Pediatric Uveitis

Pediatric Uveitis

Grace T. Liu

Alex V. Levin


Uveitis in the pediatric population is a significant cause of ophthalmic morbidity. Approximately 2% to 14% of patients seen in uveitis clinics are children (1,2,3). Unique to the pediatric age group in the management and timely diagnosis of uveitis is the threat of amblyopia. In addition, the associated systemic disease profile is much different than that seen in adults. Children more often (71%) have an associated systemic illness than adults (55%) (4). Although juvenile idiopathic arthritis (JIA) is the predominant cause of anterior uveitis in children (5,6), it is important to recognize that uveitis in a child can be due to a wide range of etiologies, including serious life-threatening masquerade syndromes such as retinoblastoma and leukemia. Presenting signs and symptoms are often not recognized until advanced stages, and the disorder may even be entirely asymptomatic until irreversible ocular damage has been sustained (Fig. 13.1). The child may be unable to verbalize his/her symptoms, and can often function normally with visual acuity well below 20/20 for activities of daily living, especially when the disease is unilateral and the child is younger. The approach to pediatric uveitis requires the understanding that early recognition through screening, where appropriate, can be of utmost importance.

Table 13.1 summarizes the diagnostic approach to the child with pediatric uveitis.


The frequency and etiology of childhood uveitis is in part dependent on geography. Widespread globalization may also affect the current distribution of the disease. A meta-analysis of worldwide studies showed that 7% of patients with uveitis are children. Parasitic anterior uveitis (49.3%) is the most common etiology globally, with idiopathic being the second most common (25.5%) (7). A group from Saudi Arabia reported idiopathic anterior non-granulomatous uveitis as the most common type of uveitis in children (26%) (8). A report from Israel found infectious diseases to be the primary etiology of uveitis in children and adolescents (31.2%) (1).

To the contrary, a study from the US National Eye Institute found that idiopathic uveitis (28.8%) was the leading etiology in the United States, followed by JIA (20.9%), and pars planitis (17.1%). A retrospective study characterizing disease characteristics and visual outcome of 527 children in the United States with uveitis, found that 54% were female; 62% White, 15% Hispanic, 12% Black, 3% Asian, and 2% multiracial (9). The median age at diagnosis was 9.4 years.


Although consortium-driven classification systems have been proposed, they may be difficult to use in the clinical setting. The Standardization of Uveitis Nomenclature (SUN) criteria were developed for classification and description of uveitis by anatomic location (Table 13.2). Specific grading criteria, such as quantitative grading of inflammation, were also elaborated (10). Morphologic classification, according to cell type, for example granulomatous versus nongranulomatous, may be less useful in children. A broader classification might separate etiologies into exogenous, representing any external injury or invasion of microorganisms from outside the globe, versus endogenous, resulting from factors that originate within the patient.


Juvenile Idiopathic Arthritis

Table 13.3 provides a differential diagnosis for pediatric anterior uveitis.

This term encompasses all idiopathic childhood arthritis in children younger than 16 years old, persisting for more than 6 weeks, as defined by the International League of Associations of Rheumatology (ILAR) (46). This classification defines seven categories of JIA.

Systemic Juvenile Idiopathic Arthritis

Systemic-onset JIA (sJIA, Still’s disease) constitutes 10% to 20% of JIA, yet accounts for approximately two-thirds of mortality associated with JIA (47,48). Morbidity and mortality is often secondary to destructive arthritis, secondary amyloidosis, and other treatment complications, including infection and osteoporosis. sJIA is characterized by arthritis, daily spiking fever, evanescent rash, serositis, and a variety of other extraarticular features (Still GF). Males and females are affected equally. Patients are both antinuclear antibody (ANA) and rheumatoid factor (RF) negative. Because of the infrequency of uveitis,
screening by ophthalmologists is advised only yearly (49,50). Acquired tenosynovitis of the superior oblique tendon (Brown syndrome) has been described (51).


Juvenile idiopathic arthritis

Juvenile spondyloarthropathies

Juvenile ankylosing spondylitis

Juvenile psoriatic arthritis

Juvenile reactive arthritis


Blau syndrome

Tubulointerstitial nephritis and uveitis

Kawasaki disease

Inflammatory bowel disease related (ulcerative colitis, Crohn’s disease)

Autoinflammatory syndromes: periodic fevers, cryoprin-associated periodic syndromes






Oligoarthritis, also known as pauciarticular arthritis, involves no more than 1 to 4 joints in the first 6 months of disease onset. It is characterized by asymmetric arthritis, early onset (before age 6), female predilection, positive ANA, and high risk of iridocyclitis (52). Oligoarthritis is further subdivided into persistent, affecting four joints or less throughout the disease course and extended, affecting more than 4 joints after the first 6 months (46). Boys are affected more commonly in the late-onset pauciarticular JIA. These patients are both ANA and RF negative, and approximately 75% of boys with this subtype are HLA-B27 positive.

Chronic iridocyclitis is seen in up to 50% of patients (53). Ophthalmologic screening is recommended every 3 months in this group for a minimum of 5 years after JIA diagnosis (50).


Polyarticular JIA affects five or more joints during the first 6 months of disease. It may be characterized by low-grade fever, anemia, and malaise within the first 3 months. Girls are more affected than boys. There are two subtypes: RF positive and RF negative. The former seldom develop uveitis and tend to develop arthritis in late childhood and adolescence. Definitive diagnosis of RF-positive polyarthritis must be confirmed on two separate occasions at least 3 months apart (46). Between 7% and 15% of patients with JIA with uveitis have polyarticular onset (54). Screening is recommended every 6 months (50).

Enthesis-Related Arthritis

Enthesis-related JIA is characterized by chronic arthritis associated with inflammation of muscle and tendon attachment to bone as well as arthritis of one or more joints. Patients are more often HLA-B27-positive boys, usually in the preadolescent to adolescent age group. Uveitis often presents suddenly, and is symptomatic and unilateral. Extraarticular manifestations can occur, such as gastrointestinal, mucosal, and cutaneous manifestations (55,56,57).

Psoriatic Arthritis

In addition to the presence of psoriasis and arthritis, this diagnosis requires at least two of the following: dactylitis, nail pitting or onycholysis, psoriasis in a first-degree relative. Positive RF is an exclusion criterion (46). Uveitis is usually insidious and chronic anterior, and is seen in 10% of affected children (58).

Undifferentiated Arthritis

This is the last subtype of JIA, which is essentially a diagnosis of exclusion of the other subtypes.


The juvenile spondyloarthropathies (JSpA) are an interrelated group of uveitic entities, in children under 16 years old, associated with systemic disease, that are strongly HLA-B27 positive and usually RF negative. They represent the third most common etiology of anterior uveitis in children (59,60,61).

Juvenile Ankylosing Spondylitis

Over 90% of patients are HLA-B27 positive (62,63). Ocular symptoms are characterized by a recurrent nongranulomatous anterior uveitis. Bilateral uveitis occurs in 80% but usually not simultaneously (64). Severe uveitis can present with hypopyon.

Boys are affected more than girls, and lower extremities are affected, as opposed to lower back involvement in adults. Peripheral arthropathy may present prior to the radiographic finding of sacroiliac joint involvement. Early detection of sacroiliitis may require contrast-enhanced magnetic resonance imaging (65). Stamato et al. reported the occurrence of aortic regurgitation in children with JSpA (66).

Juvenile Psoriatic Arthritis

Girls are affected more frequently. Ocular manifestations include chronic nongranulomatous anterior uveitis. Juvenile psoriatic arthritis (JPsA) is the cause of approximately 3% to 4% of HLA-B27-positive uveitis and accounts for approximately 5% of JIA. Joint disease may be pauciarticular or polyarticular. Systemic features include psoriatic skin changes and nail pitting (46).

Juvenile Reactive Arthritis (Previously Known as Reiter Syndrome)

Reactive arthritis is described as the classic triad of conjunctivitis, arthritis, and urethritis, though more recently, it has been recognized that all three need not necessarily occur simultaneously (67). Conjunctivitis is the most frequent presenting sign in children. Boys are more commonly affected than girls. Ocular involvement includes nongranulomatous anterior uveitis in approximately 3% to 12% of patients (54). Possible etiologies include prior infection with Salmonella or Shigella enterocolitis.

Jun 20, 2016 | Posted by in OPHTHALMOLOGY | Comments Off on Pediatric Uveitis
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