Author:
Valentini Gabriele,Iudici Michele,Walker Ulrich A,Jaeger Veronika K,Baron Murray,Carreira Patricia,Czirják László,Denton Christopher P,Distler Oliver,Hachulla Eric,Herrick Ariane L,Kowal-Bielecka Otylia,Pope Janet,Müller-Ladner Ulf,Riemekasten Gabriela,Avouac Jerome,Frerix Marc,Jordan Suzana,Minier Tünde,Siegert Elise,Ong Voon H,Vettori Serena,Allanore Yannick
Abstract
BackgroundValidity of European Scleroderma Study Group (EScSG) activity indexes currently used to assess disease activity in systemic sclerosis (SSc) has been criticised.MethodsThree 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).ResultsA 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).ConclusionsA preliminarily revised SSc activity index has been developed and validated, providing a valuable tool for clinical practice and observational studies.
Subject
General Biochemistry, Genetics and Molecular Biology,Immunology,Immunology and Allergy,Rheumatology
Cited by
146 articles.
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