髄膜炎の再燃を複数回生じたVogt-小柳-原田病の1例

A case of Vogt-Koyanagi-Harada disease with multiple recurrences of meningitis

要約　目的：主として髄膜炎の再燃を複数回生じた稀なVogt-小柳-原田病（VKH病）の1例の報告。

症例：16歳男性が近医で無菌性髄膜炎にて加療され，軽快した1か月後に両眼の充血と視力低下で当科を受診した。両眼の虹彩毛様体炎，視神経乳頭の発赤・腫脹を認めた。各種眼科画像検査と髄液検査結果から不全型VKH病と診断した。ステロイド大量漸減療法にてぶどう膜炎は軽快したものの，漸減中に二度の髄膜炎を主体とした再燃を生じ，ステロイドパルス療法にて軽快した。以後18か月後まで，ぶどう膜炎，髄膜炎ともに再燃なく経過している。

結論：本症例ではステロイドの減量が速かったことに加え，ぶどう膜炎よりも髄膜炎のほうが消炎により多くのステロイドを要したため，髄膜炎を主体とした再燃を生じたものと推察した。

Abstract　Purpose：To report a rare case of Vogt-Koyanagi-Harada (VKH) disease with recurrent meningitis.

Cases：A 16-year-old male presented with a 1-week history of conjunctival hyperemia and decreased vision in both eyes. He had been treated with the diagnosis of aseptic meningitis by a local neurologist one month before the presentation.

Findings and Clinical Course：Ophthalmic examination showed iridocyclitis and optic disc hyperemia and swelling in both eyes. The diagnosis of incomplete VKH disease was made according to the additional examinations including fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography, and cerebrospinal fluid examination. High-dose corticosteroid therapy was initiated and tapered, which led to the improvement of uveitis. However, two times of recurrent meningitis without iridocyclitis were observed while the corticosteroid was tapered. Then he was treated with pulsed corticosteroid therapy, which led to the remission of meningitis and uveitis.

Conclusion：We assumed that one of the reasons for the recurrent meningitis without definite uveitis may be the rapid tapering of corticosteroid. Another reason may be that the amount of corticosteroid to suppress the inflammation may be larger for the meningitis than that for the uveitis in this VKH patient.