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以急性視力受損為全身紅斑性狼瘡的初發表現-病例報告

Systemic Lupus Erythematosus Presenting by Sudden Vision Loss-A Case Report

摘要


目的:報告一位十四歲女孩,因阻塞性血管炎及視網膜動脈阻塞,以雙眼急性視力受損為全身紅斑性狼瘡的初發表現,及其後續的臨床變化。 方法:單一病例報告。 結果:這位十四歲女孩因突發性雙眼視力劇減,至急診求診。當時最佳矯正視力右眼20/1000,左眼20/500;眼底檢查發現雙眼除了視網膜動脈阻塞所致黃斑部缺血水腫,還併有廣泛阻塞性視網膜血管炎(occlusive retinal vasculitis)。患者臉頰有蝴蝶斑,手腳有圓盤狀紅斑,關節炎,貧血及血小板缺乏,抗雙縷DNA抗體(Anti-dsDNA Ab)及抗細胞核抗體(ANA)呈陽性反應,符合全身紅斑性狠瘡診斷。雖積極的以類固醇、免疫抑制劑、抗瘧疾藥、抗凝血劑、及視網膜雷射治療;但右眼仍併發生增殖性視網膜病變合併牽引性視網膜剝離,幸好左眼經過治療追蹤兩年半,視力仍維持在20/25至20/20之間。 結論:紅斑性狼瘡因嚴重視網膜阻塞性血管炎而以急性視力受損為初發表現,在文獻上甚少發表。急性期可以類固醇、免疫抑制劑、抗瘧疾藥、抗凝血劑輔以血漿透析治療;之後還須積極的以全視網膜雷射光凝因手術,以預防增殖性視網膜病變的發生。

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並列摘要


Purpose: To report the clinical course of systemic lupus erythematosus which was first presented by sudden vision loss due to occlusive retinal vasculitis and retinal artery occlusion on a 14 year-old girl. Methods: A case report. Result: This 14 year-old girl visited our emergency room for sudden bilateral vision loss. Her best-corrected visual acuity was OD:20/1000, OS: 20/500 at the initial visit. Occlusive retinal vasculitis (OU) with retinal infarction (OD>OS) was the initial impression. Butter-fly malar rash, discoid skin lesions over her hands and feet, arthritis, and anemia with thrombocytopenia were noted. Anti ds-DNA Ab(+) and ANA (+) were revealed later. Systemic lupus erythematosus induced retinal vasculitis (OU) with central retinal artery occlusion (OD) was the diagnosis. After aggressive systemic steroid pulse therapy, immunosuppressive agents, anti-malaria drugs with anti-coagulation therapy following retinal laser photocoagulation (OU), proliferative retinopathy with tractional retinal detachment was still present in her right eye. However, left eye retained visual acuity of 20/2520/20 after two and a half follow-up years. Conclusion: Systemic lupus erythematous rarely presents by sudden vision loss. Treatment of severe occlusive retinal vasculitis by systemic lupus erythematosus includes steroid pulse therapy, immunosuppressive agents, anti-coagulation, anti-malaria drugs, and plasmapheresis in the acute stage. Aggressive pan-retinal photocoagulation may prevent and treat the later proliferative retinopathy.

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