Bilateral drusenoid deposits with subretinal fluid and cystoid macular edema in a serum anti-phospholipase A2 receptor antibody positive patient: A case report.

Abstract

Introduction: 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.

Objective: To report a case of unique fundus manifestations in a serum anti-phospholipase A2 receptor (anti-PLA2R) antibody positive patient.

Design: Interventional case report.

Participant: 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.

Important clinical findings: 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.

Main diagnoses: Bilateral drusenoid deposits and subretinal fluid (SRF) and cystoid macular edema (CME).

Intervention: 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.

Results: 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.

Conclusions: 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.