Long-Term Follow-Up in a Patient with Purtscher-Like Retinopathy Associated with Systemic Lupus Erythematosus: A Case Report

Dear Editor,

Purtscher retinopathy is a rare retinal microangiopathy associated with trauma, characterized by distinctive retinal findings including Purtscher flecken, cotton-wool spots, retinal hemorrhages, and optic disc swelling [1]. When these signs occur in the absence of trauma, the condition is referred to as Purtscher-like retinopathy (PLR). PLR has been linked to various systemic conditions including acute pancreatitis, renal failure, and autoimmune disorders such as systemic lupus erythematosus (SLE) [1]. The severity of retinal damage in SLE varies from mild microangiopathy to severe retinal vasculitis or retinal vaso-occlusions [2], whereas PLR has been reported less frequently in SLE patients with retinal nonperfusion accompanied in most cases [3]. Herein, we report a case of PLR in a patient with SLE, along with distinct imaging features and long-term follow-up. This study was approved by the Institutional Review Board of Ajou University Hospital (No. AJOUIRB-EX-2025-375), with a waiver of informed consent.

A 31-year-old female patient with a known history of SLE presented with transient visual disturbance in the right eye lasting for about 1.5 hours. The episode resolved spontaneously, and her initial examination revealed normal visual acuity (20 / 20 in both eyes) and unremarkable ocular and imaging findings (Supplementary Fig. 1A–1F). However, two weeks later, she returned with a sudden and persistent visual impairment in the right eye. At this visit, the best-corrected visual acuity (BCVA) had deteriorated to finger counting in the right eye. Fundus examination revealed diffuse retinal whitening with perivascular sparing areas involving the posterior pole, consistent with Purtscher flecken, with few retinal hemorrhages (Fig. 1A). Optical coherence tomography (OCT) demonstrated diffuse cystoid macular edema (ME) with hyperreflective inner retinal layers (Fig. 1B). Fluorescein angiography (FA) revealed no vascular nonperfusion (Fig. 1C), but mild leakage at the optic nerve head (Fig. 1D). The patient did not report any other precipitating factors, including trauma. Based on these findings, the patient was diagnosed with unilateral PLR associated with SLE.

Fig. 1.

Imaging features of the right eye presenting Purtscher-like retinopathy. (A) Diffuse retinal whitening with perivascular sparing that involved the posterior pole including the fovea, consistent with Purtscher flecken, and few retinal hemorrhages at superonasal quadrant. (B) Diffuse macular edema with hyperreflective retinal inner layer was noted in optical coherence tomography (OCT). (C) Fluorescein angiography revealed no signs of vascular nonperfusion. (D) Discrete leakage at optic nerve head was noted at late phase. (E) One week after starting systemic and local corticosteroids therapy, retinal whitening was much improved. (F) OCT showed improved macular edema, but hyperreflectivity remained at inner layers (arrows). (G) Normalization of fundus findings was noted over a 5-year follow-up. (H) Partially thinned retina remained in the temporal side of the macula at the final follow-up OCT.

The results of the blood tests performed at this visit were not significantly different from those obtained at the most recent outpatient visit: erythrocyte sedimentation rate, 48 mm/hr; C-reactive protein, 0.47 mg/dL; complement-3, 116 mg/dL; complement-4, 19 mg/dL; and anti–double-stranded DNA, <3 IU/mL. Given the absence of standardized treatment for PLR, the patient received both local (posterior sub-Tenon triamcinolone injection) and systemic corticosteroid therapy (starting at 50 mg/day for one week, followed by a gradual taper over three consecutive months). After one week, her BCVA improved slightly to 20 / 400, accompanied by a marked reduction in retinal whitening (Fig. 1E) and ME (Fig. 1F). Her vision continued to recover over time, reaching 20 / 66 after five years, without recurrence of retinal lesions (Fig. 1G). OCT at final follow-up revealed partial thinning of the temporal macula (Fig. 1H). Notably, her systemic SLE therapy was unchanged throughout this period (hydroxychloroquine, 200 mg; methylprednisolone, 4 mg; pilocarpine hydrochloride, 1.25 mg; and an anti-inflammatory herbal tablet), suggesting that the ocular episode was an isolated manifestation of disease activity.

Ocular involvement in SLE occurs in about 3% of well-controlled cases but can affect up to one-third of patients with poorly controlled disease [2]. Lupus retinopathy, one of the common ocular presentations of SLE, typically presents with cotton-wool spots and retinal hemorrhage and may manifest as retinal vascular occlusion in severe cases [4], whereas PLR is relatively rare [3]. SLE has been reported to account for approximately 3% to 13% of the underlying causes of PLR, with an estimated 50% rate of bilaterality [1,5]. The diagnosis of PLR relies on clinical presentation: fulfilling three of the following five criteria was suggested for the diagnosis of Purtscher retinopathy and PLR: (1) Purtscher flecken, (2) retinal hemorrhages, (3) cotton-wool spots typically in the posterior pole, (4) probable or plausible explanatory etiology, and (5) complementary investigation compatible with the diagnosis [1,5]. Accordingly, the patient was diagnosed with PLR, fulfilling the following criteria: the presence of Purtscher flecken and retinal hemorrhages, and an explanatory etiology (SLE).

Among these criteria, Purtscher flecken are considered to be pathognomonic, assumed to occur as the result of precapillary arterioles occlusion [5]. Previously reported SLE-associated PLR cases often showed extensive vascular nonperfusion on FA, including capillary dropout, peripapillary staining, and delayed vascular filling [3,5]. In contrast, the present case demonstrated Purtscher flecken without any vascular nonperfusion on FA. Along with hyperreflective bands in the inner nuclear layers on OCT correlating to Purtscher flecken, PLR in this case with SLE is presumed to originate from microvascular occlusions which may be too subtle to be visualized on FA.

A standardized treatment has not been established; nonetheless, conservative observation is commonly adopted, followed by the use of corticosteroids [1,5]. ME was improved following systemic and local corticosteroids, while the recovery of the BCVA was modest. As treatment of PLR depends largely on the underlying systemic disease, establishing a specific treatment guideline for PLR remains challenging.

In summary, this case highlights a rare manifestation of unilateral PLR in a SLE patient, characterized by Purtscher flecken and ME without angiographic evidence of nonperfusion and suggests that microangiopathic changes can manifest as Purtscher flecken and hyperreflective inner retinal bands on OCT.

Supplementary Materials

Supplementary materials are available from https://doi.org/10.3341/kjo.2025.0173.