A 16-year-old boy presents with sudden onset of a red right eye, severe eye pain, tearing, photophobia, and decreased vision. He denies eye trauma. His review of systems is positive for knee and ankle pain over the previous 6 months. On examination, he has a ciliary flush (Figure 13-1) and decreased vision. He is referred to an ophthalmologist who confirms the diagnosis of acute anterior uveitis. He is also referred to a pediatric rheumatologist who makes the diagnosis of juvenile onset spondyloarthritis. His uveitis is treated with topical steroids.

Uveitis is inflammation of any component of the uveal tract: iris (anterior), ciliary body (intermediate), or choroid (posterior). Most uveitis is anterior and is also called iritis. Uveitis is caused by trauma, inflammation, or infection and the most common etiologies vary by location in the uveal tract. Patients present with vision changes and, if uveitis is anterior, eye pain, redness, tearing, and photophobia. All patients with uveitis should be referred to an ophthalmologist.

Anterior uveitis includes iritis and iridocyclitis. Iritis is when the inflammation is limited to the iris. If the ciliary body is involved too, then it is called iridocyclitis. Posterior uveitis includes choroiditis and chorioretinitis.

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  Annual incidence of uveitis is 17 to 52 per 100,000 population and prevalence is 38 to 714 per 100,000 population.¹

  
  
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  Occurs at any age, but most commonly between 20 and 59 years.¹

  
  
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  Anterior uveitis (iritis) accounts for approximately 90 percent of uveitis as seen in primary care settings.¹

  
  
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  Eighty percent of uveitis cases seen in children are caused by juvenile idiopathic arthritis.²

  
  
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  In the US, noninfectious uveitis accounts for 10 percent of legal blindness.³

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  Uveitis in children is predominantly associated with juvenile idiopathic arthritis, but can also be caused by trauma, infections, inflammation, or, rarely, neoplasms. Most likely causes differ by location.⁴

  
  
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  Iritis—Autoimmune disorders, especially juvenile idiopathic arthritis, and trauma are more common (Figure 13-2). Infection, malignancy and idiopathic causes are less common. Infections include herpes, syphilis, and tuberculosis.⁴

  
  
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  Intermediate—Most are idiopathic⁴ (Figure 13-3).

  
  
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  Posterior—In infants, congenital infections (toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus, and syphilis) are common causes. In toddlers, Toxocara canis or Toxocara cati acquired from ingestion of contaminated soil can cause a unilateral posterior uveitis. Cytomegalovirus is the most common in immunocompromised children. It may also be autoimmune, trauma, malignancy or idiopathic.

  
  
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  Panuveitis (affecting all layers)—Idiopathic (22% to 45%) and sarcoidosis (14% to 28%).⁴ Unilateral panuveitis is often endophthalmitis (endogenous or related to trauma or surgery). Bilateral panuveitis can be caused by sarcoidosis or syphilis.