Wu Hailiang, Liu Lanjing, Yan Li, Zhao Na, Yan Wei
Department of Ophthalmology, Capital Medical University Electric Teaching Hospital (State Grid Corporation of China Beijing Electric Power Hospital), No. 1, Xili, Taipingqiao, Fengtai District, Beijing, 100073, China.
Department of Ophthalmology, Beijing Daxing District People's Hospital (Capital Medical University Daxing Teaching Hospital), No. 26, Huangcun West Street, Daxing District, Beijing, China.
Heliyon. 2025 Feb 18;11(4):e42764. doi: 10.1016/j.heliyon.2025.e42764. eCollection 2025 Feb 28.
Phospholipase A2 receptor (PLA2R) is a major target antigen in idiopathic membranous nephropathy (IMN). IMN was often reported to have retinal vascular abnormalities secondary to hypercoagculability or hypertension. We describe a case of a serum anti-PLA2R antibody positive patient with bilateral drusenoid deposits and subretinal fluid (SRF) and cystoid macular edema (CME) to stress the necessity of fundus examination in anti-PLA2R antibody positive or IMN patients.
To report a case of unique fundus manifestations in a serum anti-phospholipase A2 receptor (anti-PLA2R) antibody positive patient.
Interventional case report.
A patient diagnosed with nephrotic syndrome who was positive for serum anti-PLA2R antibody.
MAIN SYMPTOMS AND/OR IMPORTANT CLINICAL FINDINGS: Main symptoms: bilateral blurred vision for half a year.
There were numerous large drusenoid lesions throughout the posterior pole of the patient's fundus symmetrically. These lesions were more obvious on autofluorescence (AF) and near-infrared reflectance imaging (NIR) with a "starry-sky' pattern. They presented hyper-reflective deposits beneath the retinal pigment epithelium on optical coherence tomography (OCT). The lesions showed hypofluorescent through all phases on FA and ICGA. Several pinpoint areas of hyperfluorescence gradually expanded in an "inkblot leak" fashion bilaterally on FA.
Bilateral drusenoid deposits and subretinal fluid (SRF) and cystoid macular edema (CME).
The patient was examined with ultrawide-field imaging, autofluorescence (AF), near-infrared reflectance imaging (NIR), optical coherence tomography (OCT), optical coherence tomography angiography (OCTA), fluorescein angiography (FA) and indocyanine green angiography (ICGA). The patient received medical treatment including ultrafiltration, anticoagulation and intravenous Obinutuzumab.
5 days after medical treatment, the patient perceived improvement in vision with the best corrected visual acuity (BCVA) improved from 4/20 OD and 5/20 OS to 8/20 OU. Follow-up OCT showed absorption of CME and SRF, while the drusenoid lesions persisted.
This is the first report of bilateral drusenoid deposits and subretinal fluid (SRF) and cystoid macular edema (CME) in a serum anti-PLA2R antibody positive patient and probably the first report in an idiopathic membranous nephropathy (IMN) patient. The importance of ophthalmological assessment in identifying ocular complications in serum anti-PLA2R antibody positive or IMN patients should be emphasized.
磷脂酶A2受体(PLA2R)是特发性膜性肾病(IMN)的主要靶抗原。IMN常被报道继发于高凝状态或高血压而出现视网膜血管异常。我们描述了1例血清抗PLA2R抗体阳性患者出现双侧玻璃膜疣样沉积物、视网膜下液(SRF)和黄斑囊样水肿(CME)的病例,以强调抗PLA2R抗体阳性或IMN患者进行眼底检查的必要性。
报告1例血清抗磷脂酶A2受体(抗PLA2R)抗体阳性患者独特的眼底表现。
介入性病例报告。
1例诊断为肾病综合征且血清抗PLA2R抗体阳性的患者。
主要症状和/或重要临床发现:主要症状:双眼视物模糊半年。
患者眼底后极部对称分布有大量大的玻璃膜疣样病变。这些病变在自发荧光(AF)和近红外反射成像(NIR)上更明显,呈“星空”样图案。在光学相干断层扫描(OCT)上,它们表现为视网膜色素上皮下的高反射沉积物。病变在荧光素血管造影(FA)和吲哚菁绿血管造影(ICGA)的各期均表现为低荧光。FA上双侧有几个点状高荧光区以“墨迹渗漏”方式逐渐扩大。
双侧玻璃膜疣样沉积物、视网膜下液(SRF)和黄斑囊样水肿(CME)。
对患者进行超广角成像、自发荧光(AF)、近红外反射成像(NIR)、光学相干断层扫描(OCT)光学相干断层扫描血管造影(OCTA)、荧光素血管造影(FA)和吲哚菁绿血管造影(ICGA)检查。患者接受了包括超滤、抗凝和静脉注射奥妥珠单抗在内的药物治疗。
药物治疗5天后,患者自觉视力改善,最佳矫正视力(BCVA)从右眼4/20和左眼5/20提高到双眼8/20。随访OCT显示CME和SRF吸收,但玻璃膜疣样病变持续存在。
这是血清抗PLA2R抗体阳性患者双侧玻璃膜疣样沉积物、视网膜下液(SRF)和黄斑囊样水肿(CME)的首例报告,可能也是特发性膜性肾病(IMN)患者的首例报告。应强调眼科评估在识别血清抗PLA2R抗体阳性或IMN患者眼部并发症中的重要性。