Retinal arteriolar occlusions due to cytomegalovirus retinitis in elderly patients without HIV

Abstract

BackgroundFive of 7 (71%) elderly immunocompetent patients with cytomegalovirus retinitis had retinal arteriolar occlusions versus 2 of 8 (25%) elderly immunocompromised patients and 1 of 19 (5%) younger HIV-infected patients. Compared to HIV-infected patients, elderly patients were more likely to have occlusive events, neovascularization or hemorrhage, and underlying vasculopathy. The purpose of this study is to report the novel finding of extensive retinal arteriolar occlusions and neovascularization in immunocompetent patients with cytomegalovirus retinitis. This is a retrospective observational cohort study of cytomegalovirus retinitis (CMVR) in a university setting. Seven patients were elderly but not immunocompromised, 8 were elderly and iatrogenically immunocompromised, and 16 were HIV-infected. All patients underwent polymerase chain reaction testing of intraocular fluid. Primary outcome measure was visual acuity. Secondary outcome measures were vascular occlusions, ischemic complications, and response to treatment.ResultsMean age was 73, 70, and 41 years for immunocompetent, immunocompromised, and HIV-infected patients, respectively. Diabetes and vascular disease were common in the elderly. Vision loss to less than 5/200 occurred in 50% of the immunocompetent elderly patients, and 17% of CMV eyes in immunocompromised and HIV patients. Occlusion of the entire retinal vasculature occurred in 4/7 (57%) of immunocompetent patients despite lack of Zone I involvement, and rubeosis occurred in three, disc neovascularization in one, and vitreous hemorrhage in two patients. Vascular occlusive events were less common in immunocompromised patients and rare in the HIV-infected.ConclusionsCMVR in non-HIV-infected elderly patients is associated with retinal arteriolar occlusions. An intact host immune response may increase damage to retinal vessels. Prompt diagnosis may avert catastrophic vision loss.

Successful treatment of cytomegalovirus retinitis with oral/intravitreal antivirals in HIV-negative patients with lymphoma

“…However, they concluded that the non-HIV patients presented more frequently with signs of intraocular inflammation such as vitritis, retinal arteritis, and vascular occlusions, compared to the HIV subjects [ 20 ]. Similar findings were described by Schneider et al in 2013 and by Davis et al in 2013, who described that CMV retinitis in non-HIV patients was associated with retinal arteriolar occlusions in elderly patients [ 12 , 21 ]. These findings correspond to the ones from our case series, where 4 out of 7 patients (57%) had anterior segment inflammation at first presentation and the same percentage presented also with vitreous haze at the first visit (Table 1 ) and 2 out of 7 demonstrated retinal vasculitis either at first presentation or as secondary retinal vein occlusion.…”

Successful treatment of cytomegalovirus retinitis with oral/intravitreal antivirals in HIV-negative patients with lymphoma

“…However, they concluded that the non-HIV patients presented more frequently with signs of intraocular inflammation such as vitritis, retinal arteritis, and vascular occlusions, compared to the HIV subjects [ 20 ]. Similar findings were described by Schneider et al in 2013 and by Davis et al in 2013, who described that CMV retinitis in non-HIV patients was associated with retinal arteriolar occlusions in elderly patients [ 12 , 21 ]. These findings correspond to the ones from our case series, where 4 out of 7 patients (57%) had anterior segment inflammation at first presentation and the same percentage presented also with vitreous haze at the first visit (Table 1 ) and 2 out of 7 demonstrated retinal vasculitis either at first presentation or as secondary retinal vein occlusion.…”

Neovascular Complications From Cytomegalovirus Necrotizing Retinopathy in Patients After Haploidentical Hematopoietic Stem Cell Transplantation

“…Limited immune dysfunction, the continuous replication of CMV in vascular endothelial cells and CMV-specific T-cell-mediated endothelial cell damage may be the possible mechanism for this phenomenon. 4,6,20,22 In addition to the spectrum proposed by Schneider et al, 4 insidious immune-mediated CMV vasculitis may be an isolated manifestation for CMV necrotizing retinopathy when immune deviation was even lesser than patients with CRN.…”

“…Ever since the first case of neovascular event complicating CMV necrotizing retinopathy reported by Saran et al in 1996, 19 a dozen of similar cases could be found in the literature at present. 6,7,[20][21][22] Neovascular complications of HIV-related CMV retinitis are rare, but the incidence was recognized to be higher in non-HIV patients. 21,22 All the cases reported share similar clinical manifestation including granular peripheral retinitis, panretinal occlusive vasculitis, and some degree of intraocular inflammation, which fit the criteria of acute retinal necrosis defined by the American Uveitis Society.…”

“…6,7,[20][21][22] Neovascular complications of HIV-related CMV retinitis are rare, but the incidence was recognized to be higher in non-HIV patients. 21,22 All the cases reported share similar clinical manifestation including granular peripheral retinitis, panretinal occlusive vasculitis, and some degree of intraocular inflammation, which fit the criteria of acute retinal necrosis defined by the American Uveitis Society. 5 Schneider et al 4 proposed that CRN caused by CMV is related to the immune status of the host.…”

Rapidly Progressive Neovascular Glaucoma From Cytomegalovirus Retinitis in a Non–human Immunodeficiency Virus Patient

“…6 Recently, there are increasing case reports of CMV retinitis in non-HIV patients with atypical features. 7 Schneider et al 8 termed the phrase "chronic retinal necrosis" in their description of five cases of smoldering CMV retinitis associated with significant vitritis and panretinal vasculitis more typical of acute retinal necrosis. These patients were reported to have symptoms for weeks to months and some were observed to have no progression of retinitis even without appropriate antiviral coverage.…”

Successful treatment of cytomegalovirus retinitis with oral/intravitreal antivirals in HIV-negative patients with lymphoma

“…However, they concluded that the non-HIV patients presented more frequently with signs of intraocular inflammation such as vitritis, retinal arteritis, and vascular occlusions, compared to the HIV subjects [ 20 ]. Similar findings were described by Schneider et al in 2013 and by Davis et al in 2013, who described that CMV retinitis in non-HIV patients was associated with retinal arteriolar occlusions in elderly patients [ 12 , 21 ]. These findings correspond to the ones from our case series, where 4 out of 7 patients (57%) had anterior segment inflammation at first presentation and the same percentage presented also with vitreous haze at the first visit (Table 1 ) and 2 out of 7 demonstrated retinal vasculitis either at first presentation or as secondary retinal vein occlusion.…”

Neovascular Complications From Cytomegalovirus Necrotizing Retinopathy in Patients After Haploidentical Hematopoietic Stem Cell Transplantation

“…Limited immune dysfunction, the continuous replication of CMV in vascular endothelial cells and CMV-specific T-cell-mediated endothelial cell damage may be the possible mechanism for this phenomenon. 4,6,20,22 In addition to the spectrum proposed by Schneider et al, 4 insidious immune-mediated CMV vasculitis may be an isolated manifestation for CMV necrotizing retinopathy when immune deviation was even lesser than patients with CRN.…”

“…Ever since the first case of neovascular event complicating CMV necrotizing retinopathy reported by Saran et al in 1996, 19 a dozen of similar cases could be found in the literature at present. 6,7,[20][21][22] Neovascular complications of HIV-related CMV retinitis are rare, but the incidence was recognized to be higher in non-HIV patients. 21,22 All the cases reported share similar clinical manifestation including granular peripheral retinitis, panretinal occlusive vasculitis, and some degree of intraocular inflammation, which fit the criteria of acute retinal necrosis defined by the American Uveitis Society.…”

“…6,7,[20][21][22] Neovascular complications of HIV-related CMV retinitis are rare, but the incidence was recognized to be higher in non-HIV patients. 21,22 All the cases reported share similar clinical manifestation including granular peripheral retinitis, panretinal occlusive vasculitis, and some degree of intraocular inflammation, which fit the criteria of acute retinal necrosis defined by the American Uveitis Society. 5 Schneider et al 4 proposed that CRN caused by CMV is related to the immune status of the host.…”

Rapidly Progressive Neovascular Glaucoma From Cytomegalovirus Retinitis in a Non–human Immunodeficiency Virus Patient

“…6 Recently, there are increasing case reports of CMV retinitis in non-HIV patients with atypical features. 7 Schneider et al 8 termed the phrase "chronic retinal necrosis" in their description of five cases of smoldering CMV retinitis associated with significant vitritis and panretinal vasculitis more typical of acute retinal necrosis. These patients were reported to have symptoms for weeks to months and some were observed to have no progression of retinitis even without appropriate antiviral coverage.…”

Successful treatment of cytomegalovirus retinitis with oral/intravitreal antivirals in HIV-negative patients with lymphoma

“…However, they concluded that the non-HIV patients presented more frequently with signs of intraocular inflammation such as vitritis, retinal arteritis, and vascular occlusions, compared to the HIV subjects [ 20 ]. Similar findings were described by Schneider et al in 2013 and by Davis et al in 2013, who described that CMV retinitis in non-HIV patients was associated with retinal arteriolar occlusions in elderly patients [ 12 , 21 ]. These findings correspond to the ones from our case series, where 4 out of 7 patients (57%) had anterior segment inflammation at first presentation and the same percentage presented also with vitreous haze at the first visit (Table 1 ) and 2 out of 7 demonstrated retinal vasculitis either at first presentation or as secondary retinal vein occlusion.…”

Neovascular Complications From Cytomegalovirus Necrotizing Retinopathy in Patients After Haploidentical Hematopoietic Stem Cell Transplantation

“…Limited immune dysfunction, the continuous replication of CMV in vascular endothelial cells and CMV-specific T-cell-mediated endothelial cell damage may be the possible mechanism for this phenomenon. 4,6,20,22 In addition to the spectrum proposed by Schneider et al, 4 insidious immune-mediated CMV vasculitis may be an isolated manifestation for CMV necrotizing retinopathy when immune deviation was even lesser than patients with CRN.…”

“…Ever since the first case of neovascular event complicating CMV necrotizing retinopathy reported by Saran et al in 1996, 19 a dozen of similar cases could be found in the literature at present. 6,7,[20][21][22] Neovascular complications of HIV-related CMV retinitis are rare, but the incidence was recognized to be higher in non-HIV patients. 21,22 All the cases reported share similar clinical manifestation including granular peripheral retinitis, panretinal occlusive vasculitis, and some degree of intraocular inflammation, which fit the criteria of acute retinal necrosis defined by the American Uveitis Society.…”

“…6,7,[20][21][22] Neovascular complications of HIV-related CMV retinitis are rare, but the incidence was recognized to be higher in non-HIV patients. 21,22 All the cases reported share similar clinical manifestation including granular peripheral retinitis, panretinal occlusive vasculitis, and some degree of intraocular inflammation, which fit the criteria of acute retinal necrosis defined by the American Uveitis Society. 5 Schneider et al 4 proposed that CRN caused by CMV is related to the immune status of the host.…”

Rapidly Progressive Neovascular Glaucoma From Cytomegalovirus Retinitis in a Non–human Immunodeficiency Virus Patient

“…6 Recently, there are increasing case reports of CMV retinitis in non-HIV patients with atypical features. 7 Schneider et al 8 termed the phrase "chronic retinal necrosis" in their description of five cases of smoldering CMV retinitis associated with significant vitritis and panretinal vasculitis more typical of acute retinal necrosis. These patients were reported to have symptoms for weeks to months and some were observed to have no progression of retinitis even without appropriate antiviral coverage.…”