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...

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Autores principales: Martín-Nares, Eduardo, Baenas, Diego Federico, Cuellar Gutiérrez, María Carolina, Hernández-Molina, Gabriela, Ortiz, Alberto Christian, Neira, Oscar, Gutiérrez, Miguel A., Calvo, Romina, Saad, Emanuel José, Elgueta Pinochet, Sergio, Gallo, Jesica, Herrera Moya, Alejandra, Mansilla Aravena, Bellanides Agustina, Crespo Espíndola, María Elena, Cairoli, Ernesto, Bertoli, Ana María, Córdoba, Mercedes, Wurmann Kiblisky, Pamela, Basualdo Arancibia, Washington Javier, Badilla Piñeiro, María Natalia, Gobbi, Carla Andrea, Berbotto, Guillermo Ariel, Pisoni, Cecilia N., Juárez, Vicente, Cosatti, Micaela Ana, Aste, Nora María, Airoldi, Carla, Llanos, Carolina, Vergara Melian, Cristian Fabián, Erlij Opazo, Daniel, Goecke, Annelise, Pastenes Montaño, Paula Andrea, Tate, Patricio, Pirola, Juan Pablo, Stange Núñez, Lilith, Burgos, Paula I., Mezzano Robinson, María Verónica, Michalland H, Susana, Silva Labra, Francisco, Labarca Solar, Cristián Humberto, Lencina, María Verónica, Izquierdo Loaiza, Jorge Hernán, Del Castillo Gil, David Julián, Caeiro, Francisco, Paira, Sergio
Formato: Artículo
Lenguaje:Español
Publicado: Lippincott Williams and Wilkins 2022
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Acceso en línea:http://pa.bibdigital.ucc.edu.ar/3334/1/Mart%C3%ADn-Nares_Baenas_Cuellar.pdf
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Sumario: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.