Progressive Outer Retinal Necrosis and Immunosuppressive Therapy in Myasthenia Gravis

Abstract

Introduction: Progressive outer retinal necrosis (PORN) is a rare but devastating infectious retinitis associated with varicella zoster virus (VZV) and responsible for severe visual loss. Case Report: A 59-year-old man treated for generalized myasthenia with oral azathioprine and prednisone presented with severe unilateral necrotizing retinitis. Polymerase chain reaction of the aqueous and vitreous humors was diagnostic for VZV PORN. Conclusion: VZV PORN is a severe potential ocular complication of immunosuppression, prompting urgent diagnosis and appropriate treatment.

Progressive outer retinal necrosis-like retinitis in immunocompetent hosts

“…The case reported was also on glucocorticoids (60 mg prednisolone) without antiviral therapy prior to the final diagnosis of necrotising retinitis 13. PORN has also been reported in patients on immunosuppressive therapy (combination of azathioprine and glucocorticoids)14 and in patients with idiopathic lymphocytopenia 15. The lack of significant inflammation in the presence of retinal necrosis and the dramatic response to valaciclovir are suggestive of herpetic viral aetiology in our cases.…”

Cytomegalovirus implicated in a case of progressive outer retinal necrosis (PORN)

“…PORN was first described in 1990 [2]. It presents generally in immunocompromised patients [7,8], but most frequently in HIV/AIDS patients [2][3][4][5][6][9][10][11][12][13]. It is characterized clinically as showing minimal or no inflammation in the aqueous and vitreous humors, an absence of retinal vasculitis, and patches of yellowish spots located deep in the retina [1,2].…”

“…Despite aggressive therapy, most of patients with PORN end up with no light perception vision [1]. Although multiple antiviral combinations have been attempted, no treatment regimen is universally accepted for progressive outer retinal necrosis [1][2][3][4][5][6][7][8][9][10][11][12][13]. However, recent case series suggest that combined intravenous and intravitreal antivirals may modestly improve final visual outcomes [13].…”

Viral retinitis: diagnosis and management in the era of biologic immunosuppression: A review

“…Engstrom et al reported a median CD4+ count of 21 cells/μL in their cohort . Non‐AIDS cases have been associated with host and/or iatrogenic immunosuppression such as following renal transplantation . Only one case in an immunocompetent patient has been reported …”

“…85 Non-AIDS cases have been associated with host and/or iatrogenic immunosuppression such as following renal transplantation. [87][88][89][90][91] Only one case in an immunocompetent patient has been reported. 92 PORN is rare and the incidence may decline in parallel with decreasing rates of AIDS due to the efficacy of antiretroviral therapy (ART).…”