What Causes Uveitis?

Patient Presentation
A 13-year-old male came to clinic with a 2 day history of blurred vision. He noticed the problem in the evening but thought that it was just because he was tired. The next day it continued and he told his parents that he also had some “black spots” that seemed to move around. He denied pain, photophobia or any visual field cuts or narrowing of the visual fields. His mother said that initially she noticed that his left eye was slightly redder but he had been rubbing it trying to relieve the symptoms. She said that it was now normal after he stopped rubbing the eye. He had been well and denied any eye, head or neck trauma. He had been born in the United States and there was no travel outside the United States nor contact with people who had been visiting overseas. He drank city water and had no animal exposures except for a guinea pig at a friend’s house but he had not touched the animal in several months. The past medical history was positive for a left tibial toddler’s fracture at age 3. The review of systems was negative for fevers, chills, nausea, emesis, diarrhea, dysuria, cough, rhinorrhea, edema, weight loss or gain, skin, hair or nail changes or phonophobia. There was also no arthralgia, myalgia, warmth in any joint or difficulty moving.

The pertinent physical exam showed a well-appearing male with normal vital signs and growth parameters around the 75%. He had a normal weight curve since his last visit. His visual acuity was 20/50 in his left eye and 20/30 in his right eye. Pupils were equal, round, and reactive to light. Extra ocular movements were intact. His eye examination showed some photophobia. There was no obvious leukocoria or hypopyon noted. Disc margins appeared sharp but vessels were difficult to evaluate because he kept closing his eyes. His HEENT was negative including thyroid masses. His skin, hair and joint examinations were normal. The rest of his examination was normal.

The diagnosis of acute onset of visual changes including decreased acuity and “floaters” with an otherwise negative history and physical examination was made. The pediatrician referred the teenager to pediatric ophthalmology the following day who diagnosed him with par planitis after various ophthalmological testing which showed snowbanks and snowballs in the vitreous bilaterally.

The laboratory evaluation included a chest radiograph, and blood and urine testing for immune diseases and infectious diseases. Quantiferon gold was performed for possible tuberulosis. All testing was eventually negative and the patient was started on oral steroids. The patient was to followup in 1 month with the ophthalmologist, but the pediatrician contacted the family by phone after 2 weeks and the patient said that he had less floaters and blurred vision subjectively.

Inflammation of the middle layer of the eye, or uvea, is termed uveitis. Uveitis can be divided into anterior, intermediate or posterior uveitis by involving the anterior (iris and ciliary body), intermediate (vitreous) or posterior (choroid and usually retina) compartments. Panuveitis involves all 3 compartments. Duration can also be used to classify uveitis. Acute is 6 weeks and > 3 months is chronic persistent uveitis. Episodic periods of inactivity and reactivity that last more than 3 months are called recurrent uveitis. A third way to characterize uveitis is if it is granulomatous or not. Uveitis increases with age with the highest incidence and prevalence in > 65 year olds. Only ~10% of uveitis occurs in the pediatric age group. Uveitis can be difficult to identify in younger children as they cannot as readily communicate their visual problems. Younger children unfortunately seem to have worse long-term outcomes too.

Pars planitis is a non-granulomatous, chronic, intermediate uveitis of unknown cause (subvariation of idiopathic), but felt to be immune-mediated with a linkage to HLA-DR2 and HLA-DR15. It occurs in children and teens and is possibly more common in males but studies disagree. Patients can be asymptomatic or often have an insidious onset of blurred or decreased vision or floaters. Other less common symptoms are pain, redeye or photophobia. Young children can have strabismus or leukocoria. The eyes are often asymmetrically involved but usually there is some uveitis present in both eyes. Pars planitis is characterized by snowball formation or inferior snowbanking in the vitreous. Potential complications of pars planitis include: cataracts, posterior synechiae, cystoid macular edema (most common cause of visual morbidity), optic disc edema, glaucoma, vitreous hemorrhage, retinoschisis or rarely retinal detachment. Despite these potential complications prognosis is usually good.

Treatment of uveitis tries to balance arresting the inflammation with the potential side effects of the medication. Steroids are often used topically, orally or periocularly injected. Immunosuppressive agents such as methotrexate or biological agents such as adalimamab or infliximab have been used. Complications may need surgical intervention. The outcome of uveitis depends on the diagnosis, treatment and complications encountered by an individual patient.

Learning Point
The workup for uveitis includes evaluation for infectious, immune and malignancies etiologies before any treatment is begun and includes many of these entities below.

Causes of uveitis include:

  • Immune
    • *Idiopathic
    • *Juvenile idiopathic arthritis (JIA)
    • Behçet’s disease
    • Blau syndrome
    • Cogan syndrome
    • Inflammatory bowel disease
    • Multiple sclerosis
    • *Pars Planitis
    • Sarcoidosis
    • Sympathetic opthalmia
    • Tubulointerstitial nephritis-uveitis (TINU) syndrome
    • Vogt-Koyanagi-Harada syndrome
  • Infectious
    • *Viral
      • Cytomegalovirus
      • Epstein Barr virus
      • Herpes simplex virus
      • Rubella
      • Varicella
    • Parasitic
      • *Toxoplasma
      • Ascaris
      • Toxocara
    • Bacteria
      • Tuberculosis
      • Bartonella
      • Borrelia burgdorferi
      • Treponema pallidum
  • Mimicks Uveitis
    • Leukemia
    • Retinoblastoma

* In a study of 527 patients in the US, the most common cause for anterior uveitis was JIA, intermediate uveitis was par planitis and posterior uveitis was toxoplasmosis.

Questions for Further Discussion
1. What is the role of a pediatrician with an uveitis patient?
2. Besides opthalmology, what other specialties may have a role in the care of an uveitis patient?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Eye Diseases.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

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Ozdal PC, Berker N, Tugal-Tutkun I. Pars Planitis: Epidemiology, Clinical Characteristics, Management and Visual Prognosis. J Ophthalmic Vis Res. 2015 Oct-Dec;10(4):469-80.

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Dajee KP, Rossen JL, Bratton ML, Whitson JT, He YG. A 10-year review of pediatric uveitis at a Hispanic-dominated tertiary pediatric ophthalmic clinic. Clin Ophthalmol. 2016 Aug 22;10:1607-12.

Shoughy SS, Kozak I, Tabbara KF. Associations of Systemic Diseases with Intermediate Uveitis. Ophthalmic Epidemiol. 2016;23(1):27-31.

Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital