Purtscher-like retinopathy as a presenting ocular manifestation of biopsy-proven giant cell arteritis: A case report

Abstract

Purtscher-like retinopathy is a rare retinal microangiopathy characterised by sudden visual loss, cotton wool spots, retinal whitening and variable retinal haemorrhage in the absence of trauma. Giant cell arteritis is an ophthalmic emergency that usually presents with ischaemic optic neuropathy or retinal arterial occlusion or choroidal ischaemia; however, in very rare cases, it may mimic Purtscher-like retinopathy. We report the case of a 60-year-old man who presented with sudden painless vision loss in the left eye. Fundus examination showed marked optic disc oedema, cotton wool spots, arterial narrowing and multiple discrete white retinal lesions in the posterior pole. Optical coherence tomography demonstrated inner retinal thickening and hyperreflectivity, whereas fluorescein angiography revealed markedly delayed retinal perfusion, capillary nonperfusion, branch retinal arterial occlusion, nasal/inferotemporal choroidal hypoperfusion and optic disc hyperfluorescence. Her erythrocyte sedimentation rate was 52 mm/h, and temporal artery biopsy confirmed giant cell arteritis. The patient received intravenous methylprednisolone followed by oral corticosteroids. Visual acuity did not improve over the first week, although optic disc oedema decreased. This case should not be interpreted as isolated or pure Purtscher-like retinopathy; rather, it represents Purtscher-like retinal findings occurring within a broader giant cell arteritis–related retinal and choroidal ischaemic process. The report broadens the spectrum of ocular presentations of giant cell arteritis and highlights the need to consider occult giant cell arteritis in the differential diagnosis of older patients with sudden painless visual loss and retinal ischaemic signs suggestive of Purtscher-like retinopathy.

Introduction

Purtscher retinopathy is an occlusive retinal microvasculopathy classically associated with trauma, whereas the term Purtscher-like retinopathy is used when a similar fundus appearance occurs in the absence of trauma.^1,2 Reported associations include acute pancreatitis, renal failure, connective tissue disease and other systemic inflammatory or embolic disorders.^1,3 Giant cell arteritis is a vasculitis of medium- and large-sized arteries and a true ophthalmic emergency because delayed treatment may lead to irreversible visual loss.^4,5 Although arteritic anterior ischaemic optic neuropathy and retinal arterial occlusions are the best recognised ocular manifestations of giant cell arteritis (GCA), Purtscher-like retinopathy is distinctly uncommon.^4,6 We describe the case of a patient with unilateral Purtscher-like retinopathy in whom temporal artery biopsy confirmed GCA.

Case presentation

A 60-year-old man with no previous ocular disease, systemic illness or history of trauma presented with sudden, painless vision loss in his left eye. He denied headache, scalp tenderness, jaw claudication, fever, weight loss and other constitutional symptoms. He was not taking regular medication.

Best corrected visual acuity was hand movements in the left eye and 0.8 (decimal Snellen) in the right eye. Intraocular pressure was within normal limits in both eyes. Slit lamp examination of the anterior segment was unremarkable. Fundus examination of the right eye was normal. The left eye showed marked optic disc oedema, arterial narrowing, venous dilatation, cotton wool spots and multiple discrete white retinal lesions in the posterior pole, consistent with Purtscher-like retinopathy. Interrupted arterial blood flow within branches of the central retinal artery raised further concern for an acute ischaemic event (Figure 1).

Figure 1.

Colour fundus photograph of the left eye showing multiple discrete white retinal lesions nasal to the macula, grade 3 optic disc oedema, cotton wool spots, arterial narrowing, venous dilatation and interrupted arterial blood flow. Optic disc oedema was an important accompanying sign of GCA-related ocular ischaemia.

GCA: giant cell arteritis.

Optical coherence tomography of the left eye demonstrated thickening and hyperreflectivity of the inner retinal layers, most pronounced nasally, in keeping with acute retinal ischaemic injury (Figure 2). There were large, confluent, extensive cotton wool spots in the peripapillary superotemporal region along the course of the branch retinal artery occlusion, superonasal to the macula (Figure 2). Additionally, inner retinal thickening and hyperreflectivity were noted in the parafoveal superonasal area along the course of the branch retinal artery occlusion (BRAO) (Figure 3), along with the presence of multiple small cotton wool spots in the posterior pole.

Figure 2.

Optical coherence tomography of the left eye showing thickening and hyperreflectivity of the inner retinal layers, especially nasally.

Figure 3.

Optical coherence tomography of the left eye demonstrating inner retinal thickening and hyperreflectivity in the parafoveal superonasal region, consistent with acute ischaemic change.

Fluorescein angiography showed markedly delayed filling in all phases with delayed choroidal flush and nasal and inferotemporal choroidal hypoperfusion/infarction (Figure 4(a)). The arterial phase demonstrated delayed arterial filling and attenuation of the retinal arterioles, reflecting impaired perfusion and branch retinal arterial occlusion. Hypofluorescent areas were observed in the superotemporal region, corresponding to cotton wool spots (Figure 4(b)). The arteriovenous phase revealed prolonged transit time with areas of capillary nonperfusion, corresponding to retinal ischaemia (Purtscher flecken) (Figure 4(c) and (d)). The venous phase demonstrated delayed venous drainage with inferior branch retinal vein occlusion and sluggish flow (Figure 4(e) and (f)). The recirculation phase showed persistent hypofluorescent ischaemic areas with minimal late leakage, supporting a diagnosis of ischaemic microangiopathy, along with a hyperfluorescent optic disc corresponding to optic disc oedema (Figure 4(g)). In the late phases, staining of the superotemporal macular branch was also observed, indicating occlusion (Figure 4(g)). Notably, although most clinical findings were located superiorly, fluorescein angiography demonstrated more pronounced ischaemia in the inferior retina.

Figure 4.

Fluorescein angiography of the left eye. (a) Choroidal flush at 15 s; (b) early arterial phase at 19 s with absent or markedly delayed choroidal flush and nasal/inferotemporal choroidal hypoperfusion; (c) arterial phase at 31 s; (d) laminar venous flow at 38 s; (e and f) venous phases at 48 and 58 s, reveals delayed filling and areas of capillary nonperfusion; (g) recirculation phase demonstrates staining of the superotemporal macular branch and hot disc.

Laboratory investigations showed an erythrocyte sedimentation rate of 52 mm/h and a C-reactive protein level of 3.1 mg/L. Platelet count was within the normal range. Other causes of optic neuritis were excluded because antinuclear antibody (ANA), cytoplasmic antineutrophil cytoplasmic antibody (cANCA), human immunodeficiency virus (HIV) and syphilis immunoglobulin G/immunoglobulin M (IgG/IgM) were negative. Given the patient's age, the retinal ischaemic findings and the raised erythrocyte sedimentation rate (ESR), GCA was strongly suspected. The patient met the 1990 American College of Rheumatology classification criteria for GCA, and temporal artery biopsy confirmed the diagnosis by demonstrating granulomatous inflammation with multinucleated giant cells, disruption of the internal elastic lamina and luminal narrowing.

Treatment was started immediately with intravenous methylprednisolone (1 g daily) for 3 days, followed by a tapering course of oral prednisone. At early follow-up after 7 days, visual acuity in the left eye remained at hand movements; however, fundus examination showed a clear reduction in optic disc oedema. No new ocular or systemic ischaemic events were documented during admission. The patient was referred for further neurological and rheumatological assessment and long-term management.

Discussion

Purtscher-like retinopathy is a rare, nontraumatic retinal microangiopathy characterised by Purtscher flecken, cotton wool spots, variable retinal haemorrhages and acute visual loss.^1,2 Proposed diagnostic criteria require at least three of the following five features: (a) Purtscher flecken; (b) a low-to-moderate number of retinal haemorrhages; (c) cotton wool spots confined to the posterior pole; (d) a plausible explanatory aetiology and (e) compatible ancillary investigations. ² In the present case, the patient fulfilled these criteria owing to the presence of Purtscher flecken, cotton wool spots in the posterior pole, a credible systemic aetiology and supportive optical coherence tomography (OCT) and fluorescein angiographic findings.

The pathophysiology of Purtscher-like retinopathy is thought to involve precapillary arteriolar occlusion and retinal microinfarction.^1,2 GCA more commonly causes ocular ischaemia through involvement of the posterior ciliary, ophthalmic and retinal arterial circulations, resulting in anterior ischaemic optic neuropathy, retinal arterial occlusion or choroidal ischaemia.^4,5 The present case is therefore notable because the retinal appearance resembled Purtscher-like retinopathy rather than a more typical arteritic optic neuropathy or isolated retinal arterial occlusion. However, the presence of BRAO, deeper retinal ischaemia and nasal/inferotemporal choroidal infarction indicates that this case should not be interpreted as isolated or pure Purtscher-like retinopathy. Instead, the findings are best regarded as a Purtscher-like retinal phenotype occurring within a broader GCA-related ocular ischaemic process.

Cotton wool spots can occur in GCA; however, the complete Purtscher-like constellation is rarely reported.^4,7 One possible explanation is that GCA usually produces clinically dominant ischaemia at the level of the posterior ciliary circulation, optic nerve head, ophthalmic artery or larger retinal arterial branches, whereas cotton wool spots represent focal retinal nerve fibre layer infarcts arising from more distal superficial retinal microvascular compromise. Such lesions may therefore be transient, under-recognised or overshadowed by more severe optic nerve head or choroidal ischaemia. Cotton wool spots and Purtscher-like retinopathy have also been described in other unusual settings, including otorhinolaryngological surgery. ⁸

An important clinical lesson from this case is that GCA may present without the classical cranial or constitutional symptoms. ⁹ Occult GCA is well recognised, and absence of headache, jaw claudication or scalp tenderness should not exclude the diagnosis in an older patient with sudden painless visual loss and retinal, choroidal or optic nerve head ischaemia. ⁹ Likewise, a normal platelet count does not rule out GCA, and inflammatory markers may be variably abnormal.^4,10

Visual recovery in biopsy-proven GCA is often limited once severe ischaemic damage has occurred.^11,12 Nevertheless, rapid institution of high-dose corticosteroid therapy remains essential to reduce the risk of further vision loss in the affected eye, prevent fellow-eye involvement and limit other systemic ischaemic complications.^4,12 In our patient, visual acuity did not improve during the early follow-up period; however, optic disc oedema decreased after treatment, suggesting partial anatomical response despite established functional damage.

To the best of our knowledge, only one other case of Purtscher-like retinopathy associated with giant cell arteritis has been reported in the literature. ⁶ Thus, this case adds to the limited literature and reinforces the importance of considering GCA in the differential diagnosis of Purtscher-like retinopathy in patients older than 50 years, particularly when multimodal imaging points to widespread retinal and choroidal hypoperfusion.

The main limitations of this report are its single-patient design, the limited early follow-up period and absence of follow-up colour fundus photography to document the resolution of cotton wool spots. In addition, the coexistence of Purtscher-like lesions with BRAO and choroidal infarction creates diagnostic overlap with other manifestations of arteritic ocular ischaemia. Therefore, this case cannot establish a causal mechanism between GCA and Purtscher-like retinopathy. Nevertheless, it broadens the recognised clinical spectrum of GCA and supports considering GCA in older patients presenting with Purtscher-like retinal findings, particularly when multimodal imaging shows retinal and choroidal hypoperfusion.

Conclusion

This case expands the spectrum of ocular manifestations of giant cell arteritis by documenting Purtscher-like retinal findings occurring together with optic disc oedema, branch retinal arterial occlusion and choroidal infarction. In older patients, sudden painless visual loss with retinal whitening, cotton wool spots, optic disc oedema and angiographic evidence of severe retinal or choroidal hypoperfusion should prompt urgent evaluation for GCA even when systemic symptoms are absent. Early recognition, multimodal imaging and prompt corticosteroid therapy remain crucial to prevent additional irreversible ocular and systemic ischaemic events.