Almost exclusive cohorts from Asia, Europe, and North America provide IgG4-related disease (IgG4-RD) data. Therefore, for a study, researchers sought to characterize the clinical presentation, phenotypic distribution, and correlation with sex, ethnicity, and serological markers in a sizable cohort of IgG4-RD patients from Latin America.

They conducted a multicenter medical records review research with 184 patients with IgG4-RD from Latin America. They divided the patients into 5 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). They concentrated the investigation on the potential effects of sex, ethnicity, and clinical phenotype on the clinical and serological presentation.

It was 50.8±15 years for the average age. About 52.2% of males and 48.8% of women were impacted equally. A total of 54 patients (29.3%) were divided into groups 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 linked to the involvement of the biliary system (odds ratio [OR], 3.4; 95% CI, 1.36–8.26), kidney (OR, 3.4; 95% CI, 1.28–9.25), and retroperitoneum (OR, 5.3; 95% CI, 1.45–20). Atopy history and gallbladder involvement were more common in Amerindian individuals. There were more women in group 3 than men.

In comparison to White and Asian cohorts, Latin American patients with IgG4-RD were younger and equally impacted by both men and women. They were more frequently seen in groups 1 and 3. Aortic and retroperitoneal involvement was uncommon. The clinical phenotype, ethnicity, and serological characteristics varied between sexes.

Reference: journals.lww.com/jclinrheum/Abstract/2022/09000/Clinical_and_Serological_Features_in_Latin.1.aspx