Clinical features of retinal amyloid angiopathy with transthyretin Gly83Arg variant  

作　　者：Gang Su Xing-Wang Chen Jun-Lin Pan Hong Li Bing Xie Shan-Jun Cai 

机构地区：[1]Department of Ophthalmology,Affiliated Hospital of Zunyi Medical University,Zunyi 563000,Guizhou Province,China [2]Guizhou Eye Hospital,Zunyi 563000,Guizhou Province,China [3]Guizhou Provincial Branch of National Eye Disease Clinical Research Center,Zunyi 563000,Guizhou Province,China [4]Special Key Laboratory of Ocular Diseases of Guizhou Province,Zunyi 563000,Guizhou Province,China

出　　处：《International Journal of Ophthalmology(English edition)》2023年第1期128-134,共7页国际眼科杂志（英文版）

基　　金：Supported by the National Natural Science Foundation of China(No.31871261);the Guizhou Science and Technology Cooperation Foundation[No.ZK(2021)general 423];the Research Initiation Fund for Masters in Affiliated Hospital of Zunyi Medical University(No.2016-43)。

摘　　要：AIM:To report on the clinical features of patients with retinal amyloid angiopathy(RAA)who were identified to be caused by the transthyretin(TTR)Gly83Arg variant.METHODS:Case series of five patients diagnosed with RAA was collected at Affiliated Hospital of Zunyi Medical University from January 2010 to December 2021.The clinical features,therapeutic strategies,and prognoses of all patients were reviewed.RESULTS:Five patients with a mean age of 52.00±7.23y were diagnosed as RAA.These patients were previously diagnosed with hereditary transthyretin amyloidosis caused by the TTR Gly83Arg variant.Vitreous opacity was found in all 10 eyes,and 7 eyes developed RAA 2 to 20y after the onset of hereditary transthyretin amyloidosis.The clinical manifestations were recurrent vitreous hemorrhage in 2 eyes(29%),neovascular glaucoma in 2 eyes(29%),and iris neovascularization in 1 eye(14%).Microangioma lesions were found in all affected eyes that underwent fundus fluorescein angiography(FFA)in this group of cases,and the incidence of the retinal non-perfusion area was 67%.Although no cases of retinal neovascularization were found,the prognosis of visual acuity was not ideal.CONCLUSION:This is the first report of RAA in patients with the TTR Gly83Arg variant.Complications such as RAA and glaucoma will seriously affect the visual prognosis of patients.Thereafter,regular ophthalmic follow-up of patients with hereditary transthyretin amyloidosis is essential.And FFA after vitrectomy is very important,which can help ophthalmologists detect RAA earlier and treat it in time.

关 键 词：retinal amyloid angiopathy hereditary transthyretin amyloidosis fundus fluorescein angiography VITRECTOMY retinal photocoagulation 

分 类 号：R77[医药卫生—眼科]