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ベーチェット病(以下ベ病)と類似の眼症状を呈し,他の全身症状を欠くか,または眼症状以外にはベ病としての特異性が少ない1症状のみを伴う24例の診断について検討した.これらのうち,経過中にベ病と確定診断できたものを除く症例を,ベ病特有の眼所見により前房蓄膿型(3眼),網膜滲出型(21眼),無特徴型(17眼)に分類した.これらの3型は前房蓄膿や網膜滲出斑以外の症状についてはよく類似しており,他に各型の特徴的な所見はなかった.網膜滲出型ではベ病に定型的な滲出斑が反復して出現する例と,非定型的なものが少数たまに出現する例とがあった.1年間の観察で網膜滲出斑が出現しない例は無特徴型の可能性が高いことが判明した.HLAでは網膜滲出型でA26をもつものが多く,無特徴型ではDQw1をもつ例が多かった.ベ病の炎症が「再発性」と「白血球の過敏性」という特徴をもつことから,再発性の前房蓄膿がある例と網膜滲出斑を反復する例は,他の疾患が否定できればベ病であろうと推定される.滲出斑の出現回数が僅かで,滲出斑の形も非定型的な例はベ病とは断定できず,長期間滲出斑の見られぬ例はベ病でないと推定される.
We evaluated the clinical features, courses and laboratory findings in 24 patients with ocular symp-toms similar to Behçet's disease in the foregoing 5-year period. All the cases had none or one of extraocular involvement. The ocular findings in-cluded anterior uveitis, diffuse vitreous opacity, venous dilatation of the retina and hyperpermeable retinal venules similar to Behçet's disease. They belonged to either of three groups : hypopyon type, exudative type with retinal exudates, and non-specific type. Cases of exudative type were further divided into two subgroups. Cases in one subgroup manifested recurrent retinal exudates that charac-terize Behcet's disease and those in the other sub-group manifested occasional tiny nonspecific exudates. Almost all the cases that failed to mani-fest retinal exudates for one year were assigned to nonspecific type. HLA-A 26 was found in 6 out of 9 cases of exudative type, and HLA-DQw1 in 6 out of 9 cases of nonspecific type.
Behget's disease is characterized by recurrence and hyperreactivity of neutrophils. Cases with recurrent hypopyon or retinal exudates, therefore, were likely to be diagnosed as Behget's disease after other diseases were ruled out. Cases with infrequent noncharacteristic retinal exudates could not be diagnosed as Behget's disease due to lack of conclusive evidences. We ultimately diagnosed 8 cases in the series as Behcet's disease.
Rinsho Ganka (Jpn J Clin Ophthalr6ol) 42(9) : 1065-1068, 1988
Copyright © 1988, Igaku-Shoin Ltd. All rights reserved.