Simultaneous bilateral purtscher like retinopathy with systemic lupus erythematosus: a case

Abstract

Backgroud

Systemic lupus erythematosus is an unexplained autoimmune disease involving multiple systems throughout the body, and its ocular changes include dry eye, monocular or binocular visual field defects, vaso-occlusive diseases, or ischemic optic neuropathy.

Case presentation

This article reports a patient with SLE complicated with bilateral Purtscher like retinopathy, who had a sudden decrease in ocular vision as the first symptom, the autoantibodies related to phospholipid syndrome showed no abnormality, and both anti-dsDNA antibodies and anti-SM antibodies were significantly positive, indicating that anti-dsDNA antibodies and anti-SM antibodies were also important factors in the pathogenesis of Purtscher like retinopathy.

Conclusion

The close relationship between SLE retinopathy and systemic inflammatory activities and emphasize the importance of systemic immunotherapy.

Background

Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple systems of the body. The course of the disease is chronic, and the clinical manifestations are diverse. It mainly manifests as multiple organ damage mediated by various autoantibodies and immune complexes represented by antinuclear antibodies in blood circulation. It mainly occurs in young and middle-aged women. Approximately 20–150 people in every 100,000 people are affected [1]. Its clinical pathogenesis is due to a variety of factors, including genetic susceptibility, environmental stimulation, and immune and hormonal disorders [1].

It has been reported that ocular changes in SLE patients may occur at any stage of the disease. Among them, dry keratoconjunctivitis is the most common ocular complication, while the incidence of retinal vascular diseases is 0.66%. Retinal vascular diseases often lead to vision loss that cannot be effectively improved in more than one-third of patients. Mild cases are characterized by cotton plaques or retinal haemorrhage caused by anterior capillary artery occlusion, and severe cases are characterized by severe fundus lesions caused by retinal vascular occlusion, which is related to the active degree of SLE [2]. However, retinal artery occlusion is rare. Here, we report a case of SLE complicated with bilateral Purtscher like retinopathy, which is characterized by acute, severe and binocular vision loss as the patient’s initial presentation.

Case presentation

A 25-year-old female complained of sudden visual acuity loss in both eyes for 3 days without obvious cause and was admitted to West China Hospital, Sichuan University on November 16, 2022. She underwent caesarean section in 2020. She denied a history of allergy or trauma and any other systemic disease.

The best corrected visual acuity (BCVA) of both eyes was CF/40 cm. The intraocular pressure was 10.00 mmHg OD and 9.4 mmHg OS. The slit-lamp examinations were normal with transparent cornea, keratic precipitates (-), normal anterior chamber depth, aqueous flare (-), dilated pupil, approximately 6 mm in diameter, delayed light reflex, transparent lens, visible macular oedema of the fundus, and optic nerve border blurring. Optical coherence tomography (OCT) showed diffuse hyperreflectivity and thickening of the inner retina with cystoid macular oedema (Fig. 1). Wide-field fundus photography showed oedema and pallor of the posterior pole of the retina in both eyes, retinal artery narrowing, and scattered dots and patches of haemorrhage in the posterior pole (Fig. 2). Fundus fluorescein angiography (FFA) showed artery fluorescein with delayed arterial phase at 14s, delayed laminar phase at 18s, large non perfused area in macular and periphery and scattered blockage. There was obvious diffuse capilary leakage in the late stage and macular oedema (Fig. 3). From the examinations, she was diagnosed with bilateral Purtscher like retinopathy and was treated with a hyperbaric oxygen chamber and retrobulbar injection of 40 mg triamcinolone acetonide.

Fig. 1

Spectral domain-Optical Coherence tomography of both eyes before and after treatment (A1-right eye before treatment, A2-left eye before treatment, B1-right eye after treatment, B2-left eye after treatment), showed a change in macular morphology before and after treatment

Fig. 2

Wide field fundus photo of both eyes before and after treatment (A1-right eye before treatment, A2-left eye before treatment, B1-right eye after treatment, B2-left eye after treatment), showed changes in the retina before and after treatment

On November 19, 2022, the patient’s laboratory test results showed that the percentage of eosinophils was 0.0%, the absolute value of eosinophils was 0.00 × 10⁹/L, the white blood cell count was 3.82 × 10⁹/L, and the platelet count was 131 × 10⁹/L. Antinuclear antibody was + 1:320 of particle type homogeneous cytoplasmic particle type, anti-double-stranded DNA antibody was 84.30 IU/ml, anti-IU-NNP antibody/Sm antibody +++, anti-Sm antibody +++, anti-ribosomal P protein antibody +, antinuclear body antibody suspicious (±), and anti-SS-A antibody suspicious (±). She was transferred to the rheumatology and immunology department, and further physical examination found that most erythema appeared on the flexion side of both hands, some of which had no fade, no pain, and ulceration. There were no abnormal autoantibodies associated with phospholipid syndrome. She was diagnosed with SLE disease. Methylprednisolone 500 mg qd was administered for pulse therapy.

On November 28, 2022, the patient underwent a follow-up ophthalmic examination. The BCVA was CF/100 cm OU. OCT showed that cystoid macular oedema was attenuated in both eyes, the inner retina had diffuse hyperreflectivity, the full layer of the retina became thinner, and the outer segment structure was disorganized and damaged (Fig. 1). Wide-field fundus photography showed segmental occlusion of the retinal artery, there are large cotton wool spots compatible to ischemia or Purtscher flecken at the posterior pole, and increased linear and patchy superficial retinal haemorrhage compared to before treatment (Fig. 2). The patient had received retrobulbar injection of triamcinolone acetonide and a high dose of methylprednisolone therapy. However, visual acuity was not significantly improved, and no new positive signs occurred. Therefore, the patient was switched to continued treatment with oral methylprednisolone 80 mg qd and was followed up intensely in the Department of Rheumatology and Immunology.

Fig. 3

Fundus fluorescein angiography of both eyes before treatment in later binocular (A-right eye, B-left eye), showed the macular area and the temporal artery are not filled, showing a large area of avascular area, scattered hemorrhage, obvious leakage of vascular wall in the later stage, and macular edema

Discussion and conclusion

SLE is an autoimmune disease with unknown causes that often occurs in young women. It can cause various inflammatory and immune reactions [3]. Autoantigens combine with antibodies to form immune complexes, which are deposited on vascular endothelial cells, leading to the destruction of the vascular intima and subsequent thrombosis [4]. Its clinical manifestations are diverse, and it can affect multiple organs of the whole body, including the skin, mucosa, eyes, joints, kidneys, blood system, central nervous system, and digestive system. The ocular lesions in SLE patients include dry eyes, unilateral or bilateral defects of the visual field, vascular obstructive disease, and ischaemic optic neuropathy. The pathogenesis of ocular symptoms in SLE patients remains unclear. Microvascular lesions and small vessel infarction caused by the deposition of immune complexes (such as IgG) and the production of inflammatory mediators may be related to this [5]. Among them, retinopathy can manifest as internal bleeding of small vessels, cotton wool spots, arteriolar stenosis of the fundus, capillary plexus and vein dilation, and changes in vascular curvature, which are usually caused by the destruction of the immune complex and vascular injury to activate complement, leading to vascular abnormalities and vascular occlusion [6]. Studies have found that SLE patients with elevated levels of serum antiphospholipid antibodies (APLA) have a higher tendency for vascular occlusion, as thrombosis during SLE is closely related to APLA [7]. In a previous case of SLE occlusivevasculitis involving both eyes, antiphospholipid antibodies were positive [8]. The author believed that its pathogenesis is related to thrombosis associated with antiphospholipid syndrome. However, in our case, phospholipid syndrome-related autoantibodies did not show abnormalities, and both anti-dsDNA and anti-SM antibodies were significantly positive. It was suggested that APLA is not the only pathogenic factor of thrombosis in SLE. A recent study revealed an important role of anti-dsDNA antibodies in promoting thrombosis through platelet dysfunction [9]. The effects of anti-dsDNA antibodies on platelets were shown by direct activation and structural changes via FcγRIIA receptors [9]. Activated platelets promote thrombus formation and impair the contraction of living blood clots, which is responsible for the high incidence of arterial and venous thrombosis in SLE patients [10]. The pathogenesis of SLE complicated with Purtscher like retinopathy has been considered to be related to hypercoagulability and immune complex-mediated vasculitis. Thus, the close association between SLE retinopathy and systemic inflammatory activity emphasizes the importance of systemic immunotherapy. Some people have suggested that systematic treatment should be initiated once SLE retinopathy is identified. The sudden decrease in visual acuity in both eyes was the initial symptom in our case. After further and thorough systemic examinations, the patient was diagnosed with SLE. Different from the previously reported symptoms of SLE patients complicated with ocular arteriovenous obstruction, in this case, the first symptoms for our patient were ocular symptoms, and the patient was diagnosed with SLE after further examination found abnormal immune function and some spots of erythema on the flexion side of both hands and fingers.

In this case, the autoantibodies related to phospholipid syndrome showed no abnormality, and both anti-dsDNA antibodies and anti-SM antibodies were significantly positive, indicating that anti-dsDNA antibodies and anti-SM antibodies were also important factors in the pathogenesis of Purtscher like retinopathy. These findings suggest the close relationship between SLE retinopathy and systemic inflammatory activities and emphasize the importance of systemic immunotherapy.