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眼窩原因不明硬化性炎症-病例報告

Idiopathic Sclerosing Inflammation of the Orbit-Report of Two Cases

摘要


本文提出兩例眼窩原因不明硬化性炎症(idiopathic sclerosing inflammation of the orbit)病例報告。眼窩原因不明硬化性炎症與其他急性或亞急性非特異性眼窩炎症比較,在臨床表現與組織學變化上特性不同,對治療的反應亦不一致,是以近來學者多將其視為一獨立之疾病。臨床表現以疼痛,凸眼,眼球運動受限,視力喪失為主。組織病理特徵為早發且廣泛分布的纖維化。目前雖仍不明瞭其病因,但一般認為乃自體免疫機制引發眼窩組織的慢性但持續進行之纖維化,與身體其他部位的原發性纖維化疾病相近。因早期診斷不易,預後往往不佳。積極的免疫抑制療法可提昇治療的成功率。

關鍵字

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並列摘要


Two cases of idiopathic sclerosing inflammation of the orbit, which has been regarded as a unique clinicopathologic entity characterized by insidious, progressive sclerosing inflammation and cicatricial entrapment of the orbit, are presented. Clinically the patients showed eye pain, proptosis, limited eye movement, and visual loss of one eye. Desmoplasia with sparse infiltrate of lymphocytes, histiocytes, and plasma cells were observed histologically. Responses to corticosteroid and immunosuppressive therapy were variable, one patients lost vision at last while the other one still maintained good visual acuity. Early and aggressive immunosuppressive therapy for this immune-mediated disease is recommended.

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