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Pediatr Rheumatol Online J. 2019 Oct 1;17(1):66. doi: 10.1186/s12969-019-0345-2.

Proposal for a definition for response to treatment, inactive disease and damage for JIA associated uveitis based on the validation of a uveitis related JIA outcome measures from the Multinational Interdisciplinary Working Group for Uveitis in Childhood (MIWGUC).

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Head of the Hamburg Centre for Pediatric and Adolescence Rheumatology Centre for Treatment of Scleroderma and Uveitis in Childhood and Adolescence Teaching Unit of the Asklepios Campus of the Semmelweis Medical School, Budapest An der Schön Klinik Hamburg Eilbek Dehnhaide, 120 22081, Hamburg, Germany.
German Rheumatism Research Centre, 10117, Berlin, Germany.
Institute for Social Medicine, Epidemiology, and Health Economics, Charité Universitaetsmedizin Berlin, Berlin, Germany.
Rheumatology Unit- A. Meyer Children's Hospital- NEUROFARBA Department, University of Florence, Florence, Italy.
Dept Rheumatology, Great Ormond Street Hospital, Great Ormond Street, London, UK.
Division of Rheumatology, Cincinnati Children's Hospital Medical Center, 3333 Burnett Avenue, Cincinnati, OH 45229; Department of Pediatrics, University of Cincinnati, Cincinnati, OH, USA.
Department of Ophthalmology, Copenhagen University Hospital Glostrup/Rigshospitalet, Copenhagen, Denmark.
UMC Utrecht, Utrecht, Netherlands.
Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany.
Pediatric Rheumatology Department, Hospital Sant Joan de Déu, Barcelona, Spain.
Institut de Recerca Sant Joan de Déu, Barcelona, Spain.
2nd Department of Pediatrics, Semmelweis University, Budapest, Hungary.
AOU Meyer, Florence, Italy.
Ophtalmology Department, Hospital Sant Pau, Barcelona, Spain.
Ophtalmology Department, Hospital Sant Joan de Déu, Barcelona, Spain.
Università di Milano - Istituto Gaetano Pini, Milan, Italy.
Pediatric Rheumatology Unit, Centro Hospitalar Universitário do Porto, Porto, Portugal.
St. Franziskus-Hospital Münster, Muenster, Germany.
Department of Ophthalmology, Helsinki University Hospital, Helsinki, Finland.
Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Rheumatology and Clinical Immunology, Berlin, Germany.
Pediatric Ophthalmologist at the Centro Hospitalar Universitario do Porto, Teaching Unit of the Abel Salazar Institute of Biomedical Sciences, Largo do Prof. Abel Salazar, 4099-001, Porto, Portugal.
Ocular Immunology and Uveitis Service, Department of Ophthalmology, San Raffaele Scientific Institute, Via Olgettina 60, 20122, Milan, Italy.
Emory Clinic, Atlanta, USA.
Department of Surgery and Surgery Specializations. Universitat de Barcelona, Barcelona, Spain.



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.


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.


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.


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.


Anterior uveitis; Damage; Inactive disease; Juvenile idiopathic arthritis; Outcome measures; Response; Uveitis

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