European Scleroderma Study Group to define disease activity criteria for systemic sclerosis. III. Assessment of the construct validity of the preliminary activity criteria

Circulating endothelial cells as a marker of ongoing vascular disease in systemic sclerosis

“…Our data, showing that the amount of CECs closely correlated with disease activity, strongly suggest that these cells could be an additional marker of active disease in patients with SSc. The clinical relevance of our results is further reinforced by the fact that we first used a validated method for the assessment of the disease activity (23). In contrast, previous studies assumed that a short duration of disease together with its rapid progression, and modification of some acute-phase reactants or parameters (e.g., the ESR and the CRP level) could quite well define those patients with active disease (32)(33)(34)(35).…”

“…For all patients with SSc, disease activity was assessed using the activity indices described by the European Scleroderma Study Group (22,23). According to these criteria, disease was considered active if the sum of the scores for detected items was Ն3.…”

“…With respect to pulmonary involvement, 11 (23.9%) of 46 patients with SSc had pulmonary fibrosis with pulmonary hypertension, and 14 (30.4%) of the 46 patients had primary pulmonary hypertension. Evaluation of disease activity by the European Scleroderma Study Group criteria (22,23) indicated that 27 (58.7%) of 46 patients had active disease (Table 1).…”

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).…”