Background JIA-associated uveitis (JIAU) is a serious, sight-threatening disease with significant long-term complications and risk of blindness, even with improved contemporary treatments. The MIWGUC was set up in order to propose specific JIAU activity and response items and to validate their applicability for clinical outcome studies. Methods The group consists of 8 paediatric rheumatologists and 7 ophthalmologists. A consensus meeting took place on November 2015 in Barcelona (Spain) with the objective of validating the previously proposed measures. The validation process was based on the results of a prospective open, international, multi-centre, cohort study designed to validate the outcome measures proposed by the initial MIWGUC group meeting in 2012. The meeting used the same Delphi and nominal group technique as previously described in the first paper from the MIWGUC group (Arthritis Care Res 64:1365–72, 2012). Patients were included with a diagnosis of JIA, aged less than 18 years, and with active uveitis or an uveitis flare which required treatment with a disease-modifying anti-rheumatic drug. The proposed outcome measures for uveitis were collected by an ophthalmologist and for arthritis by a paediatric rheumatologist. Patient reported outcome measures were also measured. Results A total of 82 patients were enrolled into the validation cohort. Fifty four percent (n = 44) had persistent oligoarthritis followed by rheumatoid factor negative polyarthritis (n = 15, 18%). The mean uveitis disease duration was 3.3 years (SD 3.0). Bilateral eye involvement was reported in 65 (79.3%) patients. The main findings are that the most significant changes, from baseline to 6 months, are found in the AC activity measures of cells and flare. These measures correlate with the presence of pre-existing structural complications and this has implications for the reporting of trials using a single measure as a primary outcome. We also found that visual analogue scales of disease activity showed significant change when reported by the ophthalmologist, rheumatologist and families. The measures formed three relatively distinct groups. The first group of measures comprised uveitis activity, ocular damage and the ophthalmologists’ VAS. The second comprised patient reported outcomes including disruption to school attendance. The third group consisted of the rheumatologists’ VAS and the joint score. Conclusions We propose distinctive and clinically significant measures of disease activity, severity and damage for JIAU. This effort is the initial step for developing a comprehensive outcome measures for JIAU, which incorporates the perspectives of rheumatologists, ophthalmologists, patients and families.

No abstract

Background Understanding how strabismus impacts a child’s quality of life, as well as their families, should be an important key to guide treatment, not only from the ophthalmological point of view but also regarding psychological and social aspects, which are fundamental for a healthy and harmonious development. Methods A cross-sectional study was performed to evaluate the functional vision and eye-related quality of life (ER-QOL) in a population of children with strabismus submitted or not to corrective surgery, using the recently developed Pediatric Eye Questionnaire (PedEyeQ) and to compare with age and gender-matched visually normal children. The PedEyeQ was applied to non-operated children with strabismus (n = 18), operated children with strabismus (n = 24), and visually normal children (n = 21). This instrument is composed of 3 components (Child, Proxy, and Parent) and has different versions according to the child’s age (0–4, 5–11, or 12–17 years-old versions). Clinical data such as age, type of strabismus, angle of deviation, amblyopia, occlusion treatment, and surgical outcome were also recorded. Results All PedEyeQ domain scores were significantly lower in children with strabismus compared with visually normal children, except the Child “functional vision” domain. Children with strabismus with successful corrective surgery had significantly lower scores in many domains of the Child, Proxy, and Parent components, compared with visually normal children. Conclusion This study showed that strabismus has an important impact on affected children and their families, as assessed by PedEyeQ. Interestingly, children with prior successful corrective strabismus surgery had worse PedEyeQ scores compared to visually normal children. Educational programs and psychosocial rehabilitation interventions should be implemented in children with strabismus and their families.

Intravitreal injections of ranibizumab should be evaluated for a potential role on the treatment of amyloid angiopathy neovascular glaucoma. Careful retinal periphery examination should be included in the ophthalmologic examination of all familial amyloidotic polyneuropathy patients.

No abstract