Systemic Sclerosis: Results from a multicenter survey of Norwegian patients

Abstract

Systemic sclerosis (SSc) is a serious multiorgan disease, characterized by progressive fibrosis, vasculopathy and distinct serum auto antibodies (1, 2). Patients with SSc have an increased mortality risk and reduced quality of life (3-5). Interstitial lung disease (ILD) is a common manifestation in SSc and the leading cause of morbidity and mortality in the SSc population (3, 6). Classification of SSc was done with the 1980 American College of Rheumatology (ACR) classification criteria until 2013, when EULAR and ACR launched unified new classification criteria for SSc (7, 8). The aim of the study was to estimate the prevalence of SSc in Norway, investigate survival and causes of deaths and to study fibrosis progression and lung function by serial assessments of high resolution computed tomography (HRCT) and concurrent pulmonary function tests (PFT). Additionally, we wanted to confirm the true value of the 2013 ACR/EULAR classification criteria for SSc. We found a prevalence of SSc in Norway of 9.9/100 000, a value comparable to other Northern European countries, supporting the notion of a north-south gradient of SSc in Europe with the lowest prevalence in Northern Europe. Our data show that mortality in SSc is still increased compared to the background population and the major causes of death are pulmonary hypertension (PH) and ILD. When studying lung fibrosis and lung function in serial assessments, we demonstrate that a normal baseline HRCT was highly predictive against later fibrosis development, and that HRCT findings at baseline predicted fibrosis progression rates and the deterioration rates of pulmonary function. Male gender, SSc subtype, antibodies, baseline fibrosis and baseline lung function were main risk factors for fibrosis development and progression. The 2013 ACR/EULAR SSc criteria performed remarkably well in our cohort and the sensitivity was significantly better than the ACR1980 criteria using the combination of the ACR 1980 criteria and/or the Leroy & Medsger criteria as the “gold standard” for SSc. Our results reinforce the view that SSc is a rare but serious disease with increased morbidity and mortality and that pulmonary disease is a devastating complication. Our data support the notion that the 2013 ACR/EULAR criteria are a major step forward and that their application should improve the quality of clinical and epidemiological SSc research in the years to come.