Wegener肉芽腫症に合併した若年者涙腺腫瘍の1例

Wegener's granulomatosis with lacrimal gland tumor in a young adult

　17歳女性が右眼瞼の腫脹と疼痛で受診した。11歳で慢性腎炎があり，1年前に腎生検で巣状糸球体硬化症と診断されている。磁気共鳴画像検査（MRI）などで右涙腺部に腫瘍が同定された。眼窩と肺の生検で炎症性のリンパ球浸潤像があり，抗好中球細胞質抗体（ANCA）はP-ANCA, C-ANCAともに陽性であることから，Wegener肉芽腫症と診断した。本症では眼合併症として眼窩病変が多いが，若年者では稀であり，その意昧で本症例は注目される。

A 17-year-old female presented with painful swelling in her right eyelid. She had had chronic ne-phritis at the age of 11 years. She had been diagnosed with focal glomerulosclerosis by renal biopsy one year before. A lacrimal gland tumor in the right orbit was identified by diagnostic imaging including magnetic reso-nance imaging (MRI). Biopsy of the orbit and the lung showed lymphoid infiltration of inflammatory nature. Anti-neutrophil cytoplasmic autoantibody (ANCA) was positive both for P-ANCA and C-ANCA. These find-ings led to the diagnosis of Wegener's granulomatosis. The present case merits due attention because orbital tumor develops at a more advanced age in this disease entity.