Flester Elena, Yassin Shaden H, Borooah Shyamanga
School of Medicine, University of California, San Diego, La Jolla, CA, USA.
Shiley Eye Institute, University of California, San Diego, La Jolla, CA, USA.
Am J Ophthalmol Case Rep. 2024 Nov 22;36:102224. doi: 10.1016/j.ajoc.2024.102224. eCollection 2024 Dec.
To report a case of pentosan polysulfate sodium (PPS) maculopathy with cystoid macular edema (CME) with relatively low cumulative PPS exposure and a history of concurrent hydroxychloroquine use.
A 46-year-old female was treated with PPS daily for 10 years, and hydroxychloroquine intermittently over a span of five years, actively taking hydroxychloroquine for a sum of three years during PPS therapy. Despite a low risk for toxicity based on the cumulative exposure to either medication, fundoscopic examination and multimodal imaging revealed severe PPS maculopathy with CME two and a half years post-PPS cessation. CME was refractory to topical therapy and intravitreal anti-VEGF but improved with intravitreal dexamethasone. Bilateral improved visibility of the ellipsoid zone (EZ) was observed on Spectral Domain Optical Coherence Tomography (SD-OCT) following dexamethasone injection.
Many reports describe an association between cumulative PPS exposure and maculopathy; however, risk factors that may contribute to PPS-associated maculopathy in the setting of low PPS exposure are not well characterized. This case indicates that other risk factors implicated in retinal pigment epithelium dysfunction should be investigated, including concurrent hydroxychloroquine use.
报告一例戊聚糖多硫酸钠（PPS）黄斑病变伴黄斑囊样水肿（CME）的病例，该患者PPS累积暴露量相对较低且有同时使用羟氯喹的病史。
一名46岁女性每天接受PPS治疗10年，在5年期间间歇性使用羟氯喹，在PPS治疗期间共积极服用羟氯喹3年。尽管基于两种药物的累积暴露量毒性风险较低，但眼底检查和多模态成像显示，在停止PPS治疗两年半后出现了严重的PPS黄斑病变伴CME。CME对局部治疗和玻璃体内抗VEGF治疗无效，但玻璃体内注射地塞米松后有所改善。地塞米松注射后，光谱域光学相干断层扫描（SD-OCT）观察到双侧椭圆体带（EZ）的可见性有所改善。
许多报告描述了PPS累积暴露与黄斑病变之间的关联；然而，在PPS暴露量较低的情况下，可能导致PPS相关黄斑病变的危险因素尚未得到充分描述。该病例表明，应研究与视网膜色素上皮功能障碍有关的其他危险因素，包括同时使用羟氯喹。
