Clinical and Serological Features in Latin American IgG4-Related Disease Patients Differ According to Sex, Ethnicity, and Clinical Phenotype

Background/Objective Data on IgG4-related disease (IgG4-RD) come almost exclusively from cohorts from Asia, Europe, and North America. We conducted this study to describe the clinical presentation, phenotype distribution, and association with sex, ethnicity, and serological markers in a large cohort of Latin American patients with IgG4-RD. Methods We performed a multicenter medical records review study including 184 Latin American IgG4-RD patients. We assigned patients to clinical phenotypes: group 1 (pancreato-hepato-biliary), group 2 (retroperitoneal/aortic), group 3 (head and neck-limited), group 4 (Mikulicz/systemic), and group 5 (undefined). We focused the analysis on how sex, ethnicity, and clinical phenotype may influence the clinical and serological presentation. Results The mean age was 50.8 ± 15 years. Men and women were equally affected (52.2% vs 48.8%). Fifty-four patients (29.3%) were assigned to group 1, 21 (11.4%) to group 2, 57 (30.9%) to group 3, 32 (17.4%) to group 4, and 20 (10.8%) to group 5. Male sex was associated with biliary tract (odds ratio [OR], 3.4; 95% confidence interval [CI], 1.36-8.26), kidney (OR, 3.4; 95% CI, 1.28-9.25), and retroperitoneal involvement (OR, 5.3; 95% CI, 1.45-20). Amerindian patients presented more frequently with atopy history and gallbladder involvement. Group 3 had a female predominance. Conclusions Latin American patients with IgG4-RD were younger, and men and women were equally affected compared with White and Asian cohorts. They belonged more commonly to group 1 and group 3. Retroperitoneal and aortic involvement was infrequent. Clinical and serological features differed according to sex, ethnicity, and clinical phenotype.Fil: Martín-Nares, Eduardo. Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. Department of Immunology and Rheumatology; MéxicoFil: Baenas, Diego Federico. Hospital Privado Universitario de Córdoba. Servicio de Reumatología; ArgentinaFil: Cuellar Gutiérrez, María Carolina. Hospital Del Salvador. Departamento de Medicina Interna. Servicio de Reumatología; ChileFil: Hernández-Molina, Gabriela. Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. Department of Immunology and Rheumatology; MéxicoFil: Ortiz, Alberto Christian. Hospital José María Cullen. Sección de Reumatología; ArgentinaFil: Neira, Oscar. Universidad de Chile. Hospital Del Salvador. Sección Reumatología; ChileFil: Neira, Oscar. Clínica Alemana de Santiago-Universidad Del Desarrollo. Unidad Reumatología; ChileFil: Gutiérrez, Miguel A. Universidad de Valparaíso. Hospital Naval Almirante Nef. Departamento de Reumatologia; ChileFil: Calvo, Romina. Hospital José María Cullen. Sección de Reumatología; ArgentinaFil: Saad, Emanuel José. Hospital Privado Universitario de Córdoba. Departamento de Clínica Médica; ArgentinaFil: Elgueta Pinochet, Sergio. Hospital Clínico de la Universidad de Chile. Sección Reumatología. Departamento de Medicina; ChileFil: Gallo, Jesica. Hospital Central de Reconquista. Sección de Reumatología; ArgentinaFil: Herrera Moya, Alejandra. Pontificia Universidad Católica de Chile. Departamento de Inmunología Clínica y Reumatología; ChileFil: Mansilla Aravena, Bellanides Agustina. Hospital Clínico Magallanes; ArgentinaFil: Crespo Espíndola, María Elena. Hospital Señor Del Milagro; ArgentinaFil: Cairoli, Ernesto. Hospital Evangélico. Unidad de Enfermedades Autoinmunes; BrasilFil: Cairoli, Ernesto. Centro Asistencial Del Sindicato Médico Del Uruguay. Unidad de Enfermedades Autoinmunes; UruguayFil: Cairoli, Ernesto. Institut Pasteur. Laboratorio de Inmunorregulación e Inflamación; UruguayFil: Bertoli, Ana María. Universidad Católica de Córdoba. Clínica Universitaria Reina Fabiola. Servicio de Reumatología; ArgentinaFil: Córdoba, Mercedes. Universidad Católica de Córdoba. Clínica Universitaria Reina Fabiola. Servicio de Reumatología; ArgentinaFil: Wurmann Kiblisky, Pamela. Hospital Clínico Universidad de Chile.Fil: Basualdo Arancibia, Washington Javier. Departamento de Medicina. Sección Reumatología; ChileFil: Badilla Piñeiro, María Natalia. Hospital Del Salvador, Universidad de Chile. Sección Reumatología; ChileFil: Gobbi, Carla Andrea. Universidad Nacional de Córdoba. Facultad de Ciencias Médicas. Hospital Córdoba; ArgentinaFil: Berbotto, Guillermo Ariel. Sanatorio Británico. Servicio de Reumatología; ArgentinaFil: Pisoni, Cecilia N. Centro de Educación Médica e Investigaciones Clínicas Norberto Quirno. Sección Reumatología e Inmunología; ArgentinaFil: Juárez, Vicente. Hospital Señor Del Milagro; ArgentinaFil: Cosatti, Micaela Ana. Centro de Educación Médica e Investigaciones Clínicas Norberto Quirno. Sección Reumatología e Inmunología; ArgentinaFil: Aste, Nora María. Reumatología; ArgentinaFil: Airoldi, Carla. Hospital Provincial. Reumatología; ArgentinaFil: Llanos, Carolina. Pontificia Universidad Católica de Chile. Departamento de Inmunología Clínica y Reumatología; ArgentinaFil: Vergara Melian, Cristian Fabián. Hospital San Martin de Quillota; ChileFil: Vergara Melian, Cristian Fabián. Clinica Ciudad Del Mar; ChileFil: Erlij Opazo, Daniel. Universidad de Chile. Hospital Del Salvador. Departamento de Medicina Oriente; ChileFil: Goecke, Annelise. Hospital Clínico Universidad de Chile. Departamento de Medicina. Servicio de Reumatología; ChileFil: Pastenes Montaño, Paula Andrea. Hospital Carlos Van Buren. Servicio de Medicina. Departamento de Reumatología; ChileFil: Tate, Patricio. Organización Médica de Investigación; ArgentinaFil: Pirola, Juan Pablo. Sanatorio Argentino; ArgentinaFil: Stange Núñez, Lilith. Clínica Ciudad Del Mar. Centro de Artritis Reumatoide; ChileFil: Burgos, Paula I. Pontificia Universidad Católica de Chile. Departamento de Inmunología Clínica y Reumatología; ChileFil: Mezzano Robinson, María Verónica. Hospital Del Salvador. Clínica Las Condes; ChileFil: Michalland H, Susana. Universidad de Chile. Hospital Del Salvador. Sección Reumatología; ChileFil: Silva Labra, Francisco. Hospital Padre Hurtado. Facultad de Medicina Clínica Alemana-Universidad Del Desarrollo; ChileFil: Labarca Solar, Cristián Humberto. Hospital Padre Hurtado. Facultad de Medicina Clínica Alemana-Universidad Del Desarrollo; ChileFil: Lencina, María Verónica. Hospital Señor Del Milagro; ArgentinaFil: Izquierdo Loaiza, Jorge Hernán. Clínica de Occidente S.A. Grupo de Reumatología; ColombiaFil: Del Castillo Gil, David Julián. Clínica de Occidente S.A. Grupo de Reumatología; ColombiaFil: Caeiro, Francisco. Hospital Privado Universitario de Córdoba. Servicio de Reumatología; ArgentinaFil: Paira, Sergio. Hospital José María Cullen. Sección de Reumatología; Argentin

Second asymptomatic carotid surgery trial (ACST-2) : a randomised comparison of carotid artery stenting versus carotid endarterectomy

Background: Among asymptomatic patients with severe carotid artery stenosis but no recent stroke or transient cerebral ischaemia, either carotid artery stenting (CAS) or carotid endarterectomy (CEA) can restore patency and reduce long-term stroke risks. However, from recent national registry data, each option causes about 1% procedural risk of disabling stroke or death. Comparison of their long-term protective effects requires large-scale randomised evidence. Methods: ACST-2 is an international multicentre randomised trial of CAS versus CEA among asymptomatic patients with severe stenosis thought to require intervention, interpreted with all other relevant trials. Patients were eligible if they had severe unilateral or bilateral carotid artery stenosis and both doctor and patient agreed that a carotid procedure should be undertaken, but they were substantially uncertain which one to choose. Patients were randomly allocated to CAS or CEA and followed up at 1 month and then annually, for a mean 5 years. Procedural events were those within 30 days of the intervention. Intention-to-treat analyses are provided. Analyses including procedural hazards use tabular methods. Analyses and meta-analyses of non-procedural strokes use Kaplan-Meier and log-rank methods. The trial is registered with the ISRCTN registry, ISRCTN21144362. Findings: Between Jan 15, 2008, and Dec 31, 2020, 3625 patients in 130 centres were randomly allocated, 1811 to CAS and 1814 to CEA, with good compliance, good medical therapy and a mean 5 years of follow-up. Overall, 1% had disabling stroke or death procedurally (15 allocated to CAS and 18 to CEA) and 2% had non-disabling procedural stroke (48 allocated to CAS and 29 to CEA). Kaplan-Meier estimates of 5-year non-procedural stroke were 2·5% in each group for fatal or disabling stroke, and 5·3% with CAS versus 4·5% with CEA for any stroke (rate ratio [RR] 1·16, 95% CI 0·86-1·57; p=0·33). Combining RRs for any non-procedural stroke in all CAS versus CEA trials, the RR was similar in symptomatic and asymptomatic patients (overall RR 1·11, 95% CI 0·91-1·32; p=0·21). Interpretation: Serious complications are similarly uncommon after competent CAS and CEA, and the long-term effects of these two carotid artery procedures on fatal or disabling stroke are comparable