Stammen J, Althaus C, Sundmacher R
Augenklinik, Heinrich-Heine-Universität Düsseldorf.
Klin Monbl Augenheilkd. 1997 Nov;211(5):306-11. doi: 10.1055/s-2008-1035140.
Mooren's ulcer is a rare autoimmunologic disease of the cornea. Many forms of medical and surgical treatments have been proposed in the past, but none of them was regularly successful. Severe progressive cases of Mooren's ulcer are therefore still a blinding disease. Only the use of systemic cyclosporin A (CSA) treatment appears for the first time to have a significant positive effect on the outcome.
One male (30 years old) and three female (52 y, 68 y, 84 y) patients with severe progressive bilateral Mooren's ulcer were treated with cyclosporin A systemically.
On the male patient constant blood levels of CSA were not achievable and he became blind suffering basically from a severe proliferative diabetic retinopathy. The 52-year-old female patient got a relapse of Mooren's ulcer in the graft after penetrating keratoplasty à chaud. With increased blood levels of CSA the progression of the relapsing of Mooren's ulcer could be stopped. Also only with high-dose CSA further progression of the rapidly progressive colliquation of both corneas of a 68-year-old female could be stopped. A definitive improvement with some residual activity could be achieved in a 84-year-old female after only three months of follow-up.
High-dose systemic CSA treatment with plasma trough levels of 150-200 ng/ml is recommended as initial treatment of choice and should be started immediately. For what period of time after clinical healing this high-dose therapy must be contained without the risk of a relapse of Mooren's ulcer remains to be seen.
蚕蚀性角膜溃疡是一种罕见的角膜自身免疫性疾病。过去曾提出多种药物和手术治疗方法,但均未取得常规成功。因此,严重进展性蚕蚀性角膜溃疡病例仍然是致盲性疾病。仅全身使用环孢素A(CSA)治疗首次似乎对治疗结果有显著的积极影响。
1名男性(30岁)和3名女性(52岁、68岁、84岁)患有严重进展性双侧蚕蚀性角膜溃疡的患者接受了全身环孢素A治疗。
男性患者无法维持恒定的CSA血药浓度,他因严重的增殖性糖尿病视网膜病变而失明。52岁女性患者在热灼式穿透性角膜移植术后移植眼出现蚕蚀性角膜溃疡复发。随着CSA血药浓度升高,蚕蚀性角膜溃疡复发的进展得以停止。同样,只有使用高剂量CSA才能阻止一名68岁女性双眼快速进行性溶解的进一步进展。一名84岁女性在仅随访3个月后就取得了有一些残余活动的明确改善。
建议将血浆谷浓度为150 - 200 ng/ml的高剂量全身CSA治疗作为首选初始治疗方法,并应立即开始。在临床愈合后,这种高剂量治疗必须持续多长时间而无蚕蚀性角膜溃疡复发风险仍有待观察。