Concurrent herpetic hypertensive anterior uveitis and cytomegalovirus retinitis in a non-HIV patient with melanoma differentiation-associated gene-5 dermatomyositis: intravenous immunoglobulin comes to the rescue

Melanoma differentiation-associated gene 5 dermatomyositis (MDA-5 DM) is a rare subtype of dermatomyositis. Cytomegalovirus (CMV) retinitis is an opportunistic ocular infection that has historically been linked to HIV infection. Herpes simplex virus (HSV) uveitis is caused by the reactivation of the varicella zoster virus. We present the case of a 69-year-old woman diagnosed with aggressive MDA-5 DM associated with interstitial lung disease, who received multiple immunosuppressive therapies and subsequently developed left eye CMV retinitis and right eye acute herpetic, hypertensive, anterior uveitis with dendritic keratitis. After the diagnosis of MDA-5 DM, she was treated with glucocorticoids (GC), followed by rituximab and triple therapy (cyclosporin, cyclophosphamide, and GC); however, she did not improve and multiple adverse effects occurred. The patient then started tofacitinib (TOF) and improved. While being on TOF, she developed right eye acute herpetic, hypertensive, anterior uveitis followed by left eye CMV retinitis. TOF was stopped, and the patient started oral acyclovir and intravenous and intravitreal ganciclovir, which was later changed to maintenance therapy with oral valganciclovir. Cyclical intravenous immunoglobulin (IVIG) administration was started, resulting in an overall improvement in disease activity associated with MDA-5 DM. This case underscores the uncommon link between CMV retinitis in a non-HIV patient and HSV anterior uveitis in a patient undergoing immunosuppressive therapy and the challenge of treating MDA-5 DM with IVIG, which led to favorable results.