Raynaud and digital ulcers in patients with juvenile systemic sclerosis: ambulatory iloprost protocol. A single center experience

Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort

“…All rights reserved < 80% of the predicted value; diffusing capacity for carbon monoxide (DLCO) < 80%; or high-resolution computed tomography (HRCT) findings consistent with interstitial lung disease (ILD). Moreover, digital ulcers were quite common in our initial cohort assessment [3] and have an impact on daily life in our jSSc patients [2,14], therefore we have incorporated the DUCAS score [15] as an outcome variable collected prospectively. Data was collected prospectively according to a standardized assessment protocol every 6 months (see Supplement CRF).…”

“…In addition to the variables listed in our prior study (3), we created a "composite pulmonary involvement" variable, defined as meeting at least 1 of the following criteria: a forced vital capacity (FVC) of <80% of the predicted value; a diffusion capacity for carbon monoxide (DLco) of <80%; or high-resolution computed tomography (HRCT) findings consistent with interstitial lung disease (ILD). Moreover, digital ulcers were quite common in our initial cohort assessment (3) and have an impact on daily life in our juvenile SSc patients (2,14); therefore, we have incorporated the Digital Ulcer Clinical Assessment Score (DUCAS) (15) as an outcome variable collected prospectively. Data were collected prospectively according to a standardized assessment protocol every 6 months (see Supplementary Appendix A, available at http://onlinelibrary.wiley.com/doi/10.1002/acr.24609).…”