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

Publikation: Beitrag in FachzeitschriftForschungsartikelBeigetragenBegutachtung

Beitragende

  • Pia Moinzadeh - , Universitätsklinikum Köln (Autor:in)
  • Francesco Bonella - , Universität Duisburg-Essen (Autor:in)
  • Max Oberste - , Universitätsklinikum Köln (Autor:in)
  • Jithmi Weliwitage - , Universitätsklinikum Köln (Autor:in)
  • Nobert Blank - , Universitätsklinikum Heidelberg (Autor:in)
  • Gabriela Riemekasten - , Universität zu Lübeck (Autor:in)
  • Ulf Müller-Ladner - , Kerckhoff Klinik (Autor:in)
  • Jörg Henes - , Universitätsklinikum Tübingen (Autor:in)
  • Elise Siegert - , Charité – Universitätsmedizin Berlin (Autor:in)
  • Claudia Günther - , Klinik und Poliklinik für Dermatologie, Universitätsklinikum Carl Gustav Carus Dresden (Autor:in)
  • Ina Kötter - , Universitätsklinikum Hamburg-Eppendorf (UKE) (Autor:in)
  • Christiane Pfeiffer - , Universitätsklinikum Ulm (Autor:in)
  • Marc Schmalzing - , Universitätsklinikum Würzburg (Autor:in)
  • Gabriele Zeidler - , Center for Rheumatology Brandenburg (Autor:in)
  • Peter Korsten - , Universitätsmedizin Göttingen (Autor:in)
  • Laura Susok - , Katholisches Klinikum Bochum gGmbH (Autor:in)
  • Aaron Juche - , Immanuel Krankenhaus Berlin (Autor:in)
  • Margitta Worm - , Klinik und Poliklinik für Dermatologie (Autor:in)
  • Ilona Jandova - , Universitätsklinikum Freiburg (Autor:in)
  • Jan Ehrchen - , Universitätsklinikum Münster (Autor:in)
  • Cord Sunderkötter - , Universitätsklinikum Halle (Autor:in)
  • Gernot Keyßer - , Universitätsklinikum Halle (Autor:in)
  • Andreas Ramming - , Friedrich-Alexander-Universität Erlangen-Nürnberg (Autor:in)
  • Tim Schmeiser - , Cellitinnen-Krankenhaus St. Josef Wuppertal-Elberfeld (Autor:in)
  • Alexander Kreuter - , Universität Witten/Herdecke (Autor:in)
  • Hanns-Martin Lorenz - , Universitätsklinikum Heidelberg (Autor:in)
  • Nicolas Hunzelmann - , Universitätsklinikum Köln (Autor:in)
  • Michael Kreuter - , Universitätsmedizin Mainz (Autor:in)

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

OriginalspracheEnglisch
Seiten (von - bis)132-145
Seitenumfang14
FachzeitschriftChest
Jahrgang165
Ausgabenummer1
PublikationsstatusVeröffentlicht - Jan. 2024
Peer-Review-StatusJa

Externe IDs

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

Schlagworte

Schlagwörter

  • 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