Mean age at diagnosis was 51.5±15.1 years and mean age at disease onset was 43±15.9 years. ACA was the most frequent autoantibody (39.5%) followed by ATA-I (16.5%). Patients with ACA had a higher prevalence of esophageal involvement, interstitial lung disease (ILD), severe ILD, scleroderma renal crisis (SRC), digital ulcers (DU), joint involvement and of an active pattern in nailfold capillaroscopy. The incidence of Raynaud phenomenon (RP), RP as the first disease symptom, and a slow pattern in nailfold capillaroscopy were higher in patients with ACA. Cutaneous subsets distribution was as follows: limited cutaneous Ssc (lcSSc) 58.3%, diffuse cutaneous SSc (dSSc) 20%, SSc sine scleroderma (ssSSc) 14.7% and "prescleroderma" (pre-SSc) 6.9%. The frequency of the clinical manifestations according to the cutaneous subsets, lcSSc and dcSSc, was similar to the frequency according to the particular autoantibody status (ACA or ATA-I). Significant differences in disease presentation found on univariate analysis were as follows: ATA-I positivity, rather than dcSSc, was associated with higher frequency of esophageal involvement; instead, patients with lcSSc had a higher prevalence of isolated pulmonary arterial hypertension (PAH) compared with those with ACA; and dcSSc, rather than ATA-I positivity had more scleroderma-related deaths due to ILD. Patients with dcSSc had a trend to develop DU in comparison with patients with ATA-I. The multivariate studies showed that the contributory effect of the antibody status for the disease manifestations was very similar to that of the clinical categorization into lcSSc and dcSSc. dcSSc was an independent predictor factor for ILD and SRC, and ACA antibodies were an independent protector factor for esophageal involvement and ILD. Second study: Among the 175 selected patients 24 had RNAp. RNAp patients presented with higher incidence of dcSSc and met the ACR / EULAR 2013 diagnostic criteria more frequently than negative RNAp patients; they also had a lower incidence of RP as the first manifestation of the disease and a higher incidence of SCR. RNAp patients tended to develop DU and cancer compared with negative RNAp patients, without reaching statistical significance. Third study: of 132 patients, 87.1% were women; 51.5% had lcSSc. Prevalence of antiSSa/Ro52 was 35.6%. No association between antiSSa/Ro52 and clinical manifestations was found. A high percentage of patients presented ACA concomitantly with antiSSa/Ro52. CONCLUSIONS: -The classification according to immunological pattern (ATA-I and ACA) was as strongly associated with the course of SE as the categorization into cutaneous subtypes. -RNAp antibodies are associated with dcSSc, SRC and other symptoms different from RP at the onset of the disease. -AntiSSa/Ro52 antibodies are often found in SSc patients. No clinical manifestations, including inflammatory myopathy, were related with antiSSa/Ro52 antibodies

Anticuerpos antinucleares específicos de la esclerodermia como determinantes del pronóstico y de los diferentes patrones clínicos de la enfermedad

La esclerodermia (ES) es una enfermedad autoinmune con expresividad clínica y pronóstico muy variables. El objetivo de este estudio es establecer la asociación de algunos anticuerpos específicos y asociados a la ES con perfiles clínico-epidemiológicos determinados, y demostrar la importancia del patrón inmunológico para predecir el curso y pronóstico de la enfermedad. MÉTODOS: Se estudió una amplia cohorte de pacientes con ES visitados en la unidad de Enfermedades autoinmunes del Hospital Vall d'Hebron (Barcelona). Todos cumplían los criterios de clasificación propuestos por Leroy y Medsger. Se recogieron datos epidemiológicos, clínicos e inmunológicos de acuerdo con un protocolo estándar. Se realizaron 3 estudios independientes centrados en los tres anticuerpos más específicos de la enfermedad (anticentrómero (ACA), anti-Topoisomerasa (ATA-I) y anti-RNA polimerasa (RNAp)), y en el anticuerpo antiSSa-Ro52. En el primero se incluyeron 319 pacientes, se compararon los grupos de pacientes con ACA y ATA-I con los grupos con ES limitada y difusa; en el segundo se seleccionaron 175 pacientes con determinación para RNAp, y en el tercero 132 pacientes con determinación para antiSSa/Ro52. RESULTADOS: Primer estudio, de los 319 pacientes, 288 (90.3%) eran mujeres. La edad media al diagnóstico fue de 51.5±15.1 años y al inicio de la enfermedad 43±15.9 años. ACA fue el anticuerpo más frecuente (39.5%), seguido por ATA-I (16.5%). La afección esofágica, enfermedad pulmonar intersticial (EPI), EPI grave, crisis renal esclerodérmica (CRE), úlceras digitales (UD), clínica osteoarticular y el patrón activo en la capilaroscopia, fueron más frecuentes en el grupo ATA-I positivo. El fenómeno de Raynaud (FR) en general y como primera manifestación, y el patrón lento capilaroscópico fueron más prevalentes en el grupo ACA positivo. La distribución por subtipos cutáneos fue: ES limitada (58.3%), ES difusa (20%), ES sine ES (14.7%) y pre-ES (6.9%). La frecuencia de manifestaciones clínicas en los pacientes con ES limitada y difusa fueron muy similares a las identificadas en los pacientes clasificados por anticuerpo, ACA y ATA-I, respectivamente. En el análisis univariable se encontraron las siguientes diferencias entre ambas categorizaciones: ATA-I presentó más afección esofágica que la ES difusa; la ES limitada más hipertensión arterial pulmonar (HAP) aislada que el anticuerpo ACA; y la ES difusa más muertes por EPI que ATA-I. Hubo una mayor tendencia a presentar UD en el grupo ES difusa que en el ATA-I positivo. El análisis multivariable mostró que la contribución del perfil inmunológico sobre las principales complicaciones orgánicas es similar a la del perfil cutáneo. La ES difusa tiene más peso sobre la EPI y CRE, y el ACA es un factor protector independiente para la afección esofágica y EPI. En el segundo estudio, de los 175 pacientes seleccionados, 24 presentaban RNAp. En el grupo RNAp positivo fue más prevalente la ES difusa, los pacientes cumplieron más frecuentemente los criterios diagnósticos ACR/EULAR 2013, presentaron con menor frecuencia FR como primera manifestación de la enfermedad y desarrollaron más CRE. Hubo mayor tendencia a presentar UD y cáncer en el grupo con RNAp, sin llegar a la significación estadística. En el tercer estudio, de los 132 pacientes, 87.1% eran mujeres; 51.5% presentaban ES limitada. La prevalencia del antiSSa/Ro52 fue 35.6%. No se encontró ninguna asociación entre antiSSa/Ro52 y manifestaciones clínicas. Un alto porcentaje de pacientes presentó ACA concomitante con antiSSa/Ro52. CONCLUSIONES: -El perfil inmunológico (ATA-I y ACA) tiene un poder similar al subtipo cutáneo para predecir el curso de la enfermedad en ES. -Los anticuerpos RNAp se asocian con el subtipo cutáneo difusa, CRE y formas de inicio de la enfermedad diferentes al FR. -Los anticuerpos antiSSa/Ro52 son frecuentes en pacientes con ES y no se asociaron con manifestaciones clínicas determinadas.Systemic Sclerosis (SSc) is an autoimmune disease with a high clinical and prognostic variability. The aims of these studies are to identify the associations between specific SSc-related antibodies and specific clinical and epidemiological profiles, and to prove the importance of the immunological pattern to predict the course and prognosis of the disease. METHODS A large cohort of SSc patients diagnosed in the Systemic Diseases Unit of Vall d'Hebron Hospital (Barcelone) was studied, all of whom fulfilled the classification criteria proposed by LeRoy and Medsger. Epidemiological, clinical and immunological data were collected according to a standard protocol. Three independent studies were performed focusing on the 3 most specific SSc-related antibodies (anticentromere [ACA], antitopoisomerase I [ATA-I] and anti-RNA polymerase [RNAp]), and on the antiSSa/Ro52 antibodies. In the first study 319 patients were recruited; both autoantibody subsets, ACA and ATA-I positive, were compared with SSc subsets (lcSSc and dcSSc). The second study comprised 175 patients with analysis of RNAp and the third study, 132 patients with analysis of antiSSa/Ro52. RESULTS: First study: Of 319 patients, 288 (90.3%) were women. Mean age at diagnosis was 51.5±15.1 years and mean age at disease onset was 43±15.9 years. ACA was the most frequent autoantibody (39.5%) followed by ATA-I (16.5%). Patients with ACA had a higher prevalence of esophageal involvement, interstitial lung disease (ILD), severe ILD, scleroderma renal crisis (SRC), digital ulcers (DU), joint involvement and of an active pattern in nailfold capillaroscopy. The incidence of Raynaud phenomenon (RP), RP as the first disease symptom, and a slow pattern in nailfold capillaroscopy were higher in patients with ACA. Cutaneous subsets distribution was as follows: limited cutaneous Ssc (lcSSc) 58.3%, diffuse cutaneous SSc (dSSc) 20%, SSc sine scleroderma (ssSSc) 14.7% and "prescleroderma" (pre-SSc) 6.9%. The frequency of the clinical manifestations according to the cutaneous subsets, lcSSc and dcSSc, was similar to the frequency according to the particular autoantibody status (ACA or ATA-I). Significant differences in disease presentation found on univariate analysis were as follows: ATA-I positivity, rather than dcSSc, was associated with higher frequency of esophageal involvement; instead, patients with lcSSc had a higher prevalence of isolated pulmonary arterial hypertension (PAH) compared with those with ACA; and dcSSc, rather than ATA-I positivity had more scleroderma-related deaths due to ILD. Patients with dcSSc had a trend to develop DU in comparison with patients with ATA-I. The multivariate studies showed that the contributory effect of the antibody status for the disease manifestations was very similar to that of the clinical categorization into lcSSc and dcSSc. dcSSc was an independent predictor factor for ILD and SRC, and ACA antibodies were an independent protector factor for esophageal involvement and ILD. Second study: Among the 175 selected patients 24 had RNAp. RNAp patients presented with higher incidence of dcSSc and met the ACR / EULAR 2013 diagnostic criteria more frequently than negative RNAp patients; they also had a lower incidence of RP as the first manifestation of the disease and a higher incidence of SCR. RNAp patients tended to develop DU and cancer compared with negative RNAp patients, without reaching statistical significance. Third study: of 132 patients, 87.1% were women; 51.5% had lcSSc. Prevalence of antiSSa/Ro52 was 35.6%. No association between antiSSa/Ro52 and clinical manifestations was found. A high percentage of patients presented ACA concomitantly with antiSSa/Ro52. CONCLUSIONS: -The classification according to immunological pattern (ATA-I and ACA) was as strongly associated with the course of SE as the categorization into cutaneous subtypes. -RNAp antibodies are associated with dcSSc, SRC and other symptoms different from RP at the onset of the disease. -AntiSSa/Ro52 antibodies are often found in SSc patients. No clinical manifestations, including inflammatory myopathy, were related with antiSSa/Ro52 antibodies