肥厚性脊椎硬膜炎により急速に四肢麻痺が進行した全身性強皮症/シェーグレン症候群オーバーラップの一例

• Published in: 臨床リウマチ Vol. 26; no. 3; pp. 176 - 183
• Main Authors: 齋藤, 榮子, 鈴木, 康夫, 中村, 直哉, 本田, 桐, 山田, 千穂, 知念, 直史, 佐藤, 慎二, 野木, 真一, 佐々木, 則子
• Format: Journal Article
• Language: Japanese
• Published: 一般社団法人 日本臨床リウマチ学会 2014; The Japanese Society for Clinical Rheumatology and Related Research
• Subjects: cervical thickened epidural lesion; hypertrophic spinal pachymeningitis; magnetic resonance imaging (MRI); Sjogren syndrome; systemic sclerosis
• ISSN: 0914-8760; 2189-0595
• DOI: 10.14961/cra.26.176

   We report a 61-year-old woman with overlap syndrome with systemic sclerosis(SSc) and Sjögren syndrome(SS) who developed neck pain and progressive quadriplegia. At the time of admission, magnetic resonance imaging(MRI) revealed cervical cord compression by thickened epidural lesions and emergency laminectomy was performed. Epidural specimen showed fibrosis with lymphoid cell infiltration. She was diagnosed as hypertrophic spinal pachymeningitis associated with SSc and SS, and treated with high-dose corticosteroid therapy. Thereafter she showed remarkable improvement in quadriplegia and the cervical thickened epidural lesion on MRI. To the best of our knowledge, this is a rare case of the overlap syndrome with SSc and SS patient complicated with hypertrophic spinal pachymeningitis. In this case, MRI was useful for differentiating hypertrophic spinal pachymeningitis from other conditions responsible for acute progressive quadriplegia.    強皮症，シェーグレン症候群にて治療中の61歳女性．頚部痛・四肢麻痺にて緊急入院．頚椎MRI にて頚髄の背側主体に硬膜肥厚および頚髄の圧迫を認めた．緊急で脊柱管拡大術が施行された．硬膜病理では線維性組織の増生とリンパ球主体の著明な細胞浸潤を認めた．自己免疫性疾患関連の肥厚性脊椎硬膜炎の診断となり，高用量ステロイド投与を行ったところ，硬膜肥厚の改善および四肢麻痺の改善を認めた．