Nervous system involvement in ANCA-associated vasculitis: Single center experience from Latin America

Introduction: Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) present heterogeneous neurological symptoms that are often misdiagnosed, contributing to delays in identification and prompt treatment. Few studies in Latin America have described the frequency of neurological involvement in AVV; none have explicitly described the characteristics of nervous system involvement. Design/Methods: This case-control study examined patient records for AVV treated at a university hospital in Colombia between 2005 and 2023. Patients with and without neurological manifestations were compared and a survival analysis was performed. Results: Forty-eight cases and seventy-nine controls were included. The median age was 58 years, 57.5 % were female. The diagnosis was made in 67.7 % of cases during the hospital stay, and in-hospital mortality was 14 %. Nervous system involvement was more frequent in undifferentiated AAV (100 %), followed by eosinophilic granulomatosis with polyangiitis (75 %), microscopic polyangiitis (33.3 %), and granulomatosis with polyangiitis (25.9 %). The most common neurological manifestations were peripheral neuropathy (50 %), patient-reported symptoms of sensory dysfunction (43.7 %), and cranial neuropathy (39.6 %); headache was frequent among patients with neurological involvement. Patients with neurological manifestations presented a lower median creatinine at admission and a lower proportion of patients with a five-factor score > 2. No differences in one-year all-cause mortality were observed. Conclusions: This study presents an exhaustive clinical characterization of the neurological profile of patients with AAV from a single center in Latin America. Patients with nervous system involvement showed less severe renal involvement and a lower proportion of 5-year risk of mortality scores; one-year all-cause mortality was similar between groups.