Nationwide epidemiological survey of polyarteritis nodosa in Japan in 2020

Le résumé fourni par la source

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]. PAN is designated as an intractable disease by the Ministry of Health, Labour and Welfare (MHLW) in Japan; although the number of registered patients in the fiscal year of 2020 was 2347 [2], the criterion for its designation as a severe disease is severity category 3 or higher [3]. Therefore, patients below that level and those who do not wish to receive subsidized medical expenses are not registered. In addition, since PAN was registered as PN together with MPA until the fiscal year of 2005 and patients with MPA may be mixed among those currently registered as PAN, the exact number of patients with PAN is unknown. Therefore, we conducted a nationwide epidemiological study to estimate the number and sex ratio of patients with PAN in Japan.

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.