Lilith Stange Núñez, Vicente Juárez, Micaela Ana Cosatti, Nora María Aste, Carla Airoldi, Carolina Llanos, Cristian Fabián Vergara Melian, Daniel Erlij Opazo, Annelise Goecke, Paula Andrea Pastenes Montaño, Patricio Tate, Juan Pablo Pirola, Cecilia N Pisoni, Paula I Burgos, María Verónica Mezzano Robinson, Susana Michalland H, Francisco Silva Labra, Cristián Humberto Labarca Solar, María Verónica Lencina, Jorge Hernán Izquierdo Loaiza, David Julián Del Castillo Gil, Francisco Caeiro, Sergio Paira, Alejandra Herrera Moya, Diego Federico Baenas, María Carolina Cuellar Gutiérrez, Gabriela Hernández-Molina, Alberto Christian Ortiz, Oscar Neira, Miguel A Gutiérrez, Romina Calvo, Emanuel José Saad, Sergio Elgueta Pinochet, Jesica Gallo, Eduardo Martín-Nares, Bellanides Agustina Mansilla Aravena, María Elena Crespo Espíndola, Ernesto Cairoli, Ana María Bertoli, Mercedes Córdoba, Pamela Wurmann Kiblisky, Washington Javier Basualdo Arancibia, María Natalia Badilla Piñeiro, Carla Andrea Gobbi, Guillermo Ariel Berbotto
Journal: Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases 2022;28(6):285-292
PMID: 35612589
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.
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