Abstract
Systemic vasculitides are rare and heterogeneous diseases affecting different organs and systems with varying degrees of severity depending on the type of vessels affected. The etiology and pathogenesis are unclear. Immunе mechanisms play a role in the pathogenesis: deposition of immune complexes, autoantibodies (anti-endothelial and anti-neutrophil cytoplasmic antibody, cellular and molecular responses, granulomas, and endothelial cell damage. All vasculitides are “rare diseases”. ANCA- associated vasculitis has an incidence of 20 cases/1 million population. Without treatment, mortality is >90% within up to 5 years. We conducted a two-centre, retrospective, observational, non- interventional, epidemiological, health-demographic, clinical-pharmacological study to evaluate ANCA-associated vasculitis in Bulgaria. From 2018 to 2021, we screened 12 individuals with Wegener’s granulomatosis. The analyzed population is approximately 60% of the patients in Bulgaria. The time from symptom onset to diagnosis is short, but the diagnosis is made at an advanced stage of the disease - the measured BVAS version 3 activity is moderate-severe.