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The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index

Valentini, G; Ludici, M; Walker, UA; Jaeger, VK; Baron, M; Carreira, P; Czirják, L; ... Allanore, Y; + view all (2017) The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index. Annals of the Rheumatic Diseases , 76 (1) pp. 270-276. 10.1136/annrheumdis-2016-209768. Green open access

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Abstract

BACKGROUND: Validity of European Scleroderma Study Group (EScSG) activity indexes currently used to assess disease activity in systemic sclerosis (SSc) has been criticised. METHODS: Three investigators assigned an activity score on a 0-10 scale for 97 clinical charts. The median score served as gold standard. Two other investigators labelled the disease as inactive/moderately active or active/very active. Univariate-multivariate linear regression analyses were used to define variables predicting the 'gold standard', their weight and derive an activity index. The cut-off point of the index best separating active/very active from inactive/moderately active disease was identified by a receiver-operating curve analysis. The index was validated on a second set of 60 charts assessed by three different investigators on a 0-10 scale and defined as inactive/moderately active or active/very active by other two investigators. One hundred and twenty-three were investigated for changes over time in the index and their relationships with those in the summed Medsger severity score (MSS). RESULTS: A weighted 10-point activity index was identified and validated: Δ-skin=1.5 (Δ=patient assessed worsening during the previous month), modified Rodnan skin score (mRss) >18=1.5, digital ulcers=1.5, tendon friction rubs=2.25, C-reactive protein >1 mg/dL=2.25 and diffusing capacity of the lung for CO (DLCO) % predicted <70%=1.0. A cut-off ≥2.5 was found to identify patients with active disease. Changes in the index paralleled those of MSS (p=0.0001). CONCLUSIONS: A preliminarily revised SSc activity index has been developed and validated, providing a valuable tool for clinical practice and observational studies.

Type: Article
Title: The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1136/annrheumdis-2016-209768
Publisher version: https://dx.doi.org/10.1136/annrheumdis-2016-209768
Language: English
Additional information: Copyright © 2016 BMJ Publishing Group Ltd & European League Against Rheumatism. All rights reserved. This article has been accepted for publication in Annals of the Rheumatic Diseases following peer review. The definitive copyedited, typeset version [Valentini, G; Ludici, M; Walker, UA; Jaeger, VK; Baron, M; Carreira, P; Czirják, L; (2017) The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index. Annals of the Rheumatic Diseases, 76 (1) pp. 270-276. 10.1136/annrheumdis-2016-209768] is available online at: http://dx.doi.org/10.1136/annrheumdis-2016-209768
Keywords: Autoimmune Diseases, Disease Activity, Systemic Sclerosis
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/1516569
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