Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database
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Objectives To determine the causes and predictors of mortality in systemic sclerosis (SSc).Methods Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality.Results Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points).Conclusion Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc.
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Systemic sclerosis is frequently associated with malignancy.
“Importantly, SSc is frequently associated with malignancy.144”
From Autoimmune diseases and immune-checkpoint inhibitors for cancer therapy: Review of the literature and personalized risk-based prevention strategy (2020) · cites this paper as [144]
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- Authors
- Tyndall, A. J., Bannert, B., Vonk, M., Airò, P., Cozzi, F., Carreira, P. E., Bancel, D. F., Allanore, Y., Müller-Ladner, U., Distler, O., Iannone, F., Pellerito, R., Pileckyte, M., Miniati, I., Ananieva, L., Gurman, A. B., Damjanov, N., Mueller, A., Valentini, G., Riemekasten, G., Tikly, M., Hummers, L., Henriques, M. J., Caramaschi, P., Scheja, A., Rozman, B., Ton, E., Kumánovics, G., Coleiro, B., Feierl, E., Szucs, G., Von Mühlen, C. A., Riccieri, V., Novak, S., Chizzolini, C., Kotulska, A., Denton, C., Coelho, P. C., Kötter, I., Simsek, I., de la Pena Lefebvre, P. G., Hachulla, E., Seibold, J. R., Rednic, S., Štork, J., Morovic-Vergles, J., & Walker, U. A.
- Affiliations
- University of Basel (CH)
- Radboud University Medical Center (NL)
- University of Padua (IT)
- Hospital Universitario 12 De Octubre (ES)
- Hôpital Cochin (FR)
- University Hospital Zurich (CH)
- University of Bari Aldo Moro (IT)
- University of Florence (IT)
- University of Regensburg (DE)
- Charité – Universitätsmedizin Berlin (DE)
- University of the Witwatersrand (ZA)
- Johns Hopkins University (US)
- Unidade Local de Saúde de Coimbra (PT)
- University of Verona (IT)
- Lund University (SE)
- University Medical Center Utrecht (NL)
- Medical University of Vienna (AT)
- Sapienza University of Rome (IT)
- University Hospital of Geneva (CH)
- Medical University of Silesia (PL)
- The Royal Free Hospital (GB)
- Instituto Português de Reumatologia (PT)
- Universitätsklinikum Tübingen (DE)
- Hôpital Claude Huriez (FR)
- University of Michigan (US)
- Iuliu Hațieganu University of Medicine and Pharmacy (RO)
- Charles University (CZ)
- Corresponding author
- Walker, Ulrich A
- ORCID
- Venue
- Annals of the Rheumatic Diseases
- Publisher
- Elsevier BV
- Published
- 2010-10
- Volume
- 69
- Issue
- 10
- Pages
- 1809-1815
- ISSN
- 0003-4967 (ISSN-L)
- OpenAlex
- W2169645723
- Cited by
- 1,443
- References
- 31
- Citations by year
- 2026: 86 · 2025: 105 · 2024: 151 · 2023: 127 · 2022: 142 · 2021: 119 · 2020: 135 · 2019: 104 · 2018: 85 · 2017: 82 · 2016: 62 · 2015: 59 · 2014: 49 · 2013: 54 · 2012: 48
- FWCI
- 29.24
- Type
- journal article
- Language
- en
- Open access
- green
- www.zora.uzh.ch
- Licence
- Locations
- Annals of the Rheumatic Diseases (publishedVersion)
- PubMed (publishedVersion)
- Zurich Open Repository and Archive (University of Zurich) (acceptedVersion, other-oa)
- University of Regensburg Publication Server (University of Regensburg) (acceptedVersion)
- Archivio Istituzionale della Ricerca - Università degli Studi della Campania "Luigi Vanvitelli" (submittedVersion)
- IRIS Research product catalog (Sapienza University of Rome) (submittedVersion)
- Lithuanian University of Health Sciences (submittedVersion)
- Lund University Publications (Lund University) (submittedVersion)
- HighWire Press Open Archive (submittedVersion)
- Radboud Repository (Radboud University) (submittedVersion)
- CINECA IRIS Institutional Research Information System (University of Bari Aldo Moro) (submittedVersion)
- Archive ouverte UNIGE (University of Geneva) (submittedVersion)
- Archive ouverte UNIGE (University of Geneva) (submittedVersion, cc-by)
- Padua Research Archive (University of Padova) (submittedVersion)
- Zurich Open Repository and Archive (University of Zurich) (submittedVersion, other-oa)
- Zurich Open Repository and Archive (University of Zurich)
- Topics
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
- Keywords
- systemic sclerosis, mortality, causes of death, risk factors, pulmonary fibrosis, pulmonary arterial hypertension, heart failure, cardiac arrhythmias, infection, cancer, proteinuria, Raynaud's phenomenon, modified Rodnan skin score, EUSTAR cohort
- MeSH terms
- Adult, Aged, Epidemiologic Methods, Female, Heart Diseases, Gastrointestinal Hemorrhage, Humans, Lung Diseases, Male, Middle Aged, Neoplasms, Pneumonia, Prognosis, Scleroderma, Systemic, Comorbidity, Sepsis
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