再発性多発性軟骨炎に視神経腫脹を伴った1例

Optic disc edema in a case of relapsing polychondritis

　62歳男性が耳介の腫脹，手背の疼痛と両眼球結膜の浮腫を伴い近医眼科を受診した。浅前房，前房炎症と視神経腫脹が出現し，頭痛，めまいも伴うようになり当院に紹介された，MRI上は視神経炎や強膜炎を疑わせる所見に乏しく，視野と視力の障害が軽度であつた。再発性多発性軟骨炎と診断し，ステロイドパルス療法を施行した。他の症状は改善したが，視神経腫脹が残存したためシクロフォスファミドを併用し，有効であった。さらにステロイドへの反応性が低かったこともあり，この症例の視神経腫脹は，再発性多発性軟骨炎に合併するとされている視神経炎以外に，強膜篩板や視神経鞘内膜の不整による脳脊髄液のうっ滞などの病態が関与している可能性が高いと考えられた。

A 62-year-old man developed bilateral chemosis, swollen earlobes and pain in the back of his hands, followed later by headache and vertigo. He was referred to us following detection of inflammation in the anterior chamber and edema of optic disc in both eyes. He showed minimum disturbances in visual acuity and visual field. Magnetic resonance imaging (MRI) showed no obvious signs of optic neuritis or scleritis. Following systemic pulse corticosteroid therapy after diagnosis of relapsing polychondritis, all symptoms improved excepting optic disc edema. Additional cyclophosphamide resulted in clinical cure. Persistent optic disc edema following systemic corticosteroid seemed to suggest that it was induced not only by optic neuritis, which frequently accompanies relapsing polychondritis, but also by congestion of cerebrospinal fluid affecting the lamina cribrosa and the internal membrane of the optic nerve sheath.