The Performance of Pulmonary Function Tests in Predicting Systemic Sclerosis—Interstitial Lung Disease in the European Scleroderma Trial and Research Database

Diagnostics, vol. 14, pp. 295

Abstract

Background and Objectives: In SSc, ILD is a major cause of morbidity and mortality. We aimed to investigate the performance of DLCO (diffusing capacity of lung carbon monoxide) and FVC (forced vital capacity) delta change (Δ) and baseline values in predicting the development of SSc-ILD. Methods: Longitudinal data of DLCO, FVC, and ILD on the HRCT of SSc patients from the EUSTAR database were evaluated at baseline (t0) and after 12 (±4) (t1) and 24 (±4) (t2) months. Results: 474/17805 patients were eligible for the study (403 females); 46 (9.7%) developed ILD at t2. Positivity for anti-topoisomerase antibodies (117 patients) showed an association with ILD development at t2 (p = 0.0031). Neither the mean t0 to t1 change (Δ) of DLCO nor the mean t0 to t1 FVCΔ predicted the appearance of ILD at t2. Investigating the possible role of baseline DLCO and FVC values in predicting ILD appearance after 24 (±4) months, we observed a moderate predictive capability of t0 DLCO < 80%, stronger than that of FVC < 80%. Conclusions: We suggest that an impaired baseline DLCO may be predictive of the appearance of ILD after 2 years of follow-up. This result advances the hypothesis that a reduction in gas exchange may be considered an early sign of lung involvement. However, further rigorous studies are warranted to understand the predictive role of DLCO evaluation in the course of SSc.

Authors 26

1. Gemma Lepri corresponding

   Azienda Ospedaliero-Universitaria Careggi · University of Florence

   Affiliation as printed

   Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy

2. Cosimo Bruni

   University of Zurich · University Hospital Zurich · Azienda Ospedaliero-Universitaria Careggi · University of Florence

   Affiliation as printed

   Department of Rheumatology, University Hospital Zurich, University of Zurich, 8006 Zurich, Switzerland

   Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy

3. Lorenzo Tofani

   Azienda Ospedaliero-Universitaria Careggi · University of Florence

   Affiliation as printed

   Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy

4. Alberto Moggi Pignone

   Azienda Ospedaliero-Universitaria Careggi · University of Florence

   Affiliation as printed

   Division of Internal Medicine, AOU Careggi, University of Florence, 50121 Florence, Italy

5. Martina Orlandi

   Azienda Ospedaliero-Universitaria Careggi · University of Florence

   Affiliation as printed

   Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy

6. Sara Tomassetti

   Azienda Ospedaliero-Universitaria Careggi · University of Florence

   Affiliation as printed

   Interventional Pulmonology Unit, AOU Careggi, University of Florence, 50121 Florence, Italy

7. Michael J. Hughes

   Royal Hallamshire Hospital · Sheffield Teaching Hospitals NHS Foundation Trust

   Affiliation as printed

   Department of Rheumatology, Sheffield Teaching Hospitals NHS Foundation Trust, Royal Hallamshire Hospital, Sheffield S10 2JF, UK

8. Francesco Del Galdo

   University of Leeds

   Affiliation as printed

   Raynaud’s and Scleroderma Programme, NIHR Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds LS2 9JT, UK

   Raynaud's and Scleroderma Programme, NIHR Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds LS2 9JT, UK

9. Rosaria Irace

   University of Campania "Luigi Vanvitelli"

   Affiliation as printed

   Rheumatology Unit, Department of Precision Medicine, University of Campania “Luigi Vanvitelli”, 81100 Naples, Italy

   Rheumatology Unit, Department of Precision Medicine, University of Campania "Luigi Vanvitelli", 81100 Naples, Italy

10. Oliver Distler

    University of Zurich · University Hospital Zurich

    Affiliation as printed

    Department of Rheumatology, University Hospital Zurich, University of Zurich, 8006 Zurich, Switzerland

11. Valeria Riccieri

    Sapienza University of Rome

    Affiliation as printed

    Rheumatology Unit, “Sapienza” University, 00185 Rome, Italy

    Rheumatology Unit, "Sapienza" University, 00185 Rome, Italy

12. Yannick Allanore

    Université Paris Cité · Hôpital Cochin

    Affiliation as printed

    Rheumatology Department, Hopital Cochin, University of Paris, 75019 Paris, France

13. Ana Maria Gheorghiu

    Carol Davila University of Medicine and Pharmacy

    Affiliation as printed

    Internal Medicine & Rheumatology Department, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania

14. Elise Siegert

    Charité - Universitätsmedizin Berlin

    Affiliation as printed

    Rheumatology, Charite University Hospital, 10117 Berlin, Germany

15. Jeska de Vries‐Bouwstra Aachen Department of Rheumatology, Leiden University Medical Center

    Leiden University Medical Center

    Affiliation as printed

    Department of Rheumatology, Leiden University Medical Center, 2333 ZA Leiden, The Netherlands

16. Eric HACHULLA

    Centre Hospitalier Universitaire de Lille

    Affiliation as printed

    Service de Médecine Interne, Centre Hospitalier Universitaire, 59000 Lille, France

17. Mohammed Tikly

    University of the Witwatersrand · Chris Hani Baragwanath Hospital

    Affiliation as printed

    Department of Internal Medicine, Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg 1864, South Africa

18. Nemanja S Damjanov

    University of Belgrade

    Affiliation as printed

    Institute of Rheumatology, University Belgrade Medical School, 11000 Belgrade, Serbia

19. François Spertini

    Centre Hospitalier Universitaire Vaudois · Hôpital Orthopédique de la Suisse Romande

    Affiliation as printed

    Service Immunologie et Allergie, CHUV, 1005 Lausanne, Switzerland

20. Luc Mouthon

    Hôpital Cochin · Université Paris Descartes

    Affiliation as printed

    National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, University Paris Descartes, 75006 Paris, France

21. Anna‐Maria Hoffmann‐Vold

    Oslo University Hospital

    Affiliation as printed

    Department of Rheumatology, Oslo University Hospital, 0372 Oslo, Norway

22. Armando Gabrielli

    Marche Polytechnic University

    Affiliation as printed

    Department of Clinical and Molecular Science, Università Politecninca delle Marche, 60121 Ancona, Italy

23. Serena Guiducci

    Azienda Ospedaliero-Universitaria Careggi · University of Florence

    Affiliation as printed

    Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy

24. Marco Matucci‐Cerinic

    Azienda Ospedaliero-Universitaria Careggi · IRCCS Ospedale San Raffaele · University of Florence

    Affiliation as printed

    Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy

    Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, 20132 Milan, Italy

25. Daniel E. Fürst

    University of California, Los Angeles · Azienda Ospedaliero-Universitaria Careggi · University of Florence

    Affiliation as printed

    Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy

    Division of Rheumatology, University California Los Angeles, Los Angeles, CA 90095, USA

26. Silvia Bellando-Randone

    Azienda Ospedaliero-Universitaria Careggi · University of Florence

    Affiliation as printed

    Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy