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?
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).
Sorry for the late reply,
I can definitely see the need to distinguish juvenile onset from onset later in life in cases where there is no association with JIA. Especially so as patients age, and distinguishing whether the onset was already in childhood remains relevant.
If precoordinating “juvenile onset” with “chronic anterior uveitis” is the way that distinction would typically work to align with SNOMED conventions than i would support that.
However, I would caution that we are gaining speed on the slippery slope of precoordination, and it may be worth asking:
Why does SNOMED does not have codes for “juvenile onset”, “adult onset” etc as a distinct clinical ideas to support coordination within local information models?
Currently SNOMED seems to represent onset via an “Occurence” Attribute like here.
Just Imho onset and occurence are not the same thing…
English is my second language, so I am unsure: would “juvenile onset” typically be taken to subsume “infantile onset”? Thankfully this is rare in uveitis and perhaps it does not have to be distinguished… “Childhood-onset” may be more aligned with the line being drawn at <16 years?
What about precoordinating with “adult onset”?
If Juvenile onset chronic anterior uveitis is worth adding I suppose the same would apply to Juvenile onset acute anterior anterior uveitis. That may actually be reasonable to add?
What about juvenile-onset uveitis at locations other than the anterior segment?
Existing codes such as 231259004 |Uveitis due to juvenile idiopathic arthritis (disorder)| attempt to precoordinate causality. What if someone has JIA-associated juvenile onset chronic anterior uveitis? Would they have to decide between the code that reflects the cause and the code that reflects the age of onset? Or would this lead to another layer of pre-coordination of “Juvenile onset chronic anterior uveitis due to juveinle idiopathic arthritis”?
Previously, attempts have been made to also precoordinate such codes with laterality (Right eye…)
Please understand that I think the colleague’s request seems perfectly appropriate as the distinction of age of onset is clinically imporant and reconized. I would support the code being added if you think it is OK from an editorial perspective.
But at the same time I worry about the long-term sustainability of pre-coordinating a disease, its location, its laterality, its acuteness/chronicity, its presumed cause and its age of onset…
An alternative I might suggest to the colleague making the request would be extend their information model by an element of “Period of life at onset”, which could be covered by these codes. But I don’t know how that would fit into their use case.