サリドマイドが著効した小児難治性腸管Behçet病の2例

Dramatic Improvement with Thalidomide Therapy in Two Juvenile Patients with Severe Entero-Behçet's Disease, Report of Two Cases

要旨　〔症例1〕は15歳，男性．9歳時発症の難治性の腸管Behçet病．複数の免疫抑制薬を併用したにもかかわらず，ステロイド依存性となり，12歳時にステロイド性骨粗鬆症による脊椎圧迫骨折を合併した．13歳時，病変の悪化から穿孔性腹膜炎に罹患し，癒着性腹膜炎治療のため回盲部切除術を施行された．〔症例2〕は12歳，男児．9歳時発症の難治性の腸管Behçet病．ステロイドへの反応は良好であったが，減量中再燃と寛解を繰り返した．各種免疫抑制薬を併用するも，プレドニゾロンを15mg/dayから減量できなかった．2例ともサリドマイド治療は極めて有効で，〔症例1〕では臨床症状および血液検査所見が著しく改善した．〔症例2〕ではサリドマイド治療を開始後，再燃徴候はなく経過し，ステロイドや免疫抑制薬を減量できた．最終的に2例ともサリドマイド以外の薬剤の中止が可能であった．

　〔Case 1〕 A 15-year-old boy had a 6-year history of severe entero-Behçet disease. In spite of the use of a number of immunosuppressive drugs, he became steroid-dependent and developed severe osteoporosis. Eventually, he developed vertebral bone fracture at the age of 12 years. In addition, perforated peritonitis had occurred at the age of 13 years.

　〔Case 2〕 A 12-years-old boy had a 3-year history of recurrent entero-BD. Prednisolone was started, initially resulting in remarked improvement. However, he also became steroid-dependent. In spite of quite a few immunosuppressive drugs, over a 15mg/day dose of predonisolone was needed because of frequent disease recurrence.

　Thalidomide therapy was extremely effective for both patients. 〔Case 1〕 patient showed dramatic improvements in his symptoms and laboratory parameters. 〔Case 2〕 patient exhibited no signs of relapsed symptoms and parameters when predonisolone and immunosuppressive drugs were reduced. Finally, both patients haven't needed any drugs except thalidomide to control the disease.