Impact of Systemic Sclerosis-Associated Interstitial Lung Disease With and Without Pulmonary Hypertension on Survival: A Large Cohort Study of the German Network for Systemic Sclerosis

Research output: Contribution to journalResearch articleContributedpeer-review

Contributors

  • Pia Moinzadeh - , Uniklinik Köln (Author)
  • Francesco Bonella - , University of Duisburg-Essen (Author)
  • Max Oberste - , Uniklinik Köln (Author)
  • Jithmi Weliwitage - , Uniklinik Köln (Author)
  • Nobert Blank - , University Hospital Heidelberg (Author)
  • Gabriela Riemekasten - , University of Lübeck (Author)
  • Ulf Müller-Ladner - , Kerckhoff Clinic (Author)
  • Jörg Henes - , University Hospital Tübingen (Author)
  • Elise Siegert - , Charité – Universitätsmedizin Berlin (Author)
  • Claudia Günther - , Department of Dermatology, University Hospital Carl Gustav Carus Dresden (Author)
  • Ina Kötter - , University Hospital Hamburg Eppendorf (Author)
  • Christiane Pfeiffer - , Ulm University Medical Center (Author)
  • Marc Schmalzing - , University Hospital of Würzburg (Author)
  • Gabriele Zeidler - , Center for Rheumatology Brandenburg (Author)
  • Peter Korsten - , University Medical Center Göttingen (Author)
  • Laura Susok - , Catholic Hospital Bochum gGmbH (Author)
  • Aaron Juche - , Immanuel Hospital Berlin (Author)
  • Margitta Worm - , Department of Dermatology (Author)
  • Ilona Jandova - , University Medical Center Freiburg (Author)
  • Jan Ehrchen - , University Hospital Münster (Author)
  • Cord Sunderkötter - , Martin Luther University Hospital (Author)
  • Gernot Keyßer - , Martin Luther University Hospital (Author)
  • Andreas Ramming - , Friedrich-Alexander University Erlangen-Nürnberg (Author)
  • Tim Schmeiser - , St. Josef Hospital Wuppertal-Elberfeld (Author)
  • Alexander Kreuter - , Witten/Herdecke University (Author)
  • Hanns-Martin Lorenz - , University Hospital Heidelberg (Author)
  • Nicolas Hunzelmann - , Uniklinik Köln (Author)
  • Michael Kreuter - , University Medical Center Mainz (Author)

Abstract

BACKGROUND: Pulmonary involvement is the leading cause of death in systemic sclerosis (SSc) and may manifest as interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), or in combination of both (ILD with pulmonary hypertension [ILD-PH]). The aim of this analysis was to determine prevalence, clinical characteristics, and survival of these different forms within the registry of the German Network for Systemic Sclerosis.

RESEARCH QUESTION: Does SSc-associated ILD-PH or ILD without PH affect survival differently, and are there any risk factors that have an additional impact?

STUDY DESIGN AND METHODS: Clinical data of 5,831 patients with SSc were collected in the German Network for Systemic Sclerosis registry. Kaplan-Meier estimates were used to compare overall survival in patients with SSc-associated ILD-PH and ILD without PH with patients without pulmonary involvement and those with PAH. The Cox proportional hazard model was used to analyze the influence of pulmonary involvement and other potential predictors on patient survival.

RESULTS: Clinical data of 3,257 patients with a mean follow-up time of 3.45 ± 1.63 years have been included in our analysis. At baseline, ILD was present in 34.5%, whereas PH without ILD had a lower prevalence with 4.5%. At the end of follow-up, 47.6% of patients with SSc had ILD, 15.2% had ILD-PH, and 6.5% had PAH. ILD was more frequent in the diffuse cutaneous form (57.3%), whereas PAH did not differ significantly between SSc subtypes. Significant differences in baseline characteristics between PAH vs ILD-PH vs ILD without PH were found for age at diagnosis, sex, SSc subsets, antibody status, FVC, diffusing capacity of the lung for carbon monoxide, and therapy. Overall survival at 5 years was 96.4% for patients without pulmonary involvement and differed significantly between patients with ILD without PH, PAH, and being worst in patients with ILD-PH. Female sex (hazard ratio [HR], 0.3), higher BMI (HR, 0.9), and higher diffusing capacity of the lung for carbon monoxide values (HR, 0.98) were associated with a lower mortality risk.

INTERPRETATION: ILD is the most prevalent pulmonary involvement in SSc, whereas the combination of ILD and PH is associated with the most detrimental survival.

Details

Original languageEnglish
Pages (from-to)132-145
Number of pages14
JournalChest
Volume165
Issue number1
Publication statusPublished - Jan 2024
Peer-reviewedYes

External IDs

Scopus 85180293046
ORCID /0000-0002-4330-1861/work/151982046

Keywords

Keywords

  • Humans, Female, Hypertension, Pulmonary/epidemiology, Cohort Studies, Carbon Monoxide, Scleroderma, Systemic/diagnosis, Lung Diseases, Interstitial/etiology, Familial Primary Pulmonary Hypertension/complications, Pulmonary Arterial Hypertension/complications