Lung vascular changes as biomarkers of severity in systemic sclerosis–associated interstitial lung disease

Author:

Bruni Cosimo12ORCID,Occhipinti Mariaelena3,Pienn Michael4,Camiciottoli Gianna56,Bartolucci Maurizio7,Bosello Silvia Laura8,Payer Christian9,Bálint Zoltán10,Larici Anna Rita1112,Tottoli Alessandra1,Tofani Lorenzo113,De Lorenzis Enrico8ORCID,Lepri Gemma1,Bellando-Randone Silvia1,Spinella Amelia14,Giuggioli Dilia14,Masini Francesco15,Cuomo Giovanna15,Lavorini Federico56,Colagrande Stefano16,Olschewski Horst417,Matucci-Cerinic Marco118

Affiliation:

1. Division of Rheumatology, Deptartment of Experimental and Clinical Medicine, Azienda Ospedaliero Universitaria Careggi , Firenze, Italy

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

3. Radiomics , Liege, Belgium

4. Ludwig Boltzmann Institute for Lung Vascular Research , Graz, Austria

5. Department of Biomedical, Experimental and Clinical Sciences, University of Florence

6. Department of CardioThoracoVascular, Careggi University Hospital , Florence

7. Department of Radiology, Santo Stefano Hospital , Prato

8. Department of Rheumatology, Fondazione Policlinico Universitario A. Gemelli—IRCCS , Roma, Italy

9. Institute of Computer Graphics and Vision, Graz University of Technology , Graz, Austria

10. Faculty of Physics, Babeș-Bolyai University , Cluj-Napoca, Romania

11. Department of Radiological and Hematological Sciences, Section of Radiology, Università Cattolica del Sacro Cuore

12. Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, Fondazione Policlinico Universitario “A. Gemelli” IRCCS , Rome

13. Department of Statistics, Computer Science, Applications, University of Florence , Florence

14. Scleroderma Unit, Rheumatology Unit, Department of Medical and Surgical Sciences for Children and Adults, University of Modena and Reggio Emilia, Azienda Ospedaliero Universitaria di Modena , Modena

15. Department of Medicine of Precision, University of Campania L. Vanvitelli , Naples

16. Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit n. 2, Careggi University Hospital, University of Florence , Florence, Italy

17. Division of Pulmonology, Department of Internal Medicine, Medical University of Graz , Graz, Austria

18. Unit of Immunology, Rheumatology, Allergy and Rare diseases (UnIRAR), IRCCS San Raffaele Hospital , Milan, Italy

Abstract

Abstract Objectives It has recently become possible to assess lung vascular and parenchymal changes quantitatively in thoracic CT images using automated software tools. We investigated the vessel parameters of patients with SSc, quantified by CT imaging, and correlated them with interstitial lung disease (ILD) features. Methods SSc patients undergoing standard of care pulmonary function testing and CT evaluation were retrospectively evaluated. CT images were analysed for ILD patterns and total pulmonary vascular volume (PVV) extents with Imbio lung texture analysis. Vascular analysis (volumes, numbers and densities of vessels, separating arteries and veins) was performed with an in-house developed software. A threshold of 5% ILD extent was chosen to define the presence of ILD, and commonly used cut-offs of lung function were adopted. Results A total of 79 patients [52 women, 40 ILD, mean age 56.2 (s.d. 14.2) years, total ILD extent 9.5 (10.7)%, PVV/lung volume % 2.8%] were enrolled. Vascular parameters for total and separated PVV significantly correlated with functional parameters and ILD pattern extents. SSc-associated ILD (SSc-ILD) patients presented with an increased number and volume of arterial vessels, in particular those between 2 and 4 mm of diameter, and with a higher density of arteries and veins of <6 mm in diameter. Considering radiological and functional criteria concomitantly, as well as the descriptive trends from the longitudinal evaluations, the normalized PVVs, vessel numbers and densities increased progressively with the increase/worsening of ILD extent and functional impairment. Conclusion In SSc patients CT vessel parameters increase in parallel with ILD extent and functional impairment, and may represent a biomarker of SSc-ILD severity.

Funder

GILS

Gruppo Italiano Lotta Sclerodermia

FIRA

Fondazione Italiana Ricerca sull’Artrite

Gruppo Italiano Lotta alla Sclerodermia

European Scleroderma Trials and Research Group

Scleroderma Clinical Trials Consortium

AbbVie

Publisher

Oxford University Press (OUP)

Subject

Pharmacology (medical),Rheumatology

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