Purtscher-like retinopathy as a first presentation of systematic lupus erythematosus

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease, characterized by the involvement of multiple organ systems. Ocular involvement, being one of the manifestations, contributes to the assessment of disease activity. Purtscher-like retinopathy has been reported as a manifestation of SLE. An otherwise healthy 21-year old female presented with a bilateral rapid decrease in vision for 1 month. Fundus examination revealed multiple cotton wool spots and flame-shaped hemorrhages in both eyes. Optical coherence tomography displayed bilateral severe macular edema. Fundus fluorescein angiography showed multiple retinal arteriolar occlusions. A rheumatologist was consulted for investigation. Based on the American College of Rheumatology 1982 Revised Criteria for diagnosing SLE, a diagnosis of SLE was made. She was subsequently treated with corticosteroids and immunosuppressive medication. Although vaso-occlusive retinopathy is a rare presentation of SLE, it is significant in diagnosing SLE in a patient, to promptly initiate proper treatment.

Purtscher‐like retinopathy associated with systemic lupus erythematosus treated with rituximab plus low‐dose interleukin‐2: A case report

“…Leukoembolization, endothelial damage, activation of complement C5 and microembolization causing arteriolar precapillary occlusion and microvascular infarcts of the retinal nerve fiber layer have been implicated in the pathogenesis of Purtscher‐like retinopathy 3,9 . Purtscher‐like retinopathy secondary to SLE is usually treated with corticosteroids, sometimes with addition of other immunosuppressive agents and local treatment (anti‐VEGF intravitreal injections, sub‐Tenon injections of triamcinolone acetonide, panretinal photocoagulation, and occasionally pars plana vitrectomy) 10,11 . Most case reports have noted the very poor visual outcomes despite the use of different regimens of corticosteroids and immunosuppressive drugs 9,12,13 .…”

“…3,9 Purtscher-like retinopathy secondary to SLE is usually treated with corticosteroids, sometimes with addition of other immunosuppressive agents and local treatment (anti-VEGF intravitreal injections, sub-Tenon injections of triamcinolone acetonide, panretinal photocoagulation, and occasionally pars plana vitrectomy). 10,11 Most case reports have noted the very poor visual outcomes despite the use of different regimens of corticosteroids and immunosuppressive drugs. 9,12,13 The hematologic abnormalities and immunologic markers were not improved when treated with a high dose of corticosteroids and immunosuppressants in this case, and the patient even progressed to retinal involvement.…”

An unusual association between pancreatic cancer and Purtscher-like retinopathy: Presentation of a unique case

“…Other pathologies less frequently associated to Purtscher's-like retinopathy are renal failure [36] , [37] , [38] , anaphylaxis [39] , pre-eclampsia and HELLP syndrome [4] , [21] , [40] , [41] . Cases of association with Covid-19 have also been found in Literature [34] , [35] , being the SARS-Cov-2 infection a predisposing risk of thrombosis.…”

“…Other causes of Purtscher's-like retinopathy include autoimmune diseases such as dermatomiositis [15] , [16] , [17] , [18] , [19] , lupus anticoagulans [20] , [21] , [22] , antiphospholipid syndrome [23] , and Still's disease [24] , [25] .…”

Purtscher-like retinopathy as presenting manifestation of systemic lupus erythromatosus following high-grade fever with myalgia and arthralgia

“…Although the majority of the cases have been described in previously diagnosed patients with SLE,[ 2 ] there are reports of PLR presenting as initial manifestation of SLE. [ 3 ]…”