TY - JOUR T1 - 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 JF - Annals of the Rheumatic Diseases JO - Ann Rheum Dis SP - 270 LP - 276 DO - 10.1136/annrheumdis-2016-209768 VL - 76 IS - 1 AU - Gabriele Valentini AU - Michele Iudici AU - Ulrich A Walker AU - Veronika K Jaeger AU - Murray Baron AU - Patricia Carreira AU - László Czirják AU - Christopher P Denton AU - Oliver Distler AU - Eric Hachulla AU - Ariane L Herrick AU - Otylia Kowal-Bielecka AU - Janet Pope AU - Ulf Müller-Ladner AU - Gabriela Riemekasten AU - Jerome Avouac AU - Marc Frerix AU - Suzana Jordan AU - Tünde Minier AU - Elise Siegert AU - Voon H Ong AU - Serena Vettori AU - Yannick Allanore Y1 - 2017/01/01 UR - http://ard.bmj.com/content/76/1/270.abstract N2 - 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. ER -