Systemic sclerosis: low survival rate despite advances in diagnostic methods

Despite advances in the understanding of systemic sclerosis (SSc) and in diagnostic methods over the past few decades, the survival rates of patients diagnosed with this disease remain low, shows a new Swedish study published in Rheumatology. In this nationwide cohort study,1 researchers investigated the survival patterns of all patients with SSc in Sweden compared to matched individuals from the general population of the country. SSc is a rare rheumatic disease of the connective tissue, with multiple organic manifestations involving the skin, the musculoskeletal system, the lungs and the kidneys,2 and has one of the highest mortality rates.3 Although more common in women, SSc in men has been described as a factor of poor prognosis.4 Read: Have you boned up on osteoporosis screening? Methodology The researchers used the National Patient Registry, which includes information on hospitalizations and diagnosis, to identify subjects with SSc diagnosed between 2004 and 2015. They used the Total Population Register to identify comparators (1:5), matched for sex, year of birth and area of residence. All these subjects were followed until their death or emigration, or to the end of 2016. The Kaplan-Meier method was used to estimate the survival of SSc patients and their comparators. Crude mortality rates and hazard ratios (HR) were also assessed using flexible parametric models adjusted for age and sex. Finally, the risk of premature death in patients with SSc, compared to their comparators, was estimated by considering deaths occurring at less than 70 years of age as premature. Read: New screening guideline for fragility fracture Principle results Some 1,139 SSc patients and 5,613 matched comparators from the general population were identified between 2004 and 2015. The mean age at start of follow-up was 58.7 years (± 14.5 years) and 80% were female. The median follow-up was five years for SSc patients and six years for their comparators. During follow-up, 268 deaths occurred in patients with SSc (64 men and 204 women) and 554 in their comparators (141 men and 413 women). The mean age at death was 72.3 years in SSc patients and 76.5 years in their comparators. The five-year survival was 79.8% and the 10-year survival was 67.7% in SSc patients versus 92.9% and 84.8%, respectively, in the comparators (p < 0.0001 ). No significant difference in survival was observed between men and women with SSc. The mortality rate in patients with SSc was 42.1 per 1,000 person-years and 15.8 per 1,000 person-years in their comparators, corresponding to an age and sex-adjusted HR of 3.7 (95% CI: 2.9-4.7) at the end of the first year of follow-up and 2.0 (95% CI: 1.4-2.8) at the end of the follow-up period. Patients with SSc had a 181% higher rate of premature death at the end of the fifth year of follow-up, then 147% at the end of the tenth year of follow-up and finally 131% at the end of the follow-up period, compared to their comparators. In conclusion, these results show that survival is still seriously affected in patients diagnosed with SSc for the time period considered by this study. References: 1. Bairkdar, M. et al. (2023). Survival in Swedish patients with systemic sclerosis: a nationwide population-based matched cohort study. Rheumatology 62(3), 1170–1178. 2. Walker, U.A. et al. (2007). Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis 66, 754–763. 3. Nikpour, M. and Baron, M. (2014). Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies. Curr Opin Rheumatol 26, 131–137. 4. Hughes, M. et al. (2020). Gender-related differences in systemic sclerosis. Autoimmune Rev 19.102494. This article has been translated from its original French and first appeared on our sister site Profession Santé.

Systemic sclerosis: low survival rate despite advances in diagnostic methods

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