FROM GUIDELINES TO MISSED OPPORTUNITIES: A CRITICAL ANALYSIS OF ANCA VASCULITIS MANAGEMENT PRACTICES

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) represents a collection of rare autoimmune disorders that primarily target small blood vessels. This group includes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). The diagnosis of AAV is multifaceted, relying on a combination of clinical assessments, biological markers, radiological imaging, and histopathological evaluations.Although the revised Chapel Hill classification has refined disease definitions, the absence of ANCAs in some patients can complicate diagnosis.

The management of AAV typically begins with an induction phase aimed at inducing remission. This phase often incorporates corticosteroids (CS) or Avacopan in conjunction with immunosuppressants such as Rituximab (RTX) or Cyclophosphamide (CYC). Following this initial treatment, patients generally transition to maintenance therapy. Despite significant advancements in the understanding and treatment of AAV, challenges persist regarding accurate prognosis and therapeutic management.

To evaluate disease severity and inform treatment decisions, clinicians utilize tools such as the Five-Factor Score (FFS) and the Birmingham Vasculitis Activity Score (BVAS). However, these assessment instruments have inherent limitations and may not fully encapsulate the complexity of the disease. Recent epidemiological research has underscored geographic variability in the incidence of AAV and highlighted the role of ANCAs as crucial diagnostic and prognostic markers.Evaluating long-term sequelae using indices such as the Vasculitis Damage Index (VDI) is essential, especially given improved survival rates and an increasing focus on enhancing patients quality of life.Current treatment strategies aim to minimize relapses and manage complications, including infections and metabolic disturbances however, there is a pressing need for more personalized approaches.This review emphasizes the importance of developing more sophisticated prognostic tools and activity scores to improve clinical management of AAV. It also advocates for continued research to optimize treatment strategies and enhance outcomes for patients affected by these challenging conditions.