増殖性網膜症を合併した全身性強皮症と多発性筋炎のoverlap症候群の1例

A case of overlap syndrome of progressive systemic sclerosis and polymyositis with proliferative retinopathy

　全身性強皮症と多発性筋炎のoverlap症候群に増殖性網膜症を合併した1例を経験した。症例は22歳女性で，両眼の眼底に軟性白斑，網膜出血を広汎に認めた。経口ステロイド療法が開始され，全身状態は改善したにもかかわらず眼底所見は進行し，4か月後に視神経乳頭上新生血管，8か月後に線維血管性増殖組織，硝子体出血，牽引性網膜剥離が生じ，硝子体手術を施行した。全身性強皮症や多発性筋炎に増殖性網膜症を合併することは稀であるが，本症例ではステロイド療法により血管炎が消退するまでの間に不可逆性の網膜毛細血管閉塞を生じたものと推察した。これらの膠原病における早期治療と眼底検査の重要性が痛感された。

A 22-year-old female presented with acute bilateral visual impairment. She had developed fever and sore throat 2 weeks before. Systemic and laboratory examinations led to the diagnosis of progressive systemic sclerosis and polymyositis. Both fundi showed multiple retinal hemorrhages and cottonwool patches. Fluorescein angiography showed diffuse perivascular staining indicating retinal vasculitis. While her systemic conditions improved after systemic corticosteroid, the retinopathy exacerbated and developed bilateral optic disc neovascularization, vitreous hemorrhage and, finally, extensive fibrovascular prolifera-tion with tractional retinal detachment necessitating vitrectomy. It appeared that extensive capillary nonperfusion had devoloped before retinal vasculitis improved by systemic corticosteroid. Early detection and treatment seemed to be essential in these collagen diseases.