血管炎の新しい分類と基本的な考え方—中枢神経血管炎の位置づけ

Central Nervous System Vasculitis According to the 2012 Revise International Chapel Hill Consensus Conference Nomenclature of Vasculitides

2012年に改訂されたInternational Chapel Hill Consensus Conference Nomenclature of Vasculitides（CHCC 2012）を中心に血管炎の分類と基本的な考え方，およびCHCC 2012における中枢神経血管炎の位置づけ，問題点などについて述べる。CHCC 2012の対象疾患は改訂前に比べて大幅に拡大されており，単一臓器の血管炎として原発性中枢神経系血管炎も記載された。今後，CHCC 2012はさまざまな分野で利用されていくものと予想される。

Abstract

Vasculitis can be primarily or secondarily to various underlying diseases. It frequently affects the nervous system, and neurological deficits may remain even after disease remission. Because the progression of vasculitides is usually acute to subacute, early initiation of treatment is important from the viewpoint of patients' functional status. However, early diagnosis may be difficult, particularly in patients with central nervous system vasculitis. Hence, it is important to understand the wide-ranging clinical manifestations of vasculitides. Here, we summarize the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides from the standpoint of central nervous system vasculitis.