Analysis of Clinical Symptoms and Laboratory Profiles in Children with Juvenile Idiopathic Arthritis in Malopolska Region (Poland) in the Years 2007-2010

Background: Juvenile idiopathic arthritis (JIA) is a heterogenic group of chronic inflammatory connective tissue diseases of unknown aetiology in children up to 16 years of age.Aim: The aim of this study was to analyse the incidence, clinical presentation and laboratory findings in children with JIA in Malopolska region.Materials and methods: A retrospective analysis included all children with JIA (N=251) hospitalized in the two reference rheumatology centres covering Malopolska region (Poland), between July 2007 and December 2010.Results: The annual incidence of JIA in Malopolska region was estimated at 9.5 per 100 000 children. Oligoarthritis (54.9%) was the most common category in all age groups with a tendency to decrease with age; from 71.4 % in children aged 1-6 years; 55.7% in aged 7-12 years to 39.3 % in aged 13-16 years. The frequency of polyarthritis and enthesitis-related arthritis was greater in adolescents (29.2 % and 22.5 %, respectively). HLA-B27 antigen and uveitis were most frequently found in children with enthesitis-related arthritis (58% and 18.5 %, respectively).Conclusions: The study suggests the improvement of diagnostic capacity of JIA during the last decade in Poland. In accordance with the existing data diverse clinical presentation of JIA categories and laboratory characteristics were proven

Definition and Validation of the American College of Rheumatology 2021 Juvenile Arthritis Disease Activity Score Cutoffs for Disease Activity States in Juvenile Idiopathic Arthritis

Export Date: 16 February 2023; Cited By: 10Peer reviewe

2016 ACR-EULAR adult dermatomyositis and polymyositis and juvenile dermatomyositis response criteria-methodological aspects

Objective. The objective was to describe the methodology used to develop new response criteria for adult DM/PM and JDM. Methods. Patient profiles from prospective natural history data and clinical trials were rated by myositis specialists to develop consensus gold-standard ratings of minimal, moderate and major improvement. Experts completed a survey regarding clinically meaningful improvement in the core set measures (CSM) and a conjoint-analysis survey (using 1000Minds software) to derive relative weights of CSM and candidate definitions. Six types of candidate definitions for response criteria were derived using survey results, logistic regression, conjoint analysis, application of conjoint-analysis weights to CSM and published definitions. Sensitivity, specificity and area under the curve were defined for candidate criteria using consensus patient profile data, and selected definitions were validated using clinical trial data. Results. Myositis specialists defined the degree of clinically meaningful improvement in CSM for minimal, moderate and major improvement. The conjoint-analysis survey established the relative weights of CSM, with muscle strength and Physician Global Activity as most important. Many candidate definitions showed excellent sensitivity, specificity and area under the curve in the consensus profiles. Trial validation showed that a number of candidate criteria differentiated between treatment groups. Top candidate criteria definitions were presented at the consensus conference. Conclusion. Consensus methodology, with definitions tested on patient profiles and validated using clinical trials, led to 18 definitions for adult PM/DM and 14 for JDM as excellent candidates for consideration in the final consensus on new response criteria for myositis