The incidence of interstitial lung disease in patients with systemic sclerosis: rate, risk factors and prognostic implications in a EUSTAR cohort analysis (CP 133).
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Petelytska Liubov, Tofani Lorenzo, Velauthapillai Arthiha, Dobrota Rucsandra, Becker Mike Oliver, Mihai Carina, Muraru Sinziana, Elhai Muriel, Jordan Suzana, Hachulla Eric, Müller-Ladner Ulf, Siegert Elise, Allanore Yannick, Riemekasten Gabriela, Bergmann Christina, Becvar Radim, Solanki Kamal, Anic Branimir, Szabo Iulia, Stamenkovic Bojana, Distler Jörg, Carreira Patricia E, Cuomo Giovanna, Castellví Ivan, Balbir-Gurman Alexandra, Airò Paolo, Litinsky Ira, Saketkoo Lesley Ann, Bertaccini Bruno, Vonk Madelon C, de Vries-Bouwstra Jeska, Hoffmann-Vold Anna-Maria, Matucci-Cerinic Marco, Distler Oliver, Bruni Cosimo, EUSTAR collaborators
Аннотация
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.