The Performance of Pulmonary Function Tests in Predicting Systemic Sclerosis—Interstitial Lung Disease in the European Scleroderma Trial and Research Database
Gemma Lepri,
Cosimo Bruni,
Lorenzo Tofani,
Alberto Moggi-Pignone,
Martina Orlandi,
Sara Tomassetti,
Michael Hughes,
Francesco Del Galdo,
Rosaria Irace,
Oliver Distler,
Valeria Riccieri,
Yannick Allanore,
Ana Maria Gheorghiu,
Elise Siegert,
Jeska De Vries-Bouwstra,
Eric Hachulla,
Mohammed Tikly,
Nemanja Damjanov,
Francois Spertini,
Luc Mouthon,
Anna-Maria Hoffmann-Vold,
Armando Gabrielli,
Serena Guiducci,
Marco Matucci-Cerinic,
Daniel Furst,
Silvia Bellando-Randone,
EUSTAR Collaborators
Affiliations
Gemma Lepri
Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy
Cosimo Bruni
Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy
Lorenzo Tofani
Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy
Alberto Moggi-Pignone
Division of Internal Medicine, AOU Careggi, University of Florence, 50121 Florence, Italy
Martina Orlandi
Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy
Sara Tomassetti
Interventional Pulmonology Unit, AOU Careggi, University of Florence, 50121 Florence, Italy
Michael Hughes
Department of Rheumatology, Sheffield Teaching Hospitals NHS Foundation Trust, Royal Hallamshire Hospital, Sheffield S10 2JF, UK
Francesco Del Galdo
Raynaud’s and Scleroderma Programme, NIHR Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds LS2 9JT, UK
Rosaria Irace
Rheumatology Unit, Department of Precision Medicine, University of Campania “Luigi Vanvitelli”, 81100 Naples, Italy
Oliver Distler
Department of Rheumatology, University Hospital Zurich, University of Zurich, 8006 Zurich, Switzerland
Valeria Riccieri
Rheumatology Unit, “Sapienza” University, 00185 Rome, Italy
Yannick Allanore
Rheumatology Department, Hopital Cochin, University of Paris, 75019 Paris, France
Ana Maria Gheorghiu
Internal Medicine & Rheumatology Department, Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, 050474 Bucharest, Romania
Elise Siegert
Rheumatology, Charite University Hospital, 10117 Berlin, Germany
Jeska De Vries-Bouwstra
Department of Rheumatology, Leiden University Medical Center, 2333 ZA Leiden, The Netherlands
Eric Hachulla
Service de Médecine Interne, Centre Hospitalier Universitaire, 59000 Lille, France
Mohammed Tikly
Department of Internal Medicine, Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, University of the Witwatersrand, Johannesburg 1864, South Africa
Nemanja Damjanov
Institute of Rheumatology, University Belgrade Medical School, 11000 Belgrade, Serbia
Francois Spertini
Service Immunologie et Allergie, CHUV, 1005 Lausanne, Switzerland
Luc Mouthon
National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, University Paris Descartes, 75006 Paris, France
Anna-Maria Hoffmann-Vold
Department of Rheumatology, Oslo University Hospital, 0372 Oslo, Norway
Armando Gabrielli
Department of Clinical and Molecular Science, Università Politecninca delle Marche, 60121 Ancona, Italy
Serena Guiducci
Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy
Marco Matucci-Cerinic
Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy
Daniel Furst
Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy
Silvia Bellando-Randone
Division of Rheumatology, AOU Careggi, University of Florence, 50121 Florence, Italy
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.
