Hypochromic red cells as a prognostic indicator of survival among patients with systemic sclerosis screened for pulmonary hypertension.


Journal

Arthritis research & therapy
ISSN: 1478-6362
Titre abrégé: Arthritis Res Ther
Pays: England
ID NLM: 101154438

Informations de publication

Date de publication:
09 03 2023
Historique:
received: 15 11 2022
accepted: 26 02 2023
entrez: 9 3 2023
pubmed: 10 3 2023
medline: 14 3 2023
Statut: epublish

Résumé

Patients with systemic sclerosis (SSc) are frequently affected by iron deficiency, particularly those with pulmonary hypertension (PH). The first data indicate the prognostic importance of hypochromic red cells (% HRC) > 2% among patients with PH. Hence, the objective of our study was to investigate the prognostic value of % HRC in SSc patients screened for PH. In this retrospective, single-center cohort study, SSc patients with a screening for PH were enrolled. Clinical characteristics and laboratory and pulmonary functional parameters associated with the prognosis of SSc were analyzed using uni- and multivariable analysis. From 280 SSc patients screened, 171 could be included in the analysis having available data of iron metabolism (81% female, 60 ± 13 years of age, 77% limited cutaneous SSc, 65 manifest PH, and 73 pulmonary fibrosis). The patients were followed for 2.4 ± 1.8 (median 2.4) years. HRC > 2% at baseline was significantly associated with worse survival in the uni- (p = 0.018) and multivariable (p = 0.031) analysis independent from the presence of PH or pulmonary parenchymal manifestations. The combination of HRC > 2% and low diffusion capacity for carbon monoxide (DLCO) ≤ 65% predicted was significantly associated with survival (p < 0.0001). This is the first study reporting that HRC > 2% is an independent prognostic predictor of mortality and can possibly be used as a biomarker among SSc patients. The combination of HRC > 2% and DLCO ≤ 65% predicted could serve in the risk stratification of SSc patients. Larger studies are required to confirm these findings.

Sections du résumé

BACKGROUND
Patients with systemic sclerosis (SSc) are frequently affected by iron deficiency, particularly those with pulmonary hypertension (PH). The first data indicate the prognostic importance of hypochromic red cells (% HRC) > 2% among patients with PH. Hence, the objective of our study was to investigate the prognostic value of % HRC in SSc patients screened for PH.
METHODS
In this retrospective, single-center cohort study, SSc patients with a screening for PH were enrolled. Clinical characteristics and laboratory and pulmonary functional parameters associated with the prognosis of SSc were analyzed using uni- and multivariable analysis.
RESULTS
From 280 SSc patients screened, 171 could be included in the analysis having available data of iron metabolism (81% female, 60 ± 13 years of age, 77% limited cutaneous SSc, 65 manifest PH, and 73 pulmonary fibrosis). The patients were followed for 2.4 ± 1.8 (median 2.4) years. HRC > 2% at baseline was significantly associated with worse survival in the uni- (p = 0.018) and multivariable (p = 0.031) analysis independent from the presence of PH or pulmonary parenchymal manifestations. The combination of HRC > 2% and low diffusion capacity for carbon monoxide (DLCO) ≤ 65% predicted was significantly associated with survival (p < 0.0001).
CONCLUSION
This is the first study reporting that HRC > 2% is an independent prognostic predictor of mortality and can possibly be used as a biomarker among SSc patients. The combination of HRC > 2% and DLCO ≤ 65% predicted could serve in the risk stratification of SSc patients. Larger studies are required to confirm these findings.

Identifiants

pubmed: 36895026
doi: 10.1186/s13075-023-03020-y
pii: 10.1186/s13075-023-03020-y
pmc: PMC9997012
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

38

Informations de copyright

© 2023. The Author(s).

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Auteurs

Panagiota Xanthouli (P)

Centre for Pulmonary Hypertension, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Röntgenstrasse 1, D-69126, Heidelberg, Germany.
Translational Lung Research Centre Heidelberg (TLRC), Member of the German Centre for Lung Research (DZL), Heidelberg, Germany.
Department of Pneumology and Critical Care Medicine, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Heidelberg, Germany.
Division of Rheumatology, Department of Internal Medicine V: Hematology, Oncology and Rheumatology, University Hospital Heidelberg, Heidelberg, Germany.

Ojan Gordjani (O)

Centre for Pulmonary Hypertension, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Röntgenstrasse 1, D-69126, Heidelberg, Germany.

Nicola Benjamin (N)

Centre for Pulmonary Hypertension, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Röntgenstrasse 1, D-69126, Heidelberg, Germany.
Translational Lung Research Centre Heidelberg (TLRC), Member of the German Centre for Lung Research (DZL), Heidelberg, Germany.

Satenik Harutyunova (S)

Centre for Pulmonary Hypertension, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Röntgenstrasse 1, D-69126, Heidelberg, Germany.
Translational Lung Research Centre Heidelberg (TLRC), Member of the German Centre for Lung Research (DZL), Heidelberg, Germany.
Department of Pneumology and Critical Care Medicine, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Heidelberg, Germany.

Benjamin Egenlauf (B)

Centre for Pulmonary Hypertension, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Röntgenstrasse 1, D-69126, Heidelberg, Germany.
Translational Lung Research Centre Heidelberg (TLRC), Member of the German Centre for Lung Research (DZL), Heidelberg, Germany.
Department of Pneumology and Critical Care Medicine, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Heidelberg, Germany.

Alberto M Marra (AM)

Department of Translational Medical Sciences, "Federico II" University and School of Medicine, Naples, Italy.

Simon Haas (S)

Centre for Pulmonary Hypertension, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Röntgenstrasse 1, D-69126, Heidelberg, Germany.
Translational Lung Research Centre Heidelberg (TLRC), Member of the German Centre for Lung Research (DZL), Heidelberg, Germany.

Nicklas Milde (N)

Centre for Pulmonary Hypertension, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Röntgenstrasse 1, D-69126, Heidelberg, Germany.

Norbert Blank (N)

Division of Rheumatology, Department of Internal Medicine V: Hematology, Oncology and Rheumatology, University Hospital Heidelberg, Heidelberg, Germany.

Hanns-Martin Lorenz (HM)

Division of Rheumatology, Department of Internal Medicine V: Hematology, Oncology and Rheumatology, University Hospital Heidelberg, Heidelberg, Germany.

Christoph Fiehn (C)

Unit for Rheumatology and Clinical Immunology, Medical Centre Baden-Baden, Baden-Baden, Germany.

Silvia Ulrich (S)

Department of Pulmonology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.

Oliver Distler (O)

Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.

Ekkehard Grünig (E)

Centre for Pulmonary Hypertension, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Röntgenstrasse 1, D-69126, Heidelberg, Germany.
Translational Lung Research Centre Heidelberg (TLRC), Member of the German Centre for Lung Research (DZL), Heidelberg, Germany.
Department of Pneumology and Critical Care Medicine, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Heidelberg, Germany.

Christina A Eichstaedt (CA)

Centre for Pulmonary Hypertension, Thoraxklinik Heidelberg gGmbH at Heidelberg University Hospital, Röntgenstrasse 1, D-69126, Heidelberg, Germany. christina.eichstaedt@med.uni-heidelberg.de.
Translational Lung Research Centre Heidelberg (TLRC), Member of the German Centre for Lung Research (DZL), Heidelberg, Germany. christina.eichstaedt@med.uni-heidelberg.de.
Laboratory for Molecular Genetic Diagnostics, Institute of Human Genetics, Heidelberg University, Heidelberg, Germany. christina.eichstaedt@med.uni-heidelberg.de.

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