Perfil Demográfico, Clínico e Laboratorial de Pacientes com Vasculites ANCA-associadas de um Centro Terciário do Nordeste Brasileiro

Abstract

Introduction: ANCA-associated vasculitides (AAVs) are a group of rare autoimmune diseases with limited epidemiological data, especially in Brazil. Most data are derived from European studies, although significant geographic variation is known. This study aimed to describe the demographic, clinical, and laboratory profiles of these patients in a specialized center in Northeastern Brazil. Methods: This was a cross-sectional study conducted through collection of demographic, clinical, laboratory, and imaging data from the medical records of patients diagnosed with AAVs between 2018 and 2024. Results: A total of 36 patients were included: 20 with GPA, 13 with EGPA, and 3 with MPA. Among GPA patients, the mean age at diagnosis was 47.15 years old, 75% were female, and 60% were multiethnic. The most common form was generalized GPA. Among those with the limited form, all tested positive for ANCA. Involvement of specific systems included: kidneys (55%), upper airways (65%), lower airways (65%), central nervous system (15%), peripheral nervous system (25%), skin (40%), and heart (10%). In EGPA patients, the mean age at diagnosis was 52.5 years old, 53.8% were female, and 60% were multiethnic. System involvement included: kidneys (7.6%), upper airways (46.1%), lower airways (100%), central nervous system (15.3%), peripheral nervous system (84.6%), skin (76.9%), and heart (7.6%). The three MPA cases were all female patients, two of mixed-race, with a mean diagnosis age of 50 years. All had renal involvement; two had pulmonary involvement, and two showed peripheral nervous system abnormalities. Conclusion: These findings align with global literature. However, women and mixed-race individuals (80% non-white) predominated, differing from global data that report similar sex distribution and a higher prevalence among caucasians.