Survival and Causes of Death in an Unselected and Complete Cohort of Norwegian Patients with Systemic Sclerosis

Abstract

Compared to the Norwegian background population, our cohort of 312 unselected patients with SSc had decreased survival. The survival rates observed were, however, better than those previously reported from SSc referral centers.

Early Mortality in a Multinational Systemic Sclerosis Inception Cohort

“…When we limited the definition of the inception cohort to those recruited within 1 year of disease onset, the SMRs were even higher, at 8.1 (95% CI 4.3–12.0) for the Australian cohort, 9.4 (95% CI 6.1–12.8) for the Canadian cohort, and 3.9 (95% CI 2.4–5.4) for the Spanish cohort. The values for Australian and Canadian 1‐year inception cohorts are much higher than those for the corresponding prevalent cohorts and higher than those reported from some previous studies , and they highlight the phenomenon of survivor bias, which leads to underestimation of the true burden of mortality in prevalent cohorts of subjects with SSc. This bias arises in large part because SSc has a substantial burden of early mortality with many deaths occurring within 5 years of disease onset, particularly in men and in the diffuse disease subtype.…”

Radiographic fibrosis score predicts survival in systemic sclerosis‐associated interstitial lung disease

“…We found that age, being male and FVC were also independent predictors of mortality according to the multivariate analysis, and several previous studies have reported similar results . In one previous study, being male was associated with severe restrictive lung disease and baseline FVC was a predictor of fibrosis outcome .…”

CCL21 as a Potential Serum Biomarker for Pulmonary Arterial Hypertension in Systemic Sclerosis

“…We observed that high CCL21 levels were associated with reduced survival but appeared to have less influence on mortality compared with age at onset. This minor impact of CCL21 on mortality probably reflects that PAH, even though it is an important mortality risk factor, accounts for only 12% of all SSc deaths in Norway .…”