CMV Retinitis in Undetected Immune Deficiency Syndrome

A 38-year-old woman presented with autoimmune hepatitis during treatment with azathioprine. Her temperature was subfebrile and she had a non-productive cough. Azathioprine was discontinued owing to the presence of lymphocytopenia and a C-reactive protein (CRP) level of 214 mg/l; Pneumocystis pneumonia was ruled out. The CRP remained high despite empirical antibiotic treatment. Acute vision loss then occurred in both eyes. The ophthalmoscopy and optical coherence tomography (OCT) findings were typical for cytomegalovirus (CMV) retinitis (Figure). Laboratory tests revealed deficiency of IgG and IgA, depleted class-switched memory B cells, lack of vaccine response, and CD4-cell lymphocytopenia, compatible with common variable immunodeficiency (CVID). Treatment with ganciclovir, anti-CMV-IgG, and IVIG reduced the retinal exudation and restored visual acuity to 20/20. After discontinuation of azathioprine, treatment of the autoimmune hepatitis with the corticosteroid budenoside, and CVID treatment with immunoglobulins, the CD4 cells eventually returned to the normal level. The prevalence of CVID is 1:25 000 to 1:40 000. Patients suffer from acute immunoglobulin deficiency together with a pathological susceptibility to infection and immune dysregulation with autoimmune phenomena.

Figure

Typical fundus image of CMV retinitis with cystoid edema on optical coherence tomography (OCT)

Prof. Dr. med. Andreas Stahl, Klinik und Poliklinik für Augenheilkunde, Universitätsmedizin Greifswald, [e-mail]

PD Dr. med. Lukas Bossaller, Bereich Rheumatologie, Klinik für Innere Medizin A, Universitätsmedizin Greifswald

Conflict of interest statement: The authors declare that no conflict of interest exists.

Translated from the original German by David Roseveare.

Cite this as: Stahl A, Bossaller L: CMV retinitis in undetected immune deficiency syndrome. Dtsch Arztebl Int 2022; 119: 494. DOI: 10.3238/arztebl.m2022.0126