Abstract Background Cytomegalovirus (CMV) retinitis has been increasingly reported in patients receiving Janus kinase (JAK) inhibitors, yet vision-threatening ischemic complications in this population remain poorly characterized. Immune recovery uveitis (IRU) and cystoid macular edema (CME) are recognized post-infectious sequelae, but delayed retinal ischemia and hemorrhage in the setting of JAK inhibition are uncommon. We present a case that illustrates these complications in a patient on upadacitinib for rheumatoid arthritis. Case presentation An 81-year-old woman with rheumatoid arthritis on upadacitinib for approximately one year and long-term hydroxychloroquine presented with decreased vision and floaters in her left eye. Examination revealed vitritis, focal hemorrhagic retinitis, and an epiretinal membrane without CME. Anterior chamber paracentesis confirmed CMV infection via aqueous humor polymerase chain reaction, guiding prompt antiviral therapy. She received oral valganciclovir and alternating intravitreal ganciclovir and foscarnet, though systemic therapy was later discontinued due to leukopenia. After cessation of upadacitinib and hydroxychloroquine, the retinitis stabilized, but she developed IRU with new-onset CME. Weeks later, she presented with a dense vitreous hemorrhage without retinal tears or detachment. Fluorescein angiography revealed temporal retinal ischemia remote from the original retinitis. Intravitreal bevacizumab led to improvement in both CME and hemorrhage. Pan-retinal photocoagulation is planned to reduce the risk of recurrent hemorrhage. Conclusions This case illustrates CMV retinitis in the setting of JAK inhibition complicated by delayed retinal ischemia and vitreous hemorrhage arising remote from the original retinitis. Although ischemic vasculopathy is a recognized complication of CMV retinitis among non-HIV immunosuppressed patients, such sequelae have been infrequently documented in JAK inhibitor-associated cases. Ophthalmologists should maintain a high index of suspicion for CMV retinitis in this emerging population and continue close surveillance for delayed ischemic complications even after apparent disease stabilization.
Johnson et al. (Mon,) studied this question.