Clinicopathologic Findings in Mass Forming ANCA-Associated Vasculitis

Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic inflammatory disease predominantly affecting small blood vessels, with few or no immune deposits, associated with myeloperoxidase ANCA or proteinase 3 ANCA.1 AAV typically presents with constitutional symptoms, involvement of upper and/or lower respiratory tract, and kidneys.2-4 AAV can form inflammatory masses in various organs, including the orbit, kidney, and even pancreas, similar to IgG4-related disease (IgG4-RD).