免疫グロブリンG4関連脊髄穿孔症

Immunoglobulin G4-related spinal pachymeningitis.

• Mousa N Alrashdi

抄録

免疫グロブリンG4関連疾患（IgG4-RD）は稀な疾患実体であり、最近では多臓器に影響を及ぼす傾向のある炎症性疾患として認識されています。IgG4関連疾患に起因する脊椎の髄膜炎は極めて稀です。IgG4関連疾患による脊椎多発性髄膜炎の通常の症状は頸部痛と脊髄圧迫であるが，多発性関節炎は珍しい疾患であり，乾癬性関節炎を模倣した1例のみが報告されている．本報告では，IgG4関連の脊椎穿孔炎を呈した中年男性の1例を紹介する．頸部磁気共鳴画像（MRI）では、頸髄肥厚が認められ、血清IgG4値が上昇していた。グルココルチコイドとリツキシマブによる治療を受けたが，放射線学的に有意に陽性であった．

Immunoglobulin G4-related disease (IgG4-RD) is rare disease entity and has recently been recognized as an inflammatory disorder with the tendency to affect multiple organs. Pachymeningitis of spine caused by IgG4-related disease is extremely rare. Neck pain and spinal cord compression symptoms consist of usual presentation of IgG4-related spinal pachymeningitis; however, polyarthritis is an unusual presentation of this disease, and it was reported in only one case that mimicked psoriatic arthritis. In this report, we describe a case of IgG4-related spinal pachymeningitis in a middle-age male who presented initially with neck pain and  rheumatoid arthritis-like symptoms and later on developed both right upper and lower limb weakness. He was found to had pachymeningeal thickening on a cervical magnetic resonance image (MRI) and elevated serum IgG4 levels. He was treated with glucocorticoids and rituximab, which led to a significantly positive radiological response.