title: Good syndrome and other causes of cytomegalovirus retinitis in HIV-negative patients-case report and comprehensive review of the literature.
abstract: We describe a 65-year-old Thai woman who developed cytomegalovirus retinitis (CMVR) in the setting of Good syndrome-a rare, acquired partial immune deficiency caused by thymoma. The patient subsequently developed vitritis with cystoid macular edema (CME) similar to immune recovery uveitis (IRU) despite control of the retinitis with antiviral agents. A comprehensive review of the literature through December, 2014, identified an additional 279 eyes of 208 patients with CMVR in the absence of human immunodeficiency virus (HIV) infection. Including our newly reported case, 9 of the 208 patients (4.3 %) had Good syndrome. Twenty-one of the 208 patients (10.1 %) had CMVR related to intraocular or periocular corticosteroid administration. The remaining 178 patients (85.6 %) acquired CMVR from other causes. Within the subset of patients who did not have Good syndrome or did not acquire CMVR followed by intraocular or periocular corticosteroid administration, there were many other factors contributing to a decline in immune function. The most common included age over 60 years (33.1 %), an underlying malignancy (28.7 %), a systemic autoimmune disorder requiring systemic immunosuppression (19.1 %), organ (15.2 %) or bone marrow (16.3 %) transplantation requiring systemic immunosuppression, and diabetes mellitus (6.1 %). Only 4.5 % of the patients had no identifiable contributor to a decline in immune function. While the clinical features of CMVR are generally similar in HIV-negative and HIV-positive patients, the rates of moderate to severe intraocular inflammation and of occlusive retinal vasculitis appear to be higher in HIV-negative patients.
authors.affiliation: The Department of Ophthalmology, California Pacific Medical Center, 2340 Clay Street 5th, San Francisco, CA, 94115, USA. kdownes07@gmail.com.
authors.forename: Kenneth M
authors.initials: KM
authors.lastname: Downes
authors.affiliation: The Department of Ophthalmology, Kaiser Permanente Medical Center, South San Francisco, CA, USA.
authors.forename: Dariusz
authors.initials: D
authors.lastname: Tarasewicz
authors.affiliation: The Department of Hematology/Oncology, Kaiser Permanente South San Francisco Medical Center, South San Francisco, CA, USA.
authors.forename: Laurie J
authors.initials: LJ
authors.lastname: Weisberg
authors.affiliation: The Department of Ophthalmology, California Pacific Medical Center, 2340 Clay Street 5th, San Francisco, CA, 94115, USA.|The Department of Ophthalmology, Stanford University School of Medicine, Stanford, CA, USA.|The Francis I. Proctor Foundation, UCSF School of Medicine, San Francisco, CA, USA.|West Coast Retina Medical Group, San Francisco, CA, USA.
authors.forename: Emmett T
authors.initials: ET
authors.lastname: Cunningham
keywords: Good syndrome; Herpetic retinitis; Immunosuppression; Thymoma; Uveitis
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