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Lepri, G.; Bruni, C.; Tofani, L.; Moggi-Pignone, A.; Orlandi, M.; Tomassetti, S.; ... ; EUSTAR Collaborators (2024)

The performance of pulmonary function tests in predicting systemic sclerosis: interstitial lung disease in the European scleroderma trial and research database

Article / Letter to editor
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 (Delta) 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) (t(2)) months. Results: 474/17805 patients were eligible for the study (403 females); 46 (9.7%) developed ILD at t(2). Positivity for anti-topoisomerase antibodies (117 patients) showed an association with ILD development at t(2) (p = 0.0031). Neither the mean t0 to t1 change (Delta) of DLCO nor the mean t(0) to t(1) FVC Delta predicted the appearance of ILD at t(2). Investigating the possible role of baseline DLCO and FVC values in predicting ILD appearance after 24 (+/- 4) months, we observed a moderate predictive...Show moreBackground 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 (Delta) 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) (t(2)) months. Results: 474/17805 patients were eligible for the study (403 females); 46 (9.7%) developed ILD at t(2). Positivity for anti-topoisomerase antibodies (117 patients) showed an association with ILD development at t(2) (p = 0.0031). Neither the mean t0 to t1 change (Delta) of DLCO nor the mean t(0) to t(1) FVC Delta predicted the appearance of ILD at t(2). 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 t(0) 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.Show less
computed tomography (CT)
interstitial lung disease
scleroderma
pulmonary function test
All authors
Lepri, G.; Bruni, C.; Tofani, L.; Moggi-Pignone, A.; Orlandi, M.; Tomassetti, S.; Hughes, M.; Galdo, F. del; Irace, R.; Distler, O.; Riccieri, V.; Allanore, Y.; Gheorghiu, A.M.; Siegert, E.; Vries-Bouwstra, J. de; Hachulla, E.; Tikly, M.; Damjanov, N.; Spertini, F.; Mouthon, L.; Hoffmann-Vold, A.M.; Gabrielli, A.; Guiducci, S.; Matucci-Cerinic, M.; Furst, D.; Bellando-Randone, S.; EUSTAR Collaborators
Date
2024-02-01
Journal
Diagnostics
Volume
14
Issue
3
DOI
doi:10.3390/diagnostics14030295