UNICORNS: Uveitis in Childhood Prospective National Cohort Study

Background:

Childhood uveitis descriptive term for a group of rare inflammatory eye disease, which are typically chronic, relapsing-remitting in nature, and of uncertain aetiology (idiopathic). Visual loss occurs due to structural damage caused by uncontrolled inflammation. Understanding of the determinants of long-term outcome is limited, particularly the predictors of therapeutic response or how to define disease control.

Aims:

To describe disease natural history and outcomes amongst a nationally representative group of children with non-infectious uveitis, describe the impact of disease course on quality of life for both child and family, and identify determinants of adverse visual, structural and developmental outcomes.

Methods:

UNICORNS is a prospective longitudinal multicentre cohort study of children newly diagnosed with uveitis about whom a core minimum clinical dataset will be collected. Participants and their families will also complete patient-reported outcome measures (PROMS) at study entry and annually from recruitment.

PROMS collected at baseline are:

• Strength and Difficulties Questionnaire (SDQ): this is to screen the emotional and behavioural aspects of the participants' lives
• Child Health Utility 9D (CHU9D): generic preference based measure of health related quality of life
• Children's Sleep Habits Questionnaire (CHSQ): this evaluates incidence of behaviours linked with typical paediatric sleep difficulties
• 100-mm General Evaluation (GE): visual analogue score 100mm long used to capture overall perception of the burden of disease

PROMS collected at year 1 and annually (subject to investigations of instrument redundancy) are:

• Child Health Utility 9D (CHU9D): generic preference based measure of health related quality of life
• 100-mm General Evaluation (GE): visual analogue score 100mm long used to capture overall perception of the burden of disease
• Paediatric Quality of life Score (PedsQL): a brief questionnaire used to evaluate health related quality of life in children
• Vision related quality of life metric - Children and Young People (VQoL_CYP): these instruments captures the functional and broader impacts of living with a visual disability The association of patient (child- and treatment- dependent) characteristics with outcome will be investigated using logistic and ordinal regression models which incorporate adjustment for within-child correspondence between eyes for those with bilateral disease and repeated outcomes measurement.

Discussion:

Through this population based, prospective longitudinal study of childhood uveitis, the investigators will describe the characteristics of childhood onset disease. Early (1-2 years following diagnosis) outcomes will be described in the first instance, and through the creation of a national inception cohort, longer term studies of outcome for affected children and families will be enabled.

Inclusion criteria

• Children newly diagnosed with uveitis (within previous 6 months)
• Age < 18years

Exclusion criteria

• Uveitis due to malignancy
• Uveitis due to ocular trauma (including iatrogenic, ie intraocular surgery)
• Uveitis due to confirmed ocular infection
• Children with developmental disorders or impairments which prevent their self-report of their function level or quality of life