Paeds · ophthalmology
Uveitis in children
Also known as Iritis · Iridocyclitis · Anterior uveitis · Juvenile idiopathic arthritis uveitis · JIA-associated uveitis · Intermediate uveitis · Pars planitis · Ocular toxoplasmosis · Ocular toxocariasis
Fellowship topic on uveitis in children: the SUN anatomical classification of anterior, intermediate, posterior and panuveitis; the chronic silent anterior uveitis associated with juvenile idiopathic arthritis that is detected only by slit-lamp screening; the T-cell mediated autoimmune breakdown of the blood-aqueous barrier producing cells and flare; the 2019 ACR and Arthritis Foundation risk-stratified screening schedule based on JIA subtype, ANA status, age at onset and disease duration; the stepwise treatment from topical prednisolone acetate one percent and cycloplegia through systemic methotrexate to biologic anti-TNF adalimumab anchored by the SYCAMORE trial; the sight-threatening complications of band keratopathy, cataract, glaucoma, posterior synechiae and amblyopia; and the differential of non-infectious versus infectious uveitis including ocular toxoplasmosis and toxocariasis and TINU syndrome.
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Red flags
- A child with juvenile idiopathic arthritis and a normal-looking eye can still have active chronic anterior uveitis — the disease is silent and detected only by slit-lamp screening, so never omit or delay surveillance slit-lamp examination in a child with JIA
- A small, irregular or poorly reactive pupil in a child with JIA suggests posterior synechiae from undiagnosed or inadequately treated chronic uveitis — urgent ophthalmology referral
- A white or grey plaque across the corneal surface in a child with JIA is band keratopathy from chronic anterior chamber inflammation — this is a late complication that signals months to years of undetected disease
- A red painful eye with photophobia in an adolescent may be acute anterior uveitis from HLA-B27-associated spondyloarthritis or inflammatory bowel disease — examine with slit-lamp and fluorescein before any topical steroid, because herpes simplex keratitis mimics this and is worsened by steroid
- A child presenting with visual impairment, leukocoria, squinting or behavioural visual change who has uveitis may already have cataract, glaucoma or amblyopia — ophthalmological emergency even if the eye is white and painless
Life stages
Care settings
Clinical exam formats
Board mappings
- General Paediatrics and Ophthalmology: uveitis in children — the silent chronic anterior uveitis of juvenile idiopathic arthritis, slit-lamp screening and the topical-to-biologic treatment ladder
- Rheumatology: the JIA-associated uveitis screening schedule by risk group, methotrexate and adalimumab systemic immunosuppression, and the joint paediatric-rheumatology and ophthalmology care model
- Renewed curriculum — Ophthalmology and rheumatology: the SUN anatomical classification, the 2019 ACR and Arthritis Foundation risk-stratified screening guideline, and the differential of non-infectious versus infectious uveitis
- Immunology and infectious diseases: T-cell mediated autoimmunity, ocular toxoplasmosis and toxocariasis, and TINU syndrome
- General Paediatrics: diagnosis and stepwise management of childhood uveitis, the JIA screening paradigm, and the red eye that is uveitis not conjunctivitis
- Systems of care: the coordination of paediatric, rheumatology and ophthalmology services for chronic uveitis surveillance and immunosuppression
- Long Case and Structured discussion: a child with juvenile idiopathic arthritis and uveitis — the screening schedule, the topical-to-biologic treatment ladder, the complications, and the transition to adult care
- Communication station: explaining to a family why a child with a normal-looking eye needs regular slit-lamp screening and immunosuppressive medication, and the safety-net for visual symptoms
- Level 2 and 3 — Ophthalmology and rheumatology: uveitis in childhood, the JIA association, the slit-lamp screening paradigm and the sight-threatening complications
- Chronic disease and long-term conditions: the joint care model, medication adherence, and the transition planning for chronic uveitis
- Foundation of Practice (FOP): the classification of uveitis, the silent presentation of JIA uveitis, and the infectious versus non-infectious differential
- Applied Knowledge in Practice (AKP): the risk-stratified screening schedule, the topical-to-biologic treatment ladder, and the complications of steroid therapy
- History-taking and management: the child with juvenile idiopathic arthritis and uveitis surveillance, and the acute red eye with photophobia
- Communication: explaining the rationale for slit-lamp screening and immunosuppression to a family, and the safety-net for visual deterioration
- General Pediatrics Content Outline — ophthalmology and rheumatology: uveitis in children, the JIA association, the SUN classification and the screening paradigm
- Rheumatology: the 2019 ACR and Arthritis Foundation risk-stratified uveitis screening guideline and the systemic immunosuppression ladder
- Patient Care: diagnosis and stepwise management of childhood uveitis from topical corticosteroid to biologic therapy, with ophthalmology and rheumatology co-management
- Systems-Based Practice and Population Health: the screening programme for JIA-associated uveitis, the barriers to surveillance in rural and remote settings, and the transition to adult care
- Medical Knowledge: the T-cell mediated autoimmune pathophysiology, the SUN classification, and the SYCAMORE trial evidence for adalimumab
- Medical Expert: uveitis in children — classification, the JIA screening paradigm, the treatment ladder and the complications, including the infectious posterior uveitides
- Health Advocate and Collaborator: screening adherence and equitable access to slit-lamp surveillance for Indigenous and remote-community children, and the joint care model
- Communicator: explaining the silent nature of JIA uveitis, the need for chronic immunosuppression, and the safety-net for visual symptoms
Non-infectious versus infectious childhood uveitis
Non-infectious (autoimmune)
Infectious
Overview & Definition
Picture a four-year-old girl with a swollen right knee for three months. She has oligoarticular juvenile idiopathic arthritis, ANA positive, diagnosed last month. Her eyes look completely normal. She has no eye pain, no redness, no photophobia, no visual complaint — yet her slit-lamp examination reveals grade 2+ cells and 1+ flare in both anterior chambers. This is the paradigm of childhood uveitis: the disease that blinds while the child looks and feels well, and the reason why slit-lamp screening exists. [3] [4]
Uveitis is inflammation of the uveal tract — the iris, the ciliary body and the choroid. In practice the term extends to inflammation of adjacent structures, so anterior uveitis involves the iris and ciliary body, intermediate uveitis involves the vitreous and pars plana, and posterior uveitis involves the retina and choroid. The Standardization of Uveitis Nomenclature (SUN) working group formalised this anatomical classification in 2005 so that disease activity and treatment response could be reported consistently across centres. [1]
Why does this matter for the paediatrician? Because the single most important thing you can do for a child with JIA is to screen for uveitis. The disease is silent, the progression to irreversible structural damage is gradual, and the window for prevention closes once cataract, band keratopathy or amblyopia develop. Every child newly diagnosed with JIA needs a baseline slit-lamp examination and a planned surveillance schedule — this is non-negotiable, and it is the general paediatrician's responsibility to ensure it happens. [7] [3]
References11ShowHide
- [1]Jabs DA; Nussenblatt RB; Rosenbaum JT; Standardization of Uveitis Nomenclature (SUN) Working Group Standardization of uveitis nomenclature for reporting clinical data. Results of the First International Workshop. Am J Ophthalmol, 2005.PMID 16196117
- [2]Heiligenhaus A; Minden K; Föll D; et al Evidence-based, interdisciplinary guidelines for anti-inflammatory treatment of uveitis associated with juvenile idiopathic arthritis. Rheumatol Int, 2012.PMID 22083610
- [3]Heiligenhaus A; Niewerth M; Ganser G; et al Review for disease of the year: epidemiology of juvenile idiopathic arthritis and its associated uveitis. Ocul Immunol Inflamm, 2013.PMID 23713827
- [4]Carvounis PE; Herman DC; Cha S; Burrus J; Moore J; Rosenbaum JT Incidence and outcomes of uveitis in juvenile rheumatoid arthritis, a synthesis of the literature. Graefes Arch Clin Exp Ophthalmol, 2006.PMID 16228217
- [5]Ramanan AV; Dick AD; Jones AP; McKay A; Williamson PR; Compeyrot-Lacassagne S; et al Adalimumab plus Methotrexate for Uveitis in Juvenile Idiopathic Arthritis. N Engl J Med, 2017.PMID 28445659
- [6]Castiblanco C; Foster CS; Di Chrstian M; et al Treatment of pediatric uveitis with adalimumab: the MERSI experience. J AAPOS, 2016.PMID 27079596
- [7]Angeles-Han ST; Yeh S; Vogel LB; Burnham J; Cooper AM; Magliya M; et al 2019 American College of Rheumatology/Arthritis Foundation Guideline for the Screening, Monitoring, and Treatment of Juvenile Idiopathic Arthritis-Associated Uveitis. Arthritis Care Res (Hoboken), 2019.PMID 31021540
- [8]Modrzejewska M; Grzybowski A; Misiuk-Hojło M; et al Uveitis in the Pediatric Population and Therapeutic Management: A Current Literature Review. Children (Basel), 2024.PMID 39062219
- [9]Modrzejewska M; Grzybowski A; Misiuk-Hojło M; et al Diagnosis and Treatment of Uveitis in Children: A Summary of the Latest Data from a 5-Year Literature Review. J Clin Med, 2024.PMID 38892808
- [10]Woodhall D; Starr MC; Montgomery SP; Jones JL; Lum F; Read RW; Moorthy RS Toxocariasis: A Review for Pediatricians. J Pediatric Infect Dis Soc, 2014.PMID 26625368
- [11]Lei B; Weng R; Wang Q; Liu Z; et al Pediatric Uveitis in a Tertiary Referral Center in East China: Clinical Patterns and Visual Outcomes. J Ophthalmol, 2024.PMID 39619646