RT Journal Article
SR Electronic
T1 Survival and Causes of Death in an Unselected and Complete Cohort of Norwegian Patients with Systemic Sclerosis
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 1127
OP 1133
DO 10.3899/jrheum.121390
VO 40
IS 7
A1 Anna-Maria Hoffmann-Vold
A1 Øyvind Molberg
A1 Øyvind Midtvedt
A1 Torhild Garen
A1 Jan Tore Gran
YR 2013
UL http://www.jrheum.org/content/40/7/1127.abstract
AB Objective. To determine survival and causes of death in an unselected and complete cohort of Norwegian patients with systemic sclerosis (SSc) compared to the background population. Methods. Multiple methods were used to identify every patient with SSc living in southeast Norway, with a denominator population of 2,707,012, between 1999 and 2009. All patients who met either the American College of Rheumatology criteria or the Medsger and LeRoy criteria for SSc were included. Every patient was matched for sex and age with 15 healthy controls drawn from the national population registry. Vital status at January 1, 2010, was provided for patients and controls by the national population registry. Causes of death were obtained from death certificates and by chart review. Results. Forty-three (14%) of 312 patients with SSc died during the study period. The standardized mortality rate (SMR) was estimated to be 2.03 for the entire cohort and 5.33 for the subgroup with diffuse cutaneous (dc) SSc. The 5- and 10-year survival rates were 91% and 70%, respectively, for dcSSc and 98% and 93% for limited cutaneous (lc) SSc. Causes of death were related to SSc in 24/43 (56%) patients, mostly cardiopulmonary diseases (n = 13), including pulmonary hypertension (n = 8). Factors associated with fatal outcome included male sex, dcSSc, pulmonary hypertension, and interstitial lung disease. Conclusion. 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.