European multicentre study to define disease activity criteria for systemic sclerosis. II. Identification of disease activity variables and development of preliminary activity indexes

Abstract

Objective-To develop criteria for disease activity in systemic sclerosis (SSc) that are valid, reliable, and easy to use. Methods-Investigators from 19 European centres completed a standardised clinical chart for a consecutive number of patients with SSc. Three protocol management members blindly evaluated each chart and assigned a disease activity score on a semiquantitative scale of 0-10. Two of them, in addition, gave a blinded, qualitative evaluation of disease activity ("inactive to moderately active" or "active to very active" disease). Both these evaluations were found to be reliable. A final disease activity score and qualitative evaluation of disease activity were arrived at by consensus for each patient; the former represented the gold standard for subsequent analyses. The correlations between individual items in the chart and this gold standard were then analysed. Results-A total of 290 patients with SSc (117 with diVuse SSc (dSSc) and 173 with limited SSc (lSSc)) were enrolled in the study. The items (including -factorsthat is, worsening according to the patient report) that were found to correlate with the gold standard on multiple regression were used to construct three separate 10-point indices of disease activity:

Growth Differentiation Factor 15, a Marker of Lung Involvement in Systemic Sclerosis, Is Involved in Fibrosis Development but Is not Indispensable for Fibrosis Development

“…Disease activity scores were determined as described previously (14). SSc organ system involvement was evaluated at all time points based on the disease severity scale of Medsger et al (15).…”

Association of C‐reactive protein with high disease activity in systemic sclerosis: Results from the Canadian Scleroderma Research Group

“…ESR was measured by the Westergren method (mm/hour) and was considered high when Ͼ20 mm/hour. Mean CRP concentrations (mg/liter) and the prevalence of elevated CRP levels in each disease subset were studied and associations with clinical parameters determined, including skin score measured by the modified Rodnan skin score (MRSS), estimated systolic pulmonary artery pressure (PAP) measured by transthoracic echocardiograms, pulmonary function tests (PFTs), ILD determined by chest radiographs and high-resolution computerized tomography (HRCT) of the chest, incidence of SRC, existence of arthritis determined by either tender joint count (TJC) or swollen joint count (SJC), tendon friction rubs, Scleroderma Disease Activity Score (SDAS) adapted from the European Scleroderma Study Group (EScSG) whole series activity index (33)(34)(35)(36), Scleroderma Disease Severity Score (SDSS) adapted from Medsger et al (37), and Health Assessment Questionnaire (HAQ) disability index (DI).…”

Systemic sclerosis is an independent risk factor for increased coronary artery calcium deposition

“…An underlying inhibitor was suspected following failure to normalize the coagulation time in a 1:1 patient:control plasma mixing assay. Total disease activity was scored according to the European Scleroderma Activity criteria, which includes the total skin score, scleredema, digital necrosis, arthritis, reduced total lung carbon monoxide transfer factor, ESR Ͼ30 mm/ hour, hypocomplementemia, and deterioration in skin, vascular, and muscular/articular conditions in the month preceding the assessment (23,24).…”