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Case of combination therapy to treat lupus retinal vasculitis refractory to steroids

2016; Elsevier BV; Volume: 52; Issue: 1 Linguagem: Inglês

10.1016/j.jcjo.2016.07.027

1715-3360

Nadeem Ali Dhirani, Vandana Ahluwalia, Sohel Somani,

Retinal and Optic Conditions

Retinal vasculitis is a known ocular manifestation of systemic lupus erythematosus (SLE). This condition is characterized by inflammation and occlusion of retinal arterioles or venules, thought to be caused by the overproduction of antibodies and/or immune complexes associated with SLE. While the most predominant way to treat conditions associated with SLE remains corticosteroid therapy, certain patients may prove refractory to such an approach.1Damato E. Chilov M. Lee R. Singh A. Harper S. Dick A. Plasma exchange and rituximab in the management of acute occlusive retinal vasculopathy secondary to systemic lupus erythematosus.Ocul Immunol Inflamm. 2011; 19: 379-381Crossref PubMed Scopus (16) Google Scholar, 2Hickman R.A. Denniston A.K. Yee C.S. Toescu V. Murray P.I. Gordon C. Bilateral retinal vasculitis in a patient with systemic lupus erythematosus and its remission with rituximab therapy.Lupus. 2010; 19: 327-329Crossref PubMed Scopus (28) Google Scholar, 3Papadaki T.G. Zacharopoulos I.P. Papaliodis G. Iaccheri B. Fiore T. Foster C.S. Plasmapheresis for lupus retinal vasculitis.Arch Ophthalmol. 2006; 124: 1654-1656Crossref PubMed Scopus (21) Google Scholar We present a case of severe retinal vasculitis that was refractory to steroid therapy but responded to a combination of plasmapheresis, intravenous (IV) rituximab, and intravitreal ranibizumab. A 34-year-old Indian female diagnosed with SLE presented with painless vision loss in her right eye. The patient's best-corrected visual acuity was no light perception (NLP) OD and 20/20 OS. Fundus examination of the right eye demonstrated diffuse retinal hemorrhage, pallid optic nerve swelling, diffusely swollen macula with a cherry-red appearance, and vascular sheathing. Fundus examination of the left eye was normal. The patient was admitted for IV high dose pulse steroids treatment (Solu-Medrol (Pfizer) 1 g daily). Three days postadmission, the patient began complaining of blurry vision in the left eye despite treatment. Visual acuity declined to 20/400 OS, and fundus examination demonstrated diffuse macular edema, retinal arteriolar sheathing, hemorrhage, and swollen optic nerve (Fig. 1). The right eye remained with NLP vision. Given these changes, the patient was diagnosed with refractory retinal vasculitis. She underwent plasmapheresis therapy (5 sessions over a 10-day span) and bilateral administration of intravitreal ranibizumab (0.5 mg). Postplasmapheresis, the patient also underwent IV rituximab therapy. Three weeks following this therapy, visual acuity was light perception (LP) OD and 20/70 OS. She was discharged with a prescription of 60 mg oral prednisone daily. One month later, visual acuity improved to 20/40 OS and fundus examination demonstrated resolution of retinal vasculitis in the left eye. At her 1-year follow-up, visual acuity was LP OD and 20/30 OS with a normal fundus appearance in the left eye (Fig. 2). Uncontrolled retinal vasculitis can lead to vaso-occlusive complications, including macular ischemia, neovascularization, vitreous hemorrhage, and tractional retinal detachment.4Hughes E.H. Dick A.D. The pathology and pathogenesis of retinal vasculitis.Neuropathol Appl Neurobiol. 2003; 29: 325-340Crossref PubMed Scopus (51) Google Scholar, 5Palejwala N.V. Walia H.S. Yeh S. Ocular manifestations of systemic lupus erythematosus: a review of the literature.Autoimmune Dis. 2012; 2012: 1-9Crossref Scopus (123) Google Scholar These complications may yield irreversible vision loss as demonstrated by the right eye of the patient. Early intervention and aggressive corticosteroid therapy is the mainstay of treatment. However, some SLE patients may be refractory to corticosteroid therapy and require further aggressive immunosuppression. Only 3 reports of refractory SLE retinal vasculitis have been published in the literature, using plasmapheresis and rituximab, rituximab alone, or plasmapheresis alone.1Damato E. Chilov M. Lee R. Singh A. Harper S. Dick A. Plasma exchange and rituximab in the management of acute occlusive retinal vasculopathy secondary to systemic lupus erythematosus.Ocul Immunol Inflamm. 2011; 19: 379-381Crossref PubMed Scopus (16) Google Scholar, 2Hickman R.A. Denniston A.K. Yee C.S. Toescu V. Murray P.I. Gordon C. Bilateral retinal vasculitis in a patient with systemic lupus erythematosus and its remission with rituximab therapy.Lupus. 2010; 19: 327-329Crossref PubMed Scopus (28) Google Scholar, 3Papadaki T.G. Zacharopoulos I.P. Papaliodis G. Iaccheri B. Fiore T. Foster C.S. Plasmapheresis for lupus retinal vasculitis.Arch Ophthalmol. 2006; 124: 1654-1656Crossref PubMed Scopus (21) Google Scholar All of these reports had varying degrees of visual success. The apparent resolution of retinal vasculitis in this patient was likely due to a combined effect of her therapy. The underlying mechanism of SLE retinal vasculitis, although not clearly understood, is thought to involve immune complex deposition in the arterioles and antibody mediated inflammation of the vessel wall leading to retinal damage.4Hughes E.H. Dick A.D. The pathology and pathogenesis of retinal vasculitis.Neuropathol Appl Neurobiol. 2003; 29: 325-340Crossref PubMed Scopus (51) Google Scholar, 5Palejwala N.V. Walia H.S. Yeh S. Ocular manifestations of systemic lupus erythematosus: a review of the literature.Autoimmune Dis. 2012; 2012: 1-9Crossref Scopus (123) Google Scholar Plasmapheresis is believed to provide a significant and immediate reduction of such intravascular inflammatory complexes.3Papadaki T.G. Zacharopoulos I.P. Papaliodis G. Iaccheri B. Fiore T. Foster C.S. Plasmapheresis for lupus retinal vasculitis.Arch Ophthalmol. 2006; 124: 1654-1656Crossref PubMed Scopus (21) Google Scholar Rituximab, an anti-CD20 chimeric antibody, is believed to provide a sustained suppression of new inflammatory mediators by selectively targeting B cells, which are central in the pathogenesis of SLE.2Hickman R.A. Denniston A.K. Yee C.S. Toescu V. Murray P.I. Gordon C. Bilateral retinal vasculitis in a patient with systemic lupus erythematosus and its remission with rituximab therapy.Lupus. 2010; 19: 327-329Crossref PubMed Scopus (28) Google Scholar Ranibizumab, an antivascular endothelial growth factor (VEGF), may have also been beneficial in reducing macular edema; however, there are limited reports for its use in this context, with the exception of a report using it as an adjunct therapy for retinal vasculitis of a different etiology.6Marín-Lambíes C. Gallego-Pinazo R. Salom D. Navarrete J. Díaz-Llopis M. Rapid regression of exudative maculopathy in idiopathic retinitis, vasculitis, aneurysms and neuroretinitis syndrome after intravitreal ranibizumab.Case Rep Ophthalmol. 2012; 3: 251-257Crossref PubMed Scopus (6) Google Scholar Although its presumed clinical effect in these cases is to control neovascularization and limit excess VEGF, further definitive therapeutic studies are warranted. To the best of our knowledge, this is the first report of refractory retinal vasculitis in an SLE patient having been successfully treated with this specific combination. Her improvement suggests that more investigation into combination immunosuppressive therapy for refractory retinal vasculitis may be warranted, particularly with these agents.