眼部病変を契機に診断された多発血管炎性肉芽腫症（Wegener肉芽腫症）

Cases of granulomatosis with polyangiitis（Wegener's）diagnosed through ocular lesions

要約　目的：眼部病変を契機に多発血管炎性肉芽腫症（GPA）と診断された症例を検討した。対象と方法：神戸海星病院または神戸大学附属病院を眼科受診し，所見からGPAを疑い診断に至った6症例を対象とし，診療録の後ろ向き調査を行った。結果：男女比2：1，初診時年齢37～70歳（中央値62歳），初発病変は眼窩病変3例，強膜炎3例であった。病理所見または臨床所見とPR3-ANCA陽性から確定診断した。眼窩生検した3例は病理診断できなかった。初診から診断までの期間は1か月未満～219か月（中央値4か月）で，最終視力は2眼で光覚なしとなった。結論：眼部所見からGPAを早期診断する方法の確立が望まれる。

Abstract.　Purpose：To report patients who were diagnosed as granulomatosis with polyangiitis（GPA）with ocular symptoms as the initial manifestations. Cases：This retrospective study was made on 6 patients of GPA who were seen during 20 years through 2011. Findings：The series comprised 4 males and 2 females. The age ranged from 37 to 70 years, with 62 years as the median. Initial ocular lesions comprised orbital mass and scleritis in 3 cases each. Diagnosis was made either histopathologically or clinical findings, or PR3-ANCA positive data. Orbital biopsy in 3 cases showed inconclusive findings. Interval between initial visit and diagnosis ranged from one month to 219 months, with 4 months as the median. Two eyes resulted in ultimate visual loss. Conclusion：An urgent need is present for criteria to facilitate early diagnosis of GPA with ocular manifestations.