国际眼科杂志2026，Vol.26Issue(5)：805-810,6.DOI:10.3980/j.issn.1672-5123.2026.5.13
原发性色素播散综合征的基础与临床研究进展
Advances in basic and clinical research on primary pigment dispersion syndrome
摘要
Abstract
·Primary pigment dispersion syndrome(PDS)is characterized by pigment granules released from the pigment epithelium of iris,and their deposition in anterior segment of eye,particularly in the trabecular meshwork(TM).This condition may lead to elevated intraocular pressure(IOP),which further causes optic nerve damage,and progresses to pigmentary glaucoma(PG).PDS predominantly affects young myopic individuals and shows heterogeneous clinical manifestations.It may occur sporadically or in familial inheritance.It has been reported that the pathogenic genes include those involved in melanogenesis and melanosome homeostasis(e.g.,PMEL,MC1R,SLC45A2,and TYR),and genes related to ocular development and anterior segment formation(e.g.,CPAMD8,GSAP,and GRM5).The pathogenesis of PDS is closely associated with posterior iris concavity and reverse pupillary block,which may change the dynamics of aqueous humor,along with persistent liberation of pigmented granules by friction of iris and zonules.Pigmented granules accumulating in the TM may cause mechanical obstruction of TM,and increase the phagocytic burden of the cells,and result in cellular dysfunction and structural damage of TM.All these changes contribute to sustained elevation of IOP and optic neuropathy.Primary management of PDS involves regular follow-up and assessment of the risk of IOP elevation.Laser peripheral iridotomy may be considered to reduce posterior iris concavity in PDS.Treatment of PG focuses on IOP control for long term,which include topical medications,laser trabeculoplasty,and filtering or drainage surgery.Development of experimental animal models harboring human pathogenic genes and exhibiting PDS phenotypes is needed to facilitate in-depth research into the pathogenesis of PDS.关键词
色素播散综合征/色素性青光眼/临床表现/发病机制/治疗Key words
pigment dispersion syndrome/pigmentary glaucoma/clinical manifestations/pathogenesis/treatment引用本文复制引用
张韵,周健..原发性色素播散综合征的基础与临床研究进展[J].国际眼科杂志,2026,26(5):805-810,6.基金项目
陕西省重点研发计划项目(No.2024SF2-GJHX-39) （No.2024SF2-GJHX-39）
空军军医大学临床研究项目(No.2024LC2414) Key Research and Development Program of Shaanxi Province(No.2024SF2-GJHX-39) （No.2024LC2414）
Clinical Research Project of Air Force Medical University(No.2024LC2414) （No.2024LC2414）
