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A New Syndrome

Marietta P. Karavellas, MD; Careen Y. Lowder, MD, PhD; J. Christopher Macdonald, MD; Cesar P. Avila, Jr, MD; William R. Freeman, MD

Arch Ophthalmol. 1998;116:169-175.

Objective  To describe a syndrome of posterior segment intraocular inflammation that causes visual loss in patients with acquired immunodeficiency syndrome and cytomegalovirus retinitis. This syndrome was associated with immune recovery mediated by combination antiretroviral treatment including protease inhibitors.

Design  A case-control study at 2 university medical centers.

Participants  One hundred thirty patients with acquired immunodeficiency syndrome and cytomegalovirus retinitis were examined at 2 medical centers for 15 months. In addition, the medical records of 509 patients examined at 1 center for 11 years before the initiation of protease inhibitor therapy were analyzed retrospectively.

Results  Five patients with symptomatic vitritis and papillitis with cystoid macular edema or epiretinal membrane formation were documented. In each patient there was inactive cytomegalovirus retinitis that had not caused visual decrease before the onset of inflammation. All patients had elevated CD4⁺ T lymphocyte levels (median increase, 86x10⁶/L [86 cells/mm³]) after combination treatment including protease inhibitors. Two patients with cystoid macular edema were treated with corticosteroids and had resolution of the cystoid macular edema and an increase in visual acuity without reactivation of the retinitis. Retrospective analysis failed to disclose similar patients with intraocular inflammation in the era before the introduction of protease inhibitors.

Conclusions  This newly described syndrome of posterior segment inflammation related to cytomegalovirus retinitis is a cause of visual morbidity in patients with acquired immunodeficiency syndrome. It is associated with increased immune competence as a result of combined antiretroviral treatment with protease inhibitors and may be amenable to corticosteroid therapy without reactivation of retinitis.

From the Department of Ophthalmology, University of California, San Diego (Drs Karavellas, Macdonald, Avila, and Freeman), and the Cleveland Clinic, Cleveland, Ohio (Dr Lowder).

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