IgG4関連疾患の臓器病変と病理

Pathological features of IgG4-related disease

IgG4関連疾患は，いずれの臓器に発生しても基本的な病理学的所見は共通しているが，いくつか臓器特異的な変化も知られている．また，最近の解析により，頭頸部病変で女性の比率が高いこと，腎病変が単独で発生することは稀であることなど，臓器別の臨床的特徴も明らかとなりつつある．本稿では，IgG4関連疾患の病理学的特徴と，臓器特異的変化について，総論的・横断的に解説する．

IgG4-related disease shares most clinicopathological characteristics irrespective of the organ of origin.　However, a recent study revealed some organ specific features : for example, the female ratio is higher in patients with sialadenitis or dacryoadenitis, and all kidney lesions are associated with extra-renal disease.　Unique pathological features are dense fibrosis in dacryoadenitis, numerous lymph follicles in sialadenitis and dacryoadenitis, and obliterative arteritis in lung lesions.　In this report, we describe pathological characteristics and some organ specific features of IgG4-related disease.