Overview of JIA-associated uveitis

Smith, 2021 Standardization of Uveitis Nomenclature (SUN) Working Group, 2021

Uveitis is an uncommon disease characterised by inflammation of the uvea. It is associated with significant morbidity, including visual impairment and blindness. Complications, such as glaucoma, macular oedema, and cataracts, are also common; however, treatment significantly reduces their likelihood.

In children and adolescents, juvenile idiopathic arthritis (JIA) is the cause of uveitis in approximately half of all cases, while another third have no identifiable causeCann, 2018. The risk of developing JIA-associated uveitis is highest in antinuclear antibody (ANA)-positive children who developed JIA disease at 6 years or younger. Given the strong association of uveitis with JIA, children with uveitis should be carefully evaluated for the presence of occult inflammatory joint disease.

Uveitis can also be caused by infection (eg herpes simplex keratitis), hypersensitivity reactions, or a range of systemic inflammatory disorders including Behçet disease, sarcoidosis, inflammatory bowel disease, and tubulointerstitial nephritis.

Chronic anterior uveitis is a term used to describe asymptomatic anterior uveitis, the most common form of JIA-associated uveitis. It is typically insidious and minimally symptomatic. If prominent symptoms, such as redness or ocular pain are present, the uveitis is usually termed ‘acute’ or ‘symptomatic’. Features, such as reduced visual acuity and photophobia, may occur in chronic anterior uveitis but are not reliably recognised by younger children. Older children may be able to recognise these features and, along with their parents, should be instructed to report them. Left undetected or undertreated, anterior uveitis is potentially blinding; regular slit-lamp screening examinations by an ophthalmologist or, in older children, an optometrist, are essential for all children and adolescents at-risk of asymptomatic anterior uveitis. For identification of at-risk groups and the recommended frequency of screening, see Screening for asymptomatic JIA-associated anterior uveitis.

Note: Regular slit-lamp screening examinations are essential for all children and adolescents at-risk of asymptomatic anterior uveitis.

Uveitis should be diagnosed and managed by an ophthalmologist with experience in this condition. Urgent treatment is required to avoid irreversible damage.

Note: Refer patients with suspected JIA-associated uveitis urgently to an ophthalmologist to prevent irreversible ocular damage.

Disease activity is measured and documented using the Standardised Uveitis NomenclatureJabs, 2005.