Causes and predictors of death among Finnish patients with systemic sclerosis

Abstract

Objective

The aim of this study was to assess causes and predictors of death among Finnish patients with systemic sclerosis (SSc).

Method

Medical records of patients registered with the ICD-10 code M34 from 1996 to 2018 in two university hospitals were reviewed retrospectively. Clinical data were collected until the end of 2020. Death certificates were obtained from Statistics Finland up to August 2021. Using death certificates and patient records, the cause of death for each patient was determined. The mean age at death, median time from SSc diagnosis, and factors predicting death were analysed.

Results

Among 313 SSc patients, 91 deaths occurred between April 2000 and September 2020. Overall 5 and 10 year survival rates were 88.4% and 80.2%, respectively. SSc was the most common primary cause of death (n = 35) and interstitial lung disease (ILD) was the most common SSc-related cause of death (n = 13). Moreover, 52% of the patients with diffuse SSc and 33% of those with limited cutaneous SSc died as a result of SSc itself. Patients who died because of SSc were significantly younger [mean ± sd age 65.6 ± 12.7 years, 95% confidence interval (CI) 61.2–70.1] than those who died from other causes (74.2 ± 9.6 years, 95% CI 71.5–76.9) (p = 0.0006). ILD, pulmonary arterial hypertension, gastrointestinal involvement, male gender, and older age at disease onset predicted death.

Conclusion

The disease itself was the major cause of death among Finnish SSc patients, in both diffuse and limited forms of SSc.

Disclosure statement

SK is an investigator in clinical psoriatic arthritis (PsA) drug studies funded by AbbVie, Pfizer, and BMS, and an SSc drug study funded by Boehringer Ingelheim, and has also received consulting and speaker fees from UCB, AbbVie, Boehringer Ingelheim, and Medac, and scientific meeting attendance support from UCB, Roche, and AbbVie, which are all unrelated to this work. JH has attended advisory boards organized by AbbVie, Amgen, Boehringer Ingelheim, Fresenius Kabi, Janssen, and Novartis, and received scientific meeting attendance support from AbbVie, Medac, Novartis, and Pfizer, which are all unrelated to this work. MK has received scientific meeting attendance support from Pfizer, which is unrelated to this work. TR has declared no conflicts of interest. JP is an investigator in clinical PsA drug studies funded by Lilly, Boehringer Ingelheim, AbbVie, Pfizer, and BMS; she has received a speaker fee from UCB and scientific meeting attendance support from Medac, Jansen-Cilag, and UCB, which are all unrelated to this work. LP is an investigator in clinical PsA drug studies funded by AbbVie, Pfizer, and BMS, and has also received consulting and speaker fees from AbbVie, Boehringer Ingelheim, Jansen-Cilag, Novartis Finland, Sandoz, Eli Lilly, UCB Finland, Celltrion, Fresenius-Gabi, and Swedish Orphan Biovirtum, and scientific meeting attendance support from Orion, Sanofi, and Amgen, which are all unrelated to this work. KT is an investigator in a clinical GCA drug study funded by Novartis, and has received consulting and speaker fees from AbbVie, Vifor Pharma, and Pfizer, and scientific meeting attendance support from UCB and Pfizer, which are all unrelated to this work.

Additional information

Funding

This work was supported by research grants from the Finnish Foundation for Rheumatic Diseases to SK and MK, Turunmaa Duodecim Society to SK, Turun Yliopistollisen Keskussairaalan Koulutus- ja Tutkimussäätiö to SK and Tampere Rheumatism Association to MK; and Turun Yliopistollisen Keskussairaalan Koulutus- ja Tutkimussäätiö.