The incidence of interstitial lung disease in patients with systemic sclerosis: rate, risk factors and prognostic implications in a EUSTAR cohort analysis (CP 133)
Por:
Petelytska, L, Tofani, L, Velauthapillai, A, Dobrota, R, Becker, MO, Mihai, C, Muraru, S, Elhai, M, Jordan, S, Hachulla, E, Müller-Ladner, U, Siegert, E, Allanore, Y, Riemekasten, G, Bergmann, C, Becvar, R, Solanki, K, Anic, B, Szabo, I, Stamenkovic, B, Distler, J, Carreira, PE, Cuomo, G, Castellví, I, Balbir-Gurman, A, Airo, P, Litinsky, I, Saketkoo, LA, Bertaccini, B, Vonk, M, de Vries-Bouwstra, J, Hoffmann-Vold, AM, Matucci-Cerinic, M, Distler, O, Bruni, C
Publicada:
1 abr 2026
Ahead of Print:
1 abr 2026
Resumen:
Objectives: Interstitial lung disease (ILD) carries significant morbidity and mortality risk in systemic sclerosis (SSc). We aimed to estimate the incidence of new-onset SSc-ILD and the associated risk factors, as well as its impact on the prognosis. Methods: Patients classified as having SSc, with the absence of ILD signs on high-resolution computed tomography (HRCT) at baseline and having at least 1 follow-up visit with available HRCT data, were selected. SSc-ILD incidence was calculated as a rate per 100 person-years. Predictors of new-onset ILD and risk factors for ILD progression and mortality were chosen according to the literature and expert opinion. Risk factors for new-onset ILD, as well as its prognostic impact on ILD progression and mortality, were tested by generalised logistic estimating equation and Cox regression models, respectively. Results: Among 5331 patients with SSc with negative baseline HRCT, the incidence of new-onset ILD was 3.83 cases per 100 person-years. Notably, there was a continuous detection of new ILD onset up to 10 years from baseline. Risk factors for new-onset ILD included New York Heart Association stage >= 2, muscle weakness, high inflammatory markers, and SSc-specific autoantibodies, but not disease duration. Despite a lower risk of ILD progression compared with prevalent ILD diagnosed at baseline, incident ILD still carried an increased risk for mortality, which was almost double when compared with ILD-negative cases. Conclusions: Patients with SSc should be considered for regular screening following a negative baseline HRCT, in particular when carrying high-risk features for new ILD onset, given its incidence and prognostic implications.
Filiaciones:
Petelytska, L:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Ramistr 100, CH-8006 Zurich, Switzerland
Bogomolets Natl Med Univ, Dept Internal Med n 3, Kiev, Ukraine
Tofani, L:
Univ Florence, Dept Stat Informat & Applicat, Florence, Italy
Velauthapillai, A:
Radboud Univ, Dept Rheumatol, Med Ctr, Nijmegen, Netherlands
Dobrota, R:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Ramistr 100, CH-8006 Zurich, Switzerland
Becker, MO:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Ramistr 100, CH-8006 Zurich, Switzerland
Mihai, C:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Ramistr 100, CH-8006 Zurich, Switzerland
Muraru, S:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Ramistr 100, CH-8006 Zurich, Switzerland
Elhai, M:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Ramistr 100, CH-8006 Zurich, Switzerland
Jordan, S:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Ramistr 100, CH-8006 Zurich, Switzerland
Hachulla, E:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Ramistr 100, CH-8006 Zurich, Switzerland
Müller-Ladner, U:
Univ Lille, Natl Referral Ctr Rare Syst Autoimmune & Autoinfla, Dept Internal Med & Clin Immunol, Inserm, CHU Lille,U1286,INFINITE, Lille, France
Justus Liebig Univ Giessen, Dept Internal Med & Clin Immunol, Bad Nauheim, Germany
Siegert, E:
Univ Lille, Natl Referral Ctr Rare Syst Autoimmune & Autoinfla, Dept Internal Med & Clin Immunol, Inserm, CHU Lille,U1286,INFINITE, Lille, France
Charite Univ Med Berlin, Dept Rheumatol & Clin Immunol, Berlin, Germany
Allanore, Y:
Univ Paris Cite, Cochin Hosp, Dept Rheumatol, Paris, France
Riemekasten, G:
Univ Lubeck Ratzeburger Allee, Univ Clin Schleswig Holstein, Dept Rheumatol & Clin Immunol, Lubeck, Germany
Bergmann, C:
Friedrich Alexander Univ Erlangen Nurnberg FAU, Dept Internal Med 3, Rheumatol & Immunol, Erlangen, Germany
Univ Hosp Erlangen, Erlangen, Germany
Becvar, R:
Charles Univ, Inst Rheumatol, Prague, Czech Republic
Charles Univ, Dept Rheumatol, Med Sch 1, Prague, Czech Republic
Solanki, K:
Te Whatu Ora Hlth New Zealand Waikato, Dept Rheumatol, Hamilton, New Zealand
Anic, B:
Univ Zagreb, Univ Hosp Ctr Zagreb, Dept Internal Med, Div Clin Immunol & Rheumatol,Sch Med, Zagreb, Croatia
Szabo, I:
Iuliu Hatieganu Univ Med & Pharm, Dept Rheumatol, Cluj Napoca, Romania
Cty Emergency Hosp, Dept Rheumatol, Cluj Napoca, Romania
Stamenkovic, B:
Inst Prevent Treatment & Rehabil Rheumat & Cardiovasc Dis, Niska Banja, Serbia
Distler, J:
Heinrich Heine Univ, Univ Hosp Dusseldorf, Clin Rheumatol, Dusseldorf, Germany
Heine Univ, Univ Hosp Dusseldorf Heinrich, Hiller Res Ctr, Dusseldorf, Germany
Carreira, PE:
Hosp 12 Octubre, Serv Reumatol, Madrid, Spain
Cuomo, G:
Luigi Vanvitelli Univ Campania, Dept Precis Med, Naples, Italy
Castellví, I:
Hosp Univ Santa Creu & St Pau, Dept Rheumatol, Barcelona, Spain
Balbir-Gurman, A:
Technion, B Shine Rheumatol Inst, Rappaport Fac Med, Rambam Hlth Care Campus, Haifa, Israel
Airo, P:
ASST Spedali Civili, Scleroderma UNIT, UOC Reumatol Immunol Clin, Brescia, Italy
Litinsky, I:
Ctr Tel Aviv Sourasky, Rheumatol Inst, Tel Aviv, Israel
Saketkoo, LA:
Tulane Univ, New Orleans, LA, USA
Louisiana State Univ, Univ Med Ctr, Scleroderma & Sarcoidosis Patient Care & Res Ctr, Sch Med, Los Angeles, CA, USA
Bertaccini, B:
Univ Florence, Dept Stat Informat & Applicat, Florence, Italy
Vonk, M:
Radboud Univ, Dept Rheumatol, Med Ctr, Nijmegen, Netherlands
de Vries-Bouwstra, J:
Leiden Univ, Med Ctr, Dept Rheumatol, Leiden, Netherlands
Hoffmann-Vold, AM:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Ramistr 100, CH-8006 Zurich, Switzerland
Oslo Univ Hosp, Dept Rheumatol, Rikshosp, Oslo, Norway
Matucci-Cerinic, M:
IRCCS Osped San Raffaele, Unit Immunol Rheumatol Allergy & Rare Dis UnIRAR, Milan, Italy
IRCCS Osped San Raffaele, Inflammat Fibrosis & Aging Initiat INFLAGE, Milan, Italy
Vita Salute San Raffaele Univ, Milan, Italy
Distler, O:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Ramistr 100, CH-8006 Zurich, Switzerland
Bruni, C:
Univ Zurich, Univ Hosp Zurich, Dept Rheumatol, Ramistr 100, CH-8006 Zurich, Switzerland
Univ Florence, Dept Expt & Clin Med, Florence, Italy
Green Submitted, hybrid
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