Tran Melanie, Agnihotri Akshay Prashant, Nagel Ines D, Wagner Naomi, Nudleman Eric, Borooah Shyamanga
Jacobs Retina Center, 9415 Campus Point Drive, La Jolla, California, United States of America, 92037.
Viterbi Family Department of Ophthalmology and Shiley Eye Institute, University of California San Diego, 9415 Campus Point Drive, La Jolla, California, United States of America, 92037.
Retin Cases Brief Rep. 2025 Jul 18. doi: 10.1097/ICB.0000000000001791.
To report a case of pentosan polysulfate sodium (PPS) maculopathy showing rapid progression of retinopathy 4 years after discontinuing PPS.
A 75-year-old woman presenting with gradual vision changes underwent comprehensive examination including multimodal imaging and genetic testing.
A 75-year-old woman presented with a history of intermediate age-related macular degeneration and progressive blurring of her central vision beginning at 69 years of age. Fundoscopic examination revealed retinal pigment epithelium (RPE) abnormalities and multifocal outer retinal loss. The patient had been first prescribed PPS for interstitial cystitis (IC) aged 63 with a cumulative PPS dose of approximately 1300 grams and ceased PPS at 71 years of age. She was noted to have only minimal retinal changes prior to drug cessation. Four years post-cessation, the patient reported marked night vision and color vision abnormalities and was noted to have severe retinal changes including macular atrophy. Genetic testing revealed the patient to be homozygous for the previously reported risk alleles.
PPS maculopathy is an acquired toxic disease that can significantly impact a patient's quality of life. Our report highlights that marked progression of PPS retinopathy can occur even after drug cessation and that patients need to be carefully monitored and given supportive management.
报告一例戊聚糖多硫酸钠（PPS）黄斑病变病例，该病例在停用PPS 4年后视网膜病变迅速进展。
一名出现渐进性视力变化的75岁女性接受了包括多模态成像和基因检测在内的全面检查。
一名75岁女性，有中度年龄相关性黄斑变性病史，69岁开始出现中心视力逐渐模糊。眼底检查发现视网膜色素上皮（RPE）异常和多灶性外层视网膜缺失。该患者63岁时因间质性膀胱炎（IC）首次服用PPS，累积PPS剂量约为1300克，71岁时停用PPS。停药前仅发现轻微的视网膜变化。停药四年后，患者报告有明显的夜视力和色觉异常，且发现有严重的视网膜变化，包括黄斑萎缩。基因检测显示该患者为先前报道的风险等位基因纯合子。
PPS黄斑病变是一种获得性中毒性疾病，可显著影响患者的生活质量。我们的报告强调，即使在停药后，PPS视网膜病变仍可能显著进展，患者需要仔细监测并给予支持性治疗。
