Applicability of the paediatric rheumatology international trials organisation disease activity core set for juvenile dermatomyositis: Comment on the article by Ruperto et al

2008 ◽  
Vol 59 (8) ◽  
pp. 1197-1198 ◽  
Author(s):  
Lisa G. Rider ◽  
Peter Lachenbruch ◽  
David A. Isenberg ◽  
Frederick W. Miller
Keyword(s):  
Disease Activity ◽  
Juvenile Dermatomyositis ◽  
Paediatric Rheumatology ◽  
Core Set

scholarly journals Juvenile Dermatomyositis Magnetic Resonance Imaging Score (JIS) does not correlate with criteria for clinically inactive disease: a single-centre retrospective evaluation

2021 ◽  
Author(s):  
Kapil Gargh ◽  
Eslam Al-Abadi ◽  
Samantha Low ◽  
Kathryn Harrison ◽  
William Coles ◽  
...  
Keyword(s):  
Disease Activity ◽  
Juvenile Dermatomyositis ◽  
Treatment Decision ◽  
Clinical Decision ◽  
Inactive Disease ◽  
Mri Findings ◽  
Clinical Criteria ◽  
Physician Decision Making ◽  
Core Set

AbstractThe Paediatric Rheumatology International Trials Organisation (PRINTO) criteria for clinically inactive disease (CID) and their proposal for glucocorticoid tapering do not consider MRI findings, despite the growing use of MRI and development of reliable MRI scoring tools. We aim to evaluate how CID correlates with MRI scores and physician decision making. We retrospectively used the Juvenile Dermatomyositis Imaging Score (JIS) to score MRIs of all children with JDM over a 10-year period. Demographic, diagnosis, treatment and core set measures data were collected. Correlation between CID and JIS was assessed as well as correlation with the physician treatment decision. There were 25 patients with 59 follow-up episodes to analyse correlation between physician treatment decision and JIS; and 50 episodes for the CID category and JIS correlation. JIS was not significantly associated with the CID category but did correlate with the physician decision. No significant association was found between clinical decision and CID category. The JIS area under the ROC curve (AUC) was 0.80 (95% CI 0.62–0.99) with a score ≥ 8 to predict an escalation. JIS sensitivity and specificity were both 78% with accuracy of 78%, compared to only 67%, 46% and 49%, respectively, for the CID criteria. Clinical criteria alone are not sufficient to assess disease activity status. Clinical decision trends correlated to MRI findings but not PRINTO CID criteria. Multi centre prospective studies are needed to replicate our findings and establish how to best use MRI as a biomarker of disease activity.

scholarly journals Validation of a score tool for measurement of histological severity in juvenile dermatomyositis and association with clinical severity of disease

2013 ◽  
Vol 74 (1) ◽  
pp. 204-210 ◽  
Author(s):  
Hemlata Varsani ◽  
Susan C Charman ◽  
Charles K Li ◽  
Suely K N Marie ◽  
Anthony A Amato ◽  
...  
Keyword(s):  
Disease Activity ◽  
Muscle Biopsy ◽  
Juvenile Dermatomyositis ◽  
Intraclass Correlation ◽  
Latin Square ◽  
Quadriceps Muscle ◽  
Clinical Severity ◽  
Intraobserver Agreement ◽  
Biopsy Tissue ◽  
Clinical Measures

ObjectivesTo study muscle biopsy tissue from patients with juvenile dermatomyositis (JDM) in order to test the reliability of a score tool designed to quantify the severity of histological abnormalities when applied to biceps humeri in addition to quadriceps femoris. Additionally, to evaluate whether elements of the tool correlate with clinical measures of disease severity.Methods55 patients with JDM with muscle biopsy tissue and clinical data available were included. Biopsy samples (33 quadriceps, 22 biceps) were prepared and stained using standardised protocols. A Latin square design was used by the International Juvenile Dermatomyositis Biopsy Consensus Group to score cases using our previously published score tool. Reliability was assessed by intraclass correlation coefficient (ICC) and scorer agreement (α) by assessing variation in scorers’ ratings. Scores from the most reliable tool items correlated with clinical measures of disease activity at the time of biopsy.ResultsInter- and intraobserver agreement was good or high for many tool items, including overall assessment of severity using a Visual Analogue Scale. The tool functioned equally well on biceps and quadriceps samples. A modified tool using the most reliable score items showed good correlation with measures of disease activity.ConclusionsThe JDM biopsy score tool has high inter- and intraobserver agreement and can be used on both biceps and quadriceps muscle tissue. Importantly, the modified tool correlates well with clinical measures of disease activity. We propose that standardised assessment of muscle biopsy tissue should be considered in diagnostic investigation and clinical trials in JDM.

Investigating the Validity of the Minimal Disease Activity State for Patients with Rheumatoid Arthritis Treated with Abatacept

2009 ◽  
Vol 36 (2) ◽  
pp. 260-265 ◽  
Author(s):  
GEORGE A. WELLS ◽  
MAARTEN BOERS ◽  
TRACY LI ◽  
PETER S. TUGWELL
Keyword(s):  
Rheumatoid Arthritis ◽  
Disease Activity ◽  
Health Assessment ◽  
Control Group ◽  
Minimal Disease Activity ◽  
The Core ◽  
Core Set ◽  
Number Of Patients ◽  
Minimal Disease ◽  
Activity State

Objective.To validate the definitions of minimal disease activity (MDA) in patients with rheumatoid arthritis (RA) and to compare abatacept to control with respect to patients attaining a state of MDA.Methods.Two randomized controlled trials comparing abatacept to control in patients with RA were considered: ATTAIN and AIM. Core set measures, Disease Activity Score 28-joint count (DAS28), and, for AIM, radiographic scores were available. The core set and DAS-based definitions for MDA were calculated and the number of patients in the treatment groups meeting the definitions was compared to determine sensitivity of the criteria to treatment differences and patient severity. The number of times achieving MDA was compared to the change in Health Assessment Questionnaire (HAQ), and for the AIM study compared to change in radiographic scores.Results.For both definitions of MDA, the change in radiographic scores showed a continual decrease in progression the more often a patient was in MDA. The change in HAQ, for both studies, showed a similar consistent improvement — the longer a patient was in MDA, then the better the HAQ score. Significantly more patients in the abatacept group met the core set and DAS-based definition of MDA than in the control group.Conclusion.The presence and persistence of MDA was associated with slowing of radiographic progression and improvement in the HAQ, providing support for discriminative and predictive validity of the measure. The MDA results were consistent with other efficacy analyses indicating a treatment advantage for abatacept.

Outcome assessment in the idiopathic inflammatory myopathies

2018 ◽  
Author(s):  
Lisa G. Rider ◽  
Frederick W. Miller
Keyword(s):  
Disease Activity ◽  
Endothelial Activation ◽  
Inactive Disease ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Muscle Enzyme ◽  
Activation Markers ◽  
Core Set ◽  
Trial Endpoints ◽  
Muscle Ultrasound

Due to their rarity, heterogeneity, and multispecialty nature, the myositis syndromes have limited data-driven consensus on appropriate outcome measures. Recently, two international, multispecialty consortia developed new tools and consensus on core set measures of myositis disease activity and damage, as well as response criteria that are now recommended for use as clinical trial endpoints but will also be useful in clinical practice. Magnetic resonance imaging, muscle ultrasound, selected laboratory tests, and immunological biomarkers—including cytokines, chemokines, lymphocyte flow cytometry, and endothelial activation markers—can all be helpful adjuncts to serum muscle enzyme levels in assessing disease activity and damage, but these have not yet been fully validated. Definitions of clinically inactive disease, complete clinical response, and remission have also been proposed but require further validation. These advances should enhance the development of therapies by standardizing our ability to demonstrate their efficacy in treating the idiopathic inflammatory myopathies.

scholarly journals Galectin‐9 and CXCL10 as Biomarkers for Disease Activity in Juvenile Dermatomyositis: A Longitudinal Cohort Study and Multicohort Validation

2019 ◽  
Vol 71 (8) ◽  
pp. 1377-1390 ◽  
Author(s):  
Judith Wienke ◽  
Felicitas Bellutti Enders ◽  
Johan Lim ◽  
Jorre S. Mertens ◽  
Luuk L. Hoogen ◽  
...  
Keyword(s):  
Cohort Study ◽  
Disease Activity ◽  
Juvenile Dermatomyositis ◽  
Longitudinal Cohort Study ◽  
Longitudinal Cohort

scholarly journals Endothelial Activation Markers as Disease Activity and Damage Measures in Juvenile Dermatomyositis

2019 ◽  
Vol 47 (7) ◽  
pp. 1011-1018
Author(s):  
Takayuki Kishi ◽  
Jonathan Chipman ◽  
Melvina Evereklian ◽  
Khanh Nghiem ◽  
Maryalice Stetler-Stevenson ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Factor Viii ◽  
Disease Activity ◽  
Peripheral Blood ◽  
Muscle Activity ◽  
Juvenile Dermatomyositis ◽  
Interleukin 10 ◽  
Circulating Endothelial Cells ◽  
Activation Markers ◽  
Functional Disturbance

Objective.Circulating endothelial cells (CEC), von Willebrand factor (vWF) antigen, P-selectin, and thrombomodulin are released from damaged endothelium, while decreases in circulating endothelial progenitor cells (CEPC) have been associated with poor vascular outcomes. We examined these markers in the peripheral blood of patients with juvenile dermatomyositis (JDM) and their correlations with disease assessments.Methods.Peripheral blood endothelial cells and biomarkers were assessed in 20 patients with JDM and matched healthy controls. CEC and CEPC were measured by flow cytometry, while vWF antigen and activity, factor VIII, P-selectin, and thrombomodulin were measured in plate-based assays. Disease activity and damage, nailfold capillary density, and brachial artery flow dilation were assessed. Serum cytokines/chemokines were measured by Luminex.Results.CEC, vWF antigen, factor VIII, and thrombomodulin, but not vWF activity, CEPC, or P-selectin, were elevated in the peripheral blood of patients with JDM. CEC correlated with pulmonary activity (rs = 0.56). The vWF antigen correlated with Patient’s/Parent’s Global, cutaneous, and extramuscular activity (rs = 0.47–0.54). CEPC negatively correlated with muscle activity and physical function (rs = −0.52 to −0.53). CEPC correlated inversely with endocrine damage. The vWF antigen and activity correlated with interleukin 10 and interferon-gamma inducible protein-10 (rs = 0.64–0.82).Conclusion.Markers of endothelial injury are increased in patients with JDM and correlate with extramuscular activity. CEPC correlate inversely with muscle activity, suggesting a functional disturbance in repair mechanisms.

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