C Ferrari

Long-term outcome and prognostic factors of juvenile dermatomyositis: A multinational, multicenter study of 490 patients

To investigate the long‐term outcome and prognostic factors of juvenile dermatomyositis (DM) through a multinational, multicenter study.

Defining Criteria for Disease Activity States in Nonsystemic Juvenile Idiopathic Arthritis Based on a Three-Variable Juvenile Arthritis Disease Activity Score

To determine cutoff values for defining the states of inactive disease (ID), low disease activity (LDA; or minimal disease activity), moderate disease activity (MDA), and high disease activity (HDA) using the clinical (3‐variable) Juvenile Arthritis Disease Activity Score (cJADAS).

Development and testing of a hybrid measure of muscle strength in juvenile dermatomyositis for use in routine care

To develop and test a hybrid measure of muscle strength for juvenile dermatomyositis (JDM), which is based on the combination of the Manual Muscle Testing in 8 muscles (MMT‐8) and the Childhood Myositis Assessment Scale (CMAS) but is more comprehensive than the former and more feasible than the latter.

PReS-FINAL-2011: Preliminary validation of a new hybrid measure of muscle strength for juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is a multisystem vasculopathic disease characterized by muscle inflammation that causes symmetrical muscle weakness. Assessment of muscle strength is, therefore, a fundamental component of the clinical evaluation of children with JDM. This assessment is traditionally made using the 8-muscle Manual Muscle Testing (MMT) and the Childhood Myositis Assessment Scale (CMAS). However, the MMT does not cover all muscles or muscle groups affected in JDM, namely abdominal muscles. Although the CMAS is more comprehensive than the MMT, it is lengthy and, therefore, may not be feasible in a busy clinical setting or when a physical therapist is not available.

OP0270 Further Validation of the Hybrid MMT/CMAS (HMC), A New Measure of Muscle Disease Activity in Juvenile Dermatomyositis

Background Assessment of muscle strength is a fundamental component of the clinical evaluation of children with juvenile dermatomyositis (JDM). This assessment is traditionally made using the 8-muscle MMT and the CMAS. However, the MMT does not cover all muscles or muscle groups affected in JDM, particularly abdominal muscles. Although the CMAS is more comprehensive than the MMT, it is lengthy and, therefore, may not be feasible in a busy clinical setting or when a physical therapist is not available. We have recently developed a new measure of muscle strength in JDM, named Hybrid MMT/CMAS (hMC), which is more comprehensive than the MMT and more feasible than the CMAS. This tool has previously demonstrated good construct validity. Objectives To further validate the hMC by assessing its discriminant validity. Methods The hMC is composed of all 8 items of MMT and of 3 items of the CMAS: 1) head lift; 2) sits-ups; 3) floor rise. The total score of the hMC ranges from 0 (worst) to 100 (normal). Validation analyses were performed in 167 patients included in a multinational study. Discriminant validity of the hMC was assessed by comparing its score between: 1) patients rated by the physician as being in remission or in high, moderate, or low disease activity; 2) patients rated in remission, continued activity, or flare by physician and parent; 3) parents satisfied or not satisfied with their childs disease outcome. The change in hMC score between 2 consecutive visits (median 3 months) was compared among patients defined as improved, stable, or worsened by physician and parent. Comparisons of quantitative variables among groups were made by nonparametric analysis of variance (Kruskal-Wallis test) and Mann-Whitney U Test. Results The hMC demonstrated strong ability to discriminate between patients in high, moderate, low disease activity or remission according to the physician, between patients defined in remission, continued active disease and flare by the parent (p<0.001), between patients with (p<0.001), and between patients satisfied or not satisfied with disease outcome (p<0.001). The change in hMC score differentiated well patients rated as improved, stable, or worsened by the physician (p<0.0006) or the parent (p=0.0049). Conclusions The hMC showed strong discriminant ability in a large multinational patient sample and is, thus, a valid tool for the assessment muscles disease activity in JDM patients in standard clinical care, observational studies, and clinical trials. Disclosure of Interest None declared

PReS-FINAL-2193: Assessment of construct validity of new measures of global disease activity, physical function and quality of life in children with juvenile dermatomyositis.

Juvenile dermatomyositis (JDM) is a multisystem vasculopathic disease that affects primarily the skin and muscle and is characterized by high risk of morbidity and long-term damage. Regular patient assessment through standardized quantitative clinical measures is important to monitor the disease course over time and to evaluate treatment effectiveness. However, only a few outcome measures specifically validated for use in JDM are available.

SAT0455 Preliminary Validation of a New Hybrid Measure of Muscle Strength for Juvenile Dermatomyositis

Background Juvenile dermatomyositis (JDM) is a multisystem vasculopathic disease characterized by muscle inflammation that causes symmetrical muscle weakness. Assessment of muscle strength is, therefore, a fundamental component of the clinical evaluation of children with JDM. This assessment is traditionally made using the 8-muscle Manual Muscle Testing (MMT) and the Childhood Myositis Assessment Scale (CMAS). However, the MMT does not cover all muscles or muscle groups affected in JDM, namely abdominal muscles. Although the CMAS is more comprehensive than the MMT, it is lengthy and, therefore, may not be feasible in a busy clinical setting or when a physical therapist is not available. Objectives To investigate the construct validity of a new hybrid measure of muscle strength developed by merging the MMT with 3 items of the CMAS Methods The Hybrid MMT/CMAS (hMC) is composed of all 8 items of the MMT and the following items of the CMAS: 1) head lift; 2) sits-ups; 3) floor rise. Item 3) is recoded on 0 to 9 scale. The total score of the hMC ranges from 0 (worst) to 100 (normal). The study sample included 322 children with JDM enrolled in a multinational long-term outcome survey (Ravelli et al. AC&R 2010;62:63-72), who had all measurements available. Validation procedures were conducted by comparing the correlation of the hMC, MMT and CMAS with other conventional measures of JDM activity, physical function and damage. Correlations were computed by means of the Spearman’s correlation coefficient and were considered good, moderate, or poor when the rs was > 0.7, 0.4-0.7, or < 0.4, respectively. Results The Spearman’s correlations of hMC, MMT and CMAS with other measures of disease activity and damage are presented in the table. Conclusions We have developed a new hybrid measure of muscle strength in JDM, which is more comprehensive than the MMT and more feasible than the CMAS. Overall, the construct validity of the hMC was comparable to that of the MMT and superior to that of the CMAS. Disclosure of Interest None Declared

Do parent's global rating of well-being and disease activity of children with juvenile idiopathic arthritis yield different information?

Background The parents of children with juvenile idiopathic arthritis (JIA) are traditionally asked to provide a global assessment of disease status by rating the childs overall level of well-being (WB) on a 10-cm visual analogue scale (VAS). However, it has been advised that this measure does not provide a precise assessment of DA because it is largely affected by the presence of disease damage. For this reason, it has been suggested to substitute the VAS for WB with a VAS assessing specifically the level of DA.

Comparison of the accuracy of different definitions of clinical remission (CR) and minimal disease activity (MDA) in juvenile idiopathic arthritis (JIA)

Background Since the introduction of biologic agents, expectations of medical treatment for chronic arthritides have increased markedly. It is now agreed upon that estimation of effectiveness of these drugs requires not only the assessment of relative improvement in signs and symptoms, but the evaluation of their ability to induce a state of CR or, at least, MDA. In recent years, several criteria for assessing CR and MDA in adult rheumatoid arthritis (RA) or JIA have been developed.

Comparison of physician's, parent's and patient's global ratings made on linear or 21-circle visual analogue scales (VAS) in juvenile idiopathic arthritis (JIA)

15th Paediatric Rheumatology European Society (PreS) Congress Wietse Kuis, Patricia Woo, Angelo Ravelli, Hermann Girschick, Michael Hofer, Johannes Roth, Rotraud K Saurenmann, Alberto Martini, Pavla Dolezova, Janjaap van der Net, Pierre Quartier, Lucy Wedderburn and Jan Scott Meeting abstracts – A single PDF containing all abstracts in this Supplement is available here .