CHOIR (childhood ocular inflammatory disease research): improving the understanding and management of sight threatening childhood onset ocular inflammation

Childhood ocular inflammatory disorders (ChOIDs: uveitis, scleritis, orbital inflammation and optic neuritis) are rare, complex, immune-mediated disorders with significant negative impact. ChOIDs are growing in incidence and complexity, a pattern seen in other (often associated) immune-mediated inflammatory disorders. Stubborn evidence gaps around disease distribution, determinants and endophenotypes, and around natural history and child or treatment specific predictors of poor outcome remain. Through a national childhood ocular inflammatory disease registry, and standardisation of routinely collected clinical data, supported by the national clinical and health informatic networks I have established, I will undertake the observational research needed to report disease distribution, natural history and determinants of outcome. I will also - adapt the novel imaging approaches I have developed (already shown to result in repeatable, sensitive and responsive metrics for disease state) - and use patient reported disease activity scores (with a novel tool I have developed) to interrogate correlations between these metrics and disease type (by therapeutic response or presence of multi-system immune mediated disease) and prediction of disease outcomes (e.g. successful withdrawal of systemic immunomodulatory therapy). In so doing, I will undertake the rare disease population discovery science needed to transform the understanding and management of these disorders.