HIV Retinopathy

Retinal disease in patients with human immunodeficiency virus infection. It includes HIV microvasculopathy (the most common ocular manifestation, caused by direct viral and immune complex damage to retinal endothelium) and opportunistic infections of the retina occurring with profound immunosuppression (CD4 count below 100 cells/microlitre).

• HIV microvasculopathy: cotton wool spots, intraretinal haemorrhages, microaneurysms and telangiectasia; seen in 50 percent of untreated patients, mostly with CD4 counts below 200, and it regresses with effective antiretroviral therapy.
• Opportunistic infections: cytomegalovirus retinitis (CD4 below 50; the most common sight-threatening infection), acute retinal necrosis and progressive outer retinal necrosis (varicella zoster virus), toxoplasma retinochoroiditis, syphilitic chorioretinitis, tuberculous choroiditis, cryptococcal and candida chorioretinitis, and pneumocystis choroidopathy.
• Immune recovery uveitis: inflammation after immune reconstitution (CD4 rising above 100) in eyes with previous cytomegalovirus retinitis, causing vitritis, macular oedema, and epiretinal membrane.
• Other: HIV-associated lymphoma, Kaposi sarcoma of the lid and conjunctiva, and optic neuropathy.

• HIV microvasculopathy: usually asymptomatic; transient white cotton wool spots (resolve in 6 to 8 weeks), flame haemorrhages, and rarely macular ischaemia with a mildly reduced colour vision.
• Cytomegalovirus retinitis: painless floaters, blurred vision, field loss; granular, white, necrotic retinal lesions along vessels with haemorrhage ("pizza pie" or "cottage cheese and ketchup" appearance), minimal vitritis, spreads centrifugally 250 micrometres per week, and leads to retinal atrophy and detachment.
• Progressive outer retinal necrosis: rapid painless loss of vision with multifocal deep white lesions, no vitritis, and a poor outcome.
• Acute retinal necrosis: peripheral white lesions, vasculitis, vitritis, and pain.
• Toxoplasmosis: dense white focus with vitritis and atypical multifocal or bilateral lesions in HIV.
• Syphilis: placoid chorioretinitis, vitritis, and optic neuropathy.

• Dilated fundus examination in all HIV patients at diagnosis and when the CD4 count is below 100 (every 3 months) or symptoms occur; HIV viral load and CD4 count.
• Cytomegalovirus retinitis is a clinical diagnosis; confirm by polymerase chain reaction on aqueous or vitreous when atypical. Check blood cytomegalovirus polymerase chain reaction (not diagnostic alone).
• Fluorescein angiography and optical coherence tomography for macular involvement; wide-field photography to monitor progression.
• Serology: toxoplasma IgG, syphilis tests (treponemal test and rapid plasma reagin), tuberculosis test, cryptococcal antigen; aqueous and vitreous polymerase chain reaction for varicella zoster virus, herpes simplex, toxoplasma.
• Brain imaging and lumbar puncture when neurological symptoms coexist.

• Start or optimise combination antiretroviral therapy in all patients (microvasculopathy regresses; immune recovery reduces opportunistic infection risk). Timing for those with opportunistic eye infection: start within 2 weeks in most (delay in cryptococcal meningitis).
• Cytomegalovirus retinitis:
  • Induction: oral valganciclovir 900 mg twice daily for 14 to 21 days (preferred when absorption reliable and lesions are not immediately sight-threatening), or intravenous ganciclovir 5 mg/kg every 12 hours for 14 to 21 days, or intravenous foscarnet 90 mg/kg every 12 hours (hydrate; monitor renal function, calcium, magnesium and potassium), or cidofovir 5 mg/kg weekly for 2 weeks with probenecid (nephrotoxicity risk).
  • Sight-threatening lesions (within 1500 micrometres of the fovea or optic disc): add intravitreal ganciclovir 2 mg in 0.05 mL or foscarnet 2.4 mg in 0.1 mL, twice weekly for 1 to 2 weeks (or until lesions are inactive).
  • Maintenance: oral valganciclovir 900 mg once daily until CD4 above 100 for at least 3 to 6 months on effective therapy; rescreen every 3 months.
  • Retinal detachment (common, 20 to 30 percent): vitrectomy with silicone oil tamponade.
  • Immune recovery uveitis: periocular or intravitreal triamcinolone (4 mg), topical steroid, and sometimes oral prednisolone with antiviral cover.
• Progressive outer retinal necrosis and acute retinal necrosis: intravenous aciclovir 10 mg/kg every 8 hours or foscarnet, with repeated intravitreal foscarnet (2.4 mg) or ganciclovir (2 mg), then oral valaciclovir 1 g three times daily for 6 weeks or more; prophylactic laser or early vitrectomy for detachment.
• Toxoplasma retinochoroiditis: pyrimethamine 200 mg then 50 to 75 mg daily with sulfadiazine 1 to 1.5 g four times daily and folinic acid 15 mg daily for 6 weeks or longer (plus oral prednisolone after 48 hours of antimicrobials if sight-threatening); alternatively trimethoprim-sulfamethoxazole 160/800 mg twice daily.
• Syphilis: aqueous benzylpenicillin 18 to 24 million units daily for 10 to 14 days.
• Counselling on adherence, repeat eye screening, and early symptom reporting (new floaters, blurred vision); registration for visual impairment when appropriate.