Factors associated with shorter time to death and causes of mortality among deceased patients with systemic sclerosis: a multicentre retrospective study

OBJECTIVE: To describe causes of death and identify clinical factors associated with shorter time to death among deceased patients with systemic sclerosis (SSc) in Turkey. METHOD: This retrospective multicentre study screened hospital databases across 19 tertiary rheumatology centres between 2004 and 2024 for deceased patients aged ≥16 years with ICD-10 codes beginning with M34. Eligibility was confirmed according to the 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria; 266 of 294 identified patients were included. Dates of death were verified through the national death registry. Baseline clinical features, organ involvement, and key echocardiographic measures were extracted from medical records. Time to death was defined as the interval from diagnosis to death. Cause-specific Cox regression models were constructed for SSc-related and non-SSc-related deaths. RESULTS: Of 266 deaths, 122 (45.9%) were SSc related and 144 (54.1%) non-SSc related. Among SSc-related deaths, interstitial lung disease (ILD) (50.0%, n = 61) and pulmonary arterial hypertension (PAH) (42.6%, n = 52) predominated. Among non-SSc-related deaths, cardiovascular causes (38.9%, n = 56), infections (33.3%, n = 48), and malignancy (19.4%, n = 28) were most frequent. In multivariable models, older age at diagnosis, scleroderma renal crisis, palpitations, ILD, and higher echocardiographic pulmonary artery systolic pressure were independently associated with shorter time to SSc-related death, whereas older age at diagnosis and malignancy were associated with shorter time to non-SSc-related death. CONCLUSION: Pulmonary complications predominated among SSc-related deaths, whereas cardiovascular disease, infections, and malignancy were the leading contributors to non-SSc-related mortality. These findings support systematic cardiopulmonary assessment and comorbidity management.

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Journal
Scandinavian Journal of Rheumatology
Published
2026-08-20
DOI
https://doi.org/10.1080/03009742.2026.2700105
Primary Topic
Systemic Sclerosis and Related Diseases
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article
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article

Factors associated with shorter time to death and causes of mortality among deceased patients with systemic sclerosis: a multicentre retrospective study

ZA Akar, Ediz Dalkılıç, Sezgin Zontul, Burak Okyar et al.
Scandinavian Journal of Rheumatology
Systemic Sclerosis and Related Diseases
article

Factors associated with shorter time to death and causes of mortality among deceased patients with systemic sclerosis: a multicentre retrospective study

ZA Akar, Ediz Dalkılıç, Sezgin Zontul, Burak Okyar, Remzi Çevik, Lütfi Akyol, Halil Harman, Özlem Kudaş, Burcu Yağız, Çiğdem Çetin, Bünyamin Kısacık, Hakan Babaoğlu, Belkıs Nihan Coşkun, Gözde Yıldırım Çetin, Ahmet Karataş, Fatih Albayrak, Yavuz Pehlivan, Osman Cüre, Dilek Tezcan, Servet Yolbaş, Uğur Karasu, Süleyman Serdar Koca, Mehmet Balcı, Enes KESKİN, Orhan Zengin, Adem Küçük, Selime Ermurat, Ayşe Nur Bayındır Akbaş, Zeynep Yağbasan, Saliha Sunkak, Elif İnanç, İ Gunduz, A Gocer
article en

Abstract

OBJECTIVE: To describe causes of death and identify clinical factors associated with shorter time to death among deceased patients with systemic sclerosis (SSc) in Turkey. METHOD: This retrospective multicentre study screened hospital databases across 19 tertiary rheumatology centres between 2004 and 2024 for deceased patients aged ≥16 years with ICD-10 codes beginning with M34. Eligibility was confirmed according to the 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria; 266 of 294 identified patients were included. Dates of death were verified through the national death registry. Baseline clinical features, organ involvement, and key echocardiographic measures were extracted from medical records. Time to death was defined as the interval from diagnosis to death. Cause-specific Cox regression models were constructed for SSc-related and non-SSc-related deaths. RESULTS: Of 266 deaths, 122 (45.9%) were SSc related and 144 (54.1%) non-SSc related. Among SSc-related deaths, interstitial lung disease (ILD) (50.0%, n = 61) and pulmonary arterial hypertension (PAH) (42.6%, n = 52) predominated. Among non-SSc-related deaths, cardiovascular causes (38.9%, n = 56), infections (33.3%, n = 48), and malignancy (19.4%, n = 28) were most frequent. In multivariable models, older age at diagnosis, scleroderma renal crisis, palpitations, ILD, and higher echocardiographic pulmonary artery systolic pressure were independently associated with shorter time to SSc-related death, whereas older age at diagnosis and malignancy were associated with shorter time to non-SSc-related death. CONCLUSION: Pulmonary complications predominated among SSc-related deaths, whereas cardiovascular disease, infections, and malignancy were the leading contributors to non-SSc-related mortality. These findings support systematic cardiopulmonary assessment and comorbidity management.

Scandinavian Journal of Rheumatology
Dicle University (TR), Bursa Uludağ Üni̇versi̇tesi̇ (TR), Fırat University (TR), Recep Tayyip Erdoğan University (TR), Bilkent University (TR), University of Health Science (KH), Inonu University (TR), Gülhane Askerî Tıp Akademisi (TR), Necmettin Erbakan University (TR), Diyarbakır Askeri Hastanesi (TR), Kayseri Eğitim ve Araştırma Hastanesi (TR), Başkent University Hospital (TR), Sağlık Bilimleri Üniversitesi (TR), Diyarbakır Gazi Yaşargil Eğitim ve Araştırma Hastanesi (TR), Sanko University (TR), Denizli Devlet Hastanesi (TR), S.B.Ü. Bursa Yüksek İhtisas Eğitim ve Araştırma Hastanesi (TR), Kahramanmaraş Sütçü İmam University (TR), Pamukkale University (TR), Gaziantep University (TR), University of Health Sciences Antigua (AG)
Good health and well-being
Openalex Percentile: Top 10%
Systemic Sclerosis and Related Diseases
DOISourceOpenAlex
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