Nationwide epidemiological survey of polyarteritis nodosa in Japan in 2020

Mod Rheumatol. 2024 Feb 7:roae010. doi: 10.1093/mr/roae010. Online ahead of print.

Abstract

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that predominantly affects medium-sized arteries. With advances in our understanding of the pathogenesis and classification of vasculitis, PAN and microscopic polyangiitis (MPA), a disease of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), were separated from the group of diseases previously diagnosed as periarteritis nodosa (PN) at the Chapel Hill Consensus Conference (CHCC) in 1994 (1).

Keywords: Japan; epidemiology; polyarteritis nodosa; prevalence; systemic vasculitis.