© Springer Nature Switzerland AG 2021
C. S. Foster et al. (eds.)Uveitishttps://doi.org/10.1007/978-3-030-52974-1_60

60. HIV-Associated Uveitis

Boonsiri Hunchangsith1  
(1)
Banphaeo Eye Hospital (Public Organization), Samutsakorn, Thailand
 
Keywords
HIV-associated uveitisHIVHIV retinopathyImmune recovery uveitisPneumocystis carinii choroidopathyMycobacterium avium complex

HIV (Human Immunodeficiency Virus)

Overview

  • Retrovirus that infects CD4+ T lymphocyte (cell-mediated immunity)

  • Severe immunodeficiency permits the development of opportunistic infections and malignancies

  • Transmission

    • Sexual contact

    • Blood: Intravenous Drug Use (IVDU), blood products

    • Mother to child

  • Highly active antiretroviral therapy (HAART) has led to dramatic improvement in the prognosis of HIV infection

Ocular Manifestations of HIV Infection

  • Most common

    • Ocular manifestation of human immunodeficiency virus, acquired immunodeficiency syndrome (HIV-AIDS): HIV retinopathy

    • Retinal opportunistic infection: Cytomegalovirus (CMV) retinitis

  • Multifocal choroiditis

    • Tuberculosis (TB), mycobacterium avium complex, histoplasmosis, cryptococcosis, pneumocystosis, other fungi, lymphoma

    • Look for disseminated systemic infection

HIV Retinopathy

Overview

  • Definition

    • Retinal microvasculopathy

    • Most common ocular manifestation of HIV-AIDS

  • Symptoms

    • Asymptomatic

    • Blurring with macular involvement

  • Laterality: Mostly bilateral

  • Course: Spontaneously resolved within weeks or months

  • Age of onset: any

  • Gender/race: no gender or racial predilection

  • Systemic association: Usually found in HIV patients with low CD4 count

Exam: Ocular

Anterior Segment

  • No inflammation

Posterior Segment

  • Cotton-wool spots

    • Posterior pole, often adjacent to retinal vessels

    • Large or progressive lesions should cause suspicion of early CMV retinitis

  • Scattered dot blot or flame-shaped hemorrhages

  • Microaneurysms

  • Isolated perivascular sheathing without opportunistic infections

    • Common in African patients (especially children)

Exam: Systemic

  • Variable depending on opportunistic infections

  • Acute systemic infection (HIV prodrome)

    • Occurs days to weeks after inoculation

    • Fever, lymphadenopathy, pharyngitis, rash, myalgia, malaise, headache, nausea, weight loss, diarrhea, night sweats, neurologic symptoms

    • Patients are more infectious during acute primary infection

  • Latent stage

    • Occurs weeks to years after inoculation

    • Lymphadenopathy

  • Acquired immune deficiency syndrome (AIDS)

    • Less common in the setting of HAART

    • Designated as AIDS with the onset of opportunistic infections or malignancies that are uncommon in the general population

Imaging

  • FA: hyperfluorescent microaneurysms; microvasculopathy, occlusive disease; blocking defects with hemorrhages

Laboratory and Radiographic Testing

  • HIV 1/2 Ag Ab immunoassay

  • CD4 count/%

  • Viral load

  • Complete Blood Count (CBC) with differential

  • Comprehensive Metabolic Panel (CMP)

  • βhCG

  • Serologic screening for various infectious comorbidities

Differential Diagnosis

  • Early CMV retinopathy

  • Diabetes/hypertensive retinopathy

Treatment

  • No treatment for HIV retinopathy

  • HAART for systemic infection

Referral/Co-management

  • Infectious Disease

  • Primary Care

CMV Retinitis

See Chap. 35 Herpesviruses

Immune Recovery Uveitis (IRU)

Overview

  • Definition
    • An ocular form of immune reconstitution inflammatory syndrome (IRIS) involving increased ocular inflammation in previously opportunistic infected eyes, after improvement of immune function from HAART therapy

      • CMV (most common)

      • TB

      • Toxoplasma

    • Typically manifests 2–16 weeks after CD4 count increases above 100 cells/μL following the initiation of HAART

  • Symptoms

    • Asymptomatic

    • Blurring

    • Floaters

  • Laterality: unilateral or bilateral

  • Course: subacute

  • Age of onset: any

  • Gender/race: no gender or racial predilection; any exposed individual

  • Systemic association
    • Diseases associated

      • CMV retinitis: most common

      • Inactive toxoplasmosis

      • Tuberculous retinochoroiditis

      • Cryptococcal infection

    • Risk factors

      • Inactive CMV retinitis involving more than 30% of retinal area

      • Patients initially treated with cidofovir

      • Low CD4+ cell before starting HAART

Exam: Ocular

Anterior Segment

  • Anterior uveitis

  • Cataract

Posterior Segment

  • Vitritis

  • Cystoid macular edema (CME)

  • Epiretinal membrane

  • Retinal and optic disc neovascularization

  • Glaucoma

Exam: Systemic

  • Variable depending on opportunistic infections

  • Same as HIV retinopathy, but expect systemic course to be improved with immune recovery

Imaging

  • OCT: CME, ERM

Laboratory and Radiographic Testing

  • CD4 count elevated above 100 cells/μL

  • Serologic testing to evaluate for/rule out other forms of inflammation

Differential Diagnosis

  • Relapse of the previous infection

  • New opportunistic infection

Treatment

  • Can be self-limiting

  • Appropriate antimicrobial therapy

  • Topical corticosteroid for anterior segment inflammation

  • Mild vitritis without CME with good vision can be observed

  • Intravitreal, periocular, or short-course systemic corticosteroids for vitritis and CME

Pneumocystis carinii Choroidopathy

Overview

  • Definition

    • Caused by fungus Pneumocystis jirovecii (former name Pneumocystis carinii)

    • Choroidal involvement is a sign of disseminated life-threatening fungal infection

  • Symptoms

    • Loss of central vision or no visual disturbance

  • Laterality: mostly bilateral

  • Course: subacute

  • Age of onset: any

  • Gender/race: no predilection; any exposed individual

  • Systemic association

    • CD4 count below 50 cells/μL (in contrast to CD4 200 cells/μL in pneumonia patient)

    • Pulmonary infection

    • Extrapulmonary: lymph nodes, spleen, liver, bone marrow

Exam: Ocular

Anterior Segment

  • No anterior chamber reaction

Posterior Segment

  • Plaque-like, deep, yellow-white, round or multilobular foci at the posterior pole

  • No vitritis or vasculitis

Exam: Systemic

  • Full physical examination may reveal findings, especially in lung, lymph nodes, and GI

Imaging

  • FA: early hypofluorescence and late staining

Laboratory and Radiographic Testing

  • Diagnosis is based on clinical and FA findings

  • Systemic workup for disseminated disease: Chest X-ray, sputum culture, CBC, liver function test

Differential Diagnosis

  • Fungus: candidiasis, cryptococcosis, histoplasmosis

  • Syphilis

  • Tuberculosis, mycobacterium avium complex

  • Primary intraocular lymphoma

Treatment

  • Systemic trimetroprim (15 mg/kg/day)–sulfamethoxazole (75 mg/kg/day) in 3 divided doses, for 21 days

  • Followed by secondary prophylaxis until immune reconstitution (CD4 >200 cells/μL)

Referral/Co-management

  • Infectious Disease

  • Pulmonologist

Mycobacterium Avium Complex (MAC)

Overview

  • Definition

    • MAC disease typically is a disseminated, multi-organ infection in AIDS patients who are not on HAART.

    • The mode of transmission: inhalation, ingestion, or inoculation via the respiratory or gastrointestinal tract.

  • Symptoms

    • Blurring

    • Floaters

  • Laterality: unilateral, but bilateral can occur

  • Course: subacute

  • Age of onset: any

  • Gender/race: no gender or racial predilection; any exposed individual

  • Systemic association

    • CD4 T cells <50 cells/μL

    • Fever, night sweats, weight loss, diarrhea, anemia, and abdominal pain

Exam: Ocular

Anterior Segment

  • Granulomatous anterior uveitis

  • Iris nodules

Posterior Segment

  • Multifocal choroiditis with panuveitis

  • Unifocal choroidal infiltrate

  • Endophthalmitis

Exam: Systemic

  • Hepatomegaly, splenomegaly, lymphadenopathy, and skin lesion

Laboratory and Radiographic Testing

  • Ocular fluid or tissue

    • Gram, modified Acid fast bacilli (AFB), AFB staining

    • Culture and PCR for mycobacterium

  • CBC, liver function test, chest X-ray

Differential Diagnosis

  • Fungus: candidiasis, cryptococcosis, histoplasmosis, pneumocystosis

  • Syphilis

  • TB

  • Intraocular lymphoma

Treatment

  • At least two antimycobacterial drugs to prevent or delay the emergence of resistance

    • Azithromycin (500 mg daily) or clarithromycin (500 mg PO twice daily) and ethambutol (15 mg/kg PO daily)

  • Alternative therapy: rifabutin (300 mg PO daily), levofloxacin (500 mg PO daily), moxifloxacin (400 mg PO daily), amikacin, streptomycin

  • Followed by secondary prophylaxis until immune reconstitution (CD4 >100 cells/μL) and disease controlled

Referral/Co-management

  • Infectious disease