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.Electronic supplementary materialThe online version of this article (doi:10.1186/s12348-016-0070-7) contains supplementary material, which is available to authorized users.
Highlights
Thymoma is an uncommon and slow-growing neoplasm that accounts for approximately 20 to 30 % of mediastinal masses in adults and 1 % in children [1]
Good syndrome typically occurs in middle-aged adults and is associated most commonly with recurrent sinus and pulmonary infections, cytomegalovirus (CMV) disease, fungal infections, pure red cell aplasia, and myasthenia gravis [8]
Comprehensive literature review We describe a patient who developed cytomegalovirus retinitis (CMVR) in the setting of Good syndrome, a rare occurrence reported in eight previous patients to date (Tables 2 and 3) [10,11,12,13,14,15,16]
Summary
Thymoma is an uncommon and slow-growing neoplasm that accounts for approximately 20 to 30 % of mediastinal masses in adults and 1 % in children [1]. While hypogammaglobulinemia in the setting of thymoma defines Good syndrome, other, often partial, immune deficiencies have been described, including decreased T cell function [9]. We describe a 65-year-old woman who developed CMV retinitis (CMVR) in the setting of Good syndrome. Comprehensive literature review We describe a patient who developed CMVR in the setting of Good syndrome, a rare occurrence reported in eight previous patients to date (Tables 2 and 3) [10,11,12,13,14,15,16]. While the CMVR in our patient occurred 1 month prior to the identification of thymoma, a thymic tumor was identified prior to the development of CMVR in the other eight patients, with a time ranging from 1 month to just over 6 years prior to the occurrence of retinitis. Arch Ophthalmol. 2011;129(11):1507–8 [115] cDosing with each modality varied widely across studies
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