Ophthalmic manifestations may be the first sign of disseminated systemic HIV infection and have been reported in up to 70% of infected people. These manifestations include
HIV-related microangiopathy of the retina
opportunistic viral, bacterial, parasitic, and fungal infections
Kaposi sarcoma of the eyelid and conjunctiva
lymphomas primarily involving the retina/vitreous (primary vitreoretinal lymphoma), adnexal structures, and orbit
squamous cell carcinoma of the conjunctiva
Reports also suggest that HIV infection itself may cause anterior or intermediate uveitis that is not responsive to corticosteroids but improves with antiretroviral therapy.
The most common ocular finding in patients infected with HIV is HIV retinopathy. It is a microangiopathy, characterized mainly by cotton-wool spots (Fig 15-1) but also by microaneurysms and retinal hemorrhages. HIV has been isolated from the human retina, and its antigen has been detected in retinal endothelial cells. The HIV endothelial infection and/or rheologic abnormalities may play a role in the development of cotton-wool spots and other vascular alterations. In addition, accelerated aging may be a part of HIV-associated eye disease, with earlier onset of macular degeneration and cataracts.
Figure 15-1 Fundus photograph of human immunodeficiency virus (HIV) retinopathy with numerous cotton-wool spots.
(Reprinted with permission from Cunningham ET Jr, Belfort R Jr. HIV/AIDS and the Eye: A Global Perspective. Ophthalmology Monograph 15. San Francisco, CA: American Academy of Ophthalmology; 2002:55.)
Other infectious agents that can affect the eye in HIV-infected patients include cytomegalovirus (CMV), herpes simplex and zoster viruses, Toxoplasma gondii, Treponema pallidum, Mycobacterium tuberculosis, Mycobacterium avium-intracellulare, Cryptococcus neoformans, Pneumocystis jirovecii, Histoplasma capsulatum, Candida species, molluscum contagiosum virus, microsporidia, and others. These pathogens can infect the ocular adnexa, anterior segment, or posterior segment. Visual morbidity, however, occurs primarily with posterior segment involvement, particularly retinitis caused by CMV, herpes simplex or zoster virus, T pallidum, or T gondii.
Dadgostar H, Holland GN, Huang X, et al. Hemorheologic abnormalities associated with HIV infection: in vivo assessment of retinal microvascular blood flow. Invest Ophthalmol Vis Sci. 2006;47(9):3933–3938.
Kalyani PS, Fawzi AA, Gangaputra S, et al; Studies of the Ocular Complications of AIDS Research Group. Retinal vessel caliber among people with acquired immunodeficiency syndrome: relationships with visual function. Am J Ophthalmol. 2012;153(3):428–433.
Before the availability of potent antiretroviral treatment regimens, disseminated CMV infection was the most common opportunistic infection in people with AIDS, and retinal infection was its most clinically important manifestation, occurring in up to 40% of patients with AIDS. CMV retinitis is found mostly in the eyes of individuals with CD4+ counts of 50 cells/μL or less. It is now uncommon in areas where potent combination antiretroviral therapy is available and is an increasing problem in the resource-limited world, particularly in Southeast Asia. Even so, it remains the most common opportunistic ocular infection in patients with AIDS and occasionally is the first AIDS-defining infection found in an individual. See Chapter 11 for a full discussion of CMV retinitis.
Ford N, Shubber Z, Saranchuk P, et al. Burden of HIV-related cytomegalovirus retinitis in resource-limited settings: a systematic review. Clin Infect Dis. 2013;57(9):1351–1361.
Holland GN, Vaudaux JD, Shiramizu KM, et al; Southern California HIV/Eye Consortium. Characteristics of untreated AIDS-related cytomegalovirus retinitis. II. Findings in the era of highly active antiretroviral therapy (1997 to 2000). Am J Ophthalmol. 2008;145(1):12–22.
Jabs DA, Van Natta ML, Thorne JE, et al; Studies of Ocular Complications of AIDS Research Group. Course of cytomegalovirus retinitis in the era of highly active antiretroviral therapy: 1. Retinitis progression. Ophthalmology. 2004;111(12):2224–2231.
Immune recovery uveitis
Immune recovery uveitis (IRU) is an inflammatory process that affects patients with AIDS who previously had CMV retinitis and whose immune status improves with combination antiretroviral therapy. Immune recovery uveitis was defined as an increase in CD4+ T-lymphocyte count of at least 50 cells/μL to the level of 100 cells/μL. The risk factors for developing inflammation depend on the extent of CMV retinitis (CMV retinitis surface area of 25% or more), amount of intraocular CMV antigen, degree of immune constitution, and previous treatment (higher in patients treated with cidofovir). Manifestations of IRU include anterior uveitis, vitritis, uveitic macular edema, epiretinal membrane formation, papillitis, neovascularization of the optic disc or retina and others. Vitreous inflammation may be transient, but some patients may need short courses of systemic or periocular corticosteroids. The risk of recurrent CMV retinitis should be weighed against the benefit of local steroid injections.
El-Bradey MH, Cheng L, Song MK, Torriani FJ, Freeman WR. Long-term results of treatment of macular complications in eyes with immune recovery uveitis using a graded treatment approach. Retina. 2004;24(3):376–382.
Kempen JH, Min YI, Freeman WR, et al; Studies of Ocular Complications of AIDS Research Group. Risk of immune recovery uveitis in patients with AIDS and cytomegalovirus retinitis. Ophthalmology. 2006;113(4):684–694.
Urban B, Bakunowicz-Łazarczyk A, Michalczuk M. Immune recovery uveitis: pathogenesis, clinical symptoms, and treatment. Mediators Inflamm. 2014;2014:971417.
Retinal detachment occurs in up to 50% of patients with CMV retinitis. It may occur during active disease or after successful treatment. With potent antiretroviral regimens available, the rate of retinal detachment has been reduced to 0.06 per patient-year. Risk factors for developing retinal detachment include involvement of all 3 retinal zones, lower CD4+ T-lymphocyte count, and more extensive retinitis. Since there is extensive retinal necrosis and multiple posterior holes, most of these detachments require pars plana vitrectomy with long-term silicone oil tamponade. Anatomical reattachment can be achieved in 90% of patients.
Jabs DA, Van Natta ML, Thorne JE, et al; Studies of Ocular Complications of AIDS Research Group. Course of cytomegalovirus retinitis in the era of highly active antiretroviral therapy: 2. Second eye involvement and retinal detachment. Ophthalmology. 2004;111(12):2232–2239.
Figure 15-2 Fundus photomontage of progressive outer retinal necrosis with relative preservation of vessels and early involvement of posterior pole in a patient with acquired immune deficiency syndrome.
(Courtesy of H. Nida Sen, MD/National Eye Institute.)
Excerpted from BCSC 2020-2021 series: Section 9 - Uveitis and Ocular Inflammation. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.