IgG4関連疾患による中枢・末梢神経障害

Central and Peripheral Nervous System Involvement in Immunoglobulin G4-Related Disease

はじめに

　IgG4関連疾患（IgG4-related disease）は血清IgG4高値，組織への著明なIgG4陽性形質細胞の浸潤，ステロイドに対する良好な反応性などを特徴とする全身性疾患である。障害臓器は膵臓をはじめとして多岐にわたるが，近年，神経内科領域のIgG4関連疾患として肥厚性硬膜炎，下垂体炎，末梢神経障害などが報告されている。これらの報告はまだ少数にとどまっており，診断方法や長期的な治療方法・予後についてはわかっていないことも多い。本稿ではIgG4関連疾患の総論，神経内科領域の各臓器病変について解説する。

Abstract

　Immunoglobulin G4-related disease (IgG4-RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4-RD can occur in various organs, including the pancreas, lacrimal gland, salivary gland, thyroid, lung, bile duct, liver, gastrointestinal tract, kidney, prostate, retroperitoneum, arteries, lymph nodes, skin, and breast. Steroid therapy is often effective. In the field of neurology, pachymeningitis (IgG4-related pachymeningitis) and hypophysitis (IgG4-related hypophysitis) are known to be related to IgG4-RD. Recently, a few papers have described the involvement of peripheral nerves in IgG4-RD. Here, we describe the concept of IgG4-RD and highlight the involvement of the central and peripheral nervous systems in IgG4-RD.