Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia.
Department of Ophthalmology, Oregon Health & Science University Casey Eye Institute, Portland.
JAMA Ophthalmol. 2020 Aug 1;138(8):894-900. doi: 10.1001/jamaophthalmol.2020.2349.
Recent studies have linked a vision-threatening maculopathy with long-term use of pentosan polysulfate sodium (PPS).
To evaluate the disease course in PPS-associated maculopathy after drug cessation.
DESIGN, SETTING, AND PARTICIPANTS: In this retrospective case series, patients diagnosed with PPS-associated maculopathy with at least 6 months of follow-up after drug cessation who were treated at the Emory Eye Center, Atlanta, Georgia, or the Casey Eye Institute, Portland, Oregon, were included. Data were collected from April 2014 through November 2019.
Change in visual acuity and retinal imaging characteristics over time.
Of the 11 included patients, all were female, and the median (interquartile range [IQR]) age was 53 (44-63) years. Participants had a baseline visit at a median (IQR) of 2 (0-4) months after drug cessation and were subsequently observed for a median (IQR) of 12 (8-26) months. The median (IQR) cumulative PPS exposure was 1.97 (1.55-2.18) kg. No eyes exhibited a demonstrable improvement in disease after discontinuing PPS. A total of 9 of 11 patients (82%) reported worsening visual symptoms at the final visit. The mean (SD) logMAR visual acuity was 0.14 (0.23) and 0.14 (0.34) at the baseline and final visit, respectively. Visual acuity improved by 2 or more Snellen lines in 1 eye (5%) and declined by 2 or more Snellen lines in 2 eyes of 1 patient (9%). There was evolution in the pattern of fundus autofluorescence changes and/or optical coherence tomography findings in all eyes. A total of 17 eyes (77%) exhibited expansion of the area of involved tissue. A total of 7 eyes (32%) had macular retinal pigment epithelium atrophy at the baseline visit, and atrophy enlarged after discontinuation of PPS in all 7 eyes, with a median (IQR) growth rate of 0.32 (0.13-0.38) mm per year.
These retrospective data among 11 patients suggest PPS-associated maculopathy continues to evolve after drug cessation for at least 10 years. In some cases, progressive retinal pigment epithelium atrophy encroaches on the foveal center and thus may pose a long-term threat to central vision.
最近的研究将一种与长期使用戊聚糖多硫酸钠（PPS）有关的致盲性黄斑病变与 PPS 联系起来。
评估 PPS 相关性黄斑病变停药后的疾病进程。
设计、地点和参与者：在这项回顾性病例系列研究中，纳入了至少在停药后 6 个月接受亚特兰大埃默里眼科中心或波特兰凯西眼科研究所治疗的 PPS 相关性黄斑病变患者，并进行了诊断。数据收集时间为 2014 年 4 月至 2019 年 11 月。
视觉敏度和视网膜成像特征随时间的变化。
11 名纳入患者均为女性，中位（四分位距 [IQR]）年龄为 53（44-63）岁。患者在停药后中位（IQR）2（0-4）个月进行基线检查，随后中位（IQR）12（8-26）个月接受观察。中位（IQR）PPS 累计暴露量为 1.97（1.55-2.18）kg。在停止使用 PPS 后，没有眼睛显示疾病有明显改善。11 名患者中有 9 名（82%）在最后一次就诊时报告视力症状恶化。平均（SD）logMAR 视力分别为 0.14（0.23）和 0.14（0.34），基线和最后一次就诊时。1 只眼（5%）视力提高 2 行或以上，1 名患者的 2 只眼（9%）视力下降 2 行或以上。所有眼睛的眼底自发荧光变化和/或光学相干断层扫描结果均发生演变。17 只眼（77%）显示受累组织面积扩大。7 只眼（32%）在基线时有黄斑视网膜色素上皮萎缩，在停止使用 PPS 后，所有 7 只眼的萎缩均有扩大，每年平均（IQR）增长率为 0.32（0.13-0.38）mm。
这是 11 名患者的回顾性数据，表明至少在 10 年内，PPS 相关性黄斑病变在停药后仍在继续发展。在某些情况下，进行性视网膜色素上皮萎缩侵犯中央凹，因此可能对中心视力构成长期威胁。
