Search

Introduction: In systemic sclerosis (SSc), disease severity is comprised of both disease activity and disease-related damage. While disease activity is potentially reversible, damage is permanent. To date, proposed outcome measures in SSc have focused primarily on disease activity features. Objective(s): To describe disease-related damage and determine its frequency in a cohort of patients with SSc. Method(s): All patients enrolled in the Australian Scleroderma Screening Program (ASSP) - a multicentre prospective database of patients with SSc - were included in the study. Significant damage in each organ system was defined as the presence ever of the following: skin/musculoskeletal - one or more of digital gangrene, digital amputation, joint contractures, myositis with proximal muscle atrophy; gastro-intestinal - one or more of esophageal stricture, bowel dysmotility, pseudo-obstruction, anal incontinence; cardiovascular - one or more of SSc myocardial disease with either conduction abnormality or LV systolic/diastolic dysfunction, ejection fraction <50% on echocardiography without regional wall abnormality; respiratory - one or more of pulmonary fibrosis with either FVC <75% or DLCO <50%, moderate-severe extent of fibrosis on HRCT, pulmonary hypertension with RV dysfunction or dilatation; renal - renal crisis with eGFR < 60 ml/sec. The prevalence of damage in each organ system was determined for the cohort as a whole and also for those recruited within 4 years of diagnosis of SSc. Result(s): There were 831 patients in the study [724(87.1%) female, 522(62.8%) had limited and 227(27.3%) had diffuse SSc]. Mean+/-SD age at onset and disease duration at first visit were 45.6+/-14.1 and 12.2+/-10.3 years, respectively. 193 patients were recruited within 4 years of diagnosis. Among this inception cohort, the following n(%) fulfilled the definition of damage in each organ system within the first 4 years of disease: skin/musculoskeletal 71(36.8%), gastro-intestinal 24(12.4%

Introduction: In systemic sclerosis (SSc), disease severity is comprised of both disease activity and disease-related damage. While disease activity is potentially reversible, damage is permanent. To date, proposed outcome measures in SSc have focused primarily on disease activity features. Objective(s): To describe disease-related damage and determine its frequency in a cohort of patients with SSc. Method(s): All patients enrolled in the Australian Scleroderma Screening Program (ASSP) - a multicentre prospective database of patients with SSc - were included in the study. Significant damage in each organ system was defined as the presence ever of the following: skin/musculoskeletal - one or more of digital gangrene, digital amputation, joint contractures, myositis with proximal muscle atrophy; gastro-intestinal - one or more of esophageal stricture, bowel dysmotility, pseudo-obstruction, anal incontinence; cardiovascular - one or more of SSc myocardial disease with either conduction abnormality or LV systolic/diastolic dysfunction, ejection fraction <50% on echocardiography without regional wall abnormality; respiratory - one or more of pulmonary fibrosis with either FVC <75% or DLCO <50%, moderate-severe extent of fibrosis on HRCT, pulmonary hypertension with RV dysfunction or dilatation; renal - renal crisis with eGFR < 60 ml/sec. The prevalence of damage in each organ system was determined for the cohort as a whole and also for those recruited within 4 years of diagnosis of SSc. Result(s): There were 831 patients in the study [724(87.1%) female, 522(62.8%) had limited and 227(27.3%) had diffuse SSc]. Mean+/-SD age at onset and disease duration at first visit were 45.6+/-14.1 and 12.2+/-10.3 years, respectively. 193 patients were recruited within 4 years of diagnosis. Among this inception cohort, the following n(%) fulfilled the definition of damage in each organ system within the first 4 years of disease: skin/musculoskeletal 71(36.8%), gastro-intestinal 24(12.4%