UK NRC request for input – Juvenile onset chronic anterior uveitis

Dear colleagues,

The UK National Release Centre (UK NRC) has received a request for a new SNOMED CT concept: “Juvenile onset chronic anterior uveitis (disorder)”. They have asked for Eye Care CRG input on whether this represents a distinct clinical entity that warrants addition.

The requester’s rationale is that onset before age 16 represents a distinct phenotype with different treatment approaches and outcomes.

Existing concepts such as 398155003 |Chronic anterior uveitis (disorder)| and 1217647006 |Idiopathic chronic anterior uveitis (disorder)| do not capture age at onset.

They have also noted that the following concepts represent only a minority of childhood anterior uveitis cases:

  • 1231259004 |Uveitis due to juvenile idiopathic arthritis (disorder)|

  • 416666007 |Anterior uveitis due to juvenile idiopathic arthritis (disorder)|

They add that approximately a quarter to half of children with idiopathic uveitis will also be diagnosed with Juvenile Idiopathic Arthritis. However, for the majority of children, the eye disease occurs in isolation.

Could you please review this and advise whether you consider “Juvenile onset chronic anterior uveitis” to be a distinct disorder that warrants a new SNOMED CT concept?

References provided:

  1. https://doi.org/10.1038/s41584-024-01181-x

  2. https://pmc.ncbi.nlm.nih.gov/articles/PMC12320610/

  3. https://pmc.ncbi.nlm.nih.gov/articles/PMC1771761/

  4. https://pmc.ncbi.nlm.nih.gov/articles/PMC4556262/

  5. https://pmc.ncbi.nlm.nih.gov/articles/PMC3536914/

  6. https://pmc.ncbi.nlm.nih.gov/articles/PMC2937251/

For context, the request has come from a paediatric ophthalmology consultant specialising in uveitis, with an academic and clinical role across UCL GOS Institute of Child Health, Great Ormond Street Hospital, and Moorfields Eye Hospital. They lead the UK multicentre childhood uveitis inception cohort study (UNICORNS), established the UK Paediatric Ocular Inflammation Group, and have contributed to national work including the NCEPOD review into JIA care, as well as international collaborations (e.g. OHDSI paediatric ophthalmology and uveitis groups).

Many thanks

Elaine

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