Uveitis

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Basics

Description

  • A nonspecific term used to describe any intraocular inflammatory disorder
  • The uvea is the middle layer of the eye between the sclera and retina. The anterior part of the uvea includes the iris and ciliary body. The posterior part of the uvea is the choroid.
    • Anterior uveitis: refers to ocular inflammation limited to the iris (iritis) alone or iris and ciliary body (iridocyclitis)
    • Intermediate uveitis: refers to inflammation of the structures just posterior to the lens (pars planitis or peripheral uveitis)
    • Posterior uveitis: refers to inflammation of the choroid (choroiditis), retina (retinitis), or vitreous near the optic nerve and macula
    • Panuveitis: refers to inflammation of all of the above areas of the uvea
  • System(s) affected: nervous
  • Synonym(s): iritis; iridocyclitis; choroiditis; retinochoroiditis; chorioretinitis; anterior uveitis; posterior uveitis; pars planitis; panuveitis. Synonyms are anatomic descriptions of the focus of the uveal inflammation.

Pediatric Considerations

  • Infection should be the primary consideration.
  • Allergies and psychological factors (depression, stress) may serve as a trigger.
  • Trauma is also a common cause in this population.

Epidemiology

Predominant sex: male = female, except for human leukocyte antigen B27 (HLA-B27) anterior uveitis: male > female, autoimmune etiology: female > male

Incidence
Overall incidence is 17 to 52 cases/100,000 per year in the developed world.

Prevalence

  • Overall prevalence is 38 to 714 cases/100,000 annual incidence.
  • Anterior uveitis is the most common (80% of cases) with iritis being 4 times more prevalent than posterior uveitis.

Etiology and Pathophysiology

  • Infectious: may result from viral, bacterial, parasitic, or fungal etiologies
  • Suspected immune mediated: possible autoimmune or immune complex–mediated mechanism postulated in association with systemic (especially rheumatologic) disorders
    • Autoimmune uveitis (AIU) patients should be referred to an ophthalmologist for local treatment.
  • Isolated eye disease
  • Undifferentiated (~25%)
  • Some medications may cause uveitis. The most causative medications include rifabutin, bisphosphonates, sulfonamides, metipranolol, brimonidine, prostaglandin analogs, immune checkpoint inhibitors, TNF-α inhibitors, protein kinase inhibitors (BRAF and MEK), anti–vascular endothelial growth factor (anti-VEGF) agents, bacillus Calmette-Guérin (BCG) vaccination, and systemic and intraocular cidofovir.
  • Masquerade syndromes: diseases such as malignancies that may be mistaken for primary inflammation of the eye

Genetics

  • Iritis: 50–70% are HLA-B27 positive.
  • Predisposing gene for posterior uveitis associated with Behçet disease may include HLA-B51.

Risk Factors

No specific risk factors

Commonly Associated Conditions

  • Viral infections: herpes simplex, herpes zoster, HIV, cytomegalovirus, congenital Zika virus
  • Bacterial infections: brucellosis, leprosy, leptospirosis, Lyme disease, propionibacterium infection, syphilis, tuberculosis (TB), Whipple disease
  • Parasitic infections: acanthamebiasis, cysticercosis, onchocerciasis, toxocariasis, toxoplasmosis
  • Fungal infections: aspergillosis, blastomycosis, candidiasis, coccidioidomycosis, cryptococcosis, histoplasmosis, sporotrichosis
  • Suspected immune mediated: ankylosing spondylitis, Behçet disease, Crohn disease, drug or hypersensitivity reaction, interstitial nephritis, juvenile rheumatoid arthritis, Kawasaki disease, multiple sclerosis, psoriatic arthritis, Reiter syndrome, relapsing polychondritis, sarcoidosis, Sjögren syndrome, systemic lupus erythematosus, ulcerative colitis, vasculitis, vitiligo, Vogt-Koyanagi (Harada) syndrome
  • Isolated eye disease: acute multifocal placoid pigmentary epitheliopathy, acute retinal necrosis, birdshot choroidopathy, Fuchs heterochromic cyclitis, glaucomatocyclitic crisis, lens-induced uveitis, multifocal choroiditis, pars planitis, serpiginous choroiditis, sympathetic ophthalmia, trauma
  • Masquerade syndromes: leukemia, lymphoma, retinitis pigmentosa, retinoblastoma

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Basics

Description

  • A nonspecific term used to describe any intraocular inflammatory disorder
  • The uvea is the middle layer of the eye between the sclera and retina. The anterior part of the uvea includes the iris and ciliary body. The posterior part of the uvea is the choroid.
    • Anterior uveitis: refers to ocular inflammation limited to the iris (iritis) alone or iris and ciliary body (iridocyclitis)
    • Intermediate uveitis: refers to inflammation of the structures just posterior to the lens (pars planitis or peripheral uveitis)
    • Posterior uveitis: refers to inflammation of the choroid (choroiditis), retina (retinitis), or vitreous near the optic nerve and macula
    • Panuveitis: refers to inflammation of all of the above areas of the uvea
  • System(s) affected: nervous
  • Synonym(s): iritis; iridocyclitis; choroiditis; retinochoroiditis; chorioretinitis; anterior uveitis; posterior uveitis; pars planitis; panuveitis. Synonyms are anatomic descriptions of the focus of the uveal inflammation.

Pediatric Considerations

  • Infection should be the primary consideration.
  • Allergies and psychological factors (depression, stress) may serve as a trigger.
  • Trauma is also a common cause in this population.

Epidemiology

Predominant sex: male = female, except for human leukocyte antigen B27 (HLA-B27) anterior uveitis: male > female, autoimmune etiology: female > male

Incidence
Overall incidence is 17 to 52 cases/100,000 per year in the developed world.

Prevalence

  • Overall prevalence is 38 to 714 cases/100,000 annual incidence.
  • Anterior uveitis is the most common (80% of cases) with iritis being 4 times more prevalent than posterior uveitis.

Etiology and Pathophysiology

  • Infectious: may result from viral, bacterial, parasitic, or fungal etiologies
  • Suspected immune mediated: possible autoimmune or immune complex–mediated mechanism postulated in association with systemic (especially rheumatologic) disorders
    • Autoimmune uveitis (AIU) patients should be referred to an ophthalmologist for local treatment.
  • Isolated eye disease
  • Undifferentiated (~25%)
  • Some medications may cause uveitis. The most causative medications include rifabutin, bisphosphonates, sulfonamides, metipranolol, brimonidine, prostaglandin analogs, immune checkpoint inhibitors, TNF-α inhibitors, protein kinase inhibitors (BRAF and MEK), anti–vascular endothelial growth factor (anti-VEGF) agents, bacillus Calmette-Guérin (BCG) vaccination, and systemic and intraocular cidofovir.
  • Masquerade syndromes: diseases such as malignancies that may be mistaken for primary inflammation of the eye

Genetics

  • Iritis: 50–70% are HLA-B27 positive.
  • Predisposing gene for posterior uveitis associated with Behçet disease may include HLA-B51.

Risk Factors

No specific risk factors

Commonly Associated Conditions

  • Viral infections: herpes simplex, herpes zoster, HIV, cytomegalovirus, congenital Zika virus
  • Bacterial infections: brucellosis, leprosy, leptospirosis, Lyme disease, propionibacterium infection, syphilis, tuberculosis (TB), Whipple disease
  • Parasitic infections: acanthamebiasis, cysticercosis, onchocerciasis, toxocariasis, toxoplasmosis
  • Fungal infections: aspergillosis, blastomycosis, candidiasis, coccidioidomycosis, cryptococcosis, histoplasmosis, sporotrichosis
  • Suspected immune mediated: ankylosing spondylitis, Behçet disease, Crohn disease, drug or hypersensitivity reaction, interstitial nephritis, juvenile rheumatoid arthritis, Kawasaki disease, multiple sclerosis, psoriatic arthritis, Reiter syndrome, relapsing polychondritis, sarcoidosis, Sjögren syndrome, systemic lupus erythematosus, ulcerative colitis, vasculitis, vitiligo, Vogt-Koyanagi (Harada) syndrome
  • Isolated eye disease: acute multifocal placoid pigmentary epitheliopathy, acute retinal necrosis, birdshot choroidopathy, Fuchs heterochromic cyclitis, glaucomatocyclitic crisis, lens-induced uveitis, multifocal choroiditis, pars planitis, serpiginous choroiditis, sympathetic ophthalmia, trauma
  • Masquerade syndromes: leukemia, lymphoma, retinitis pigmentosa, retinoblastoma

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