
Background: Interstitial lung disease (ILD) affects 10-15% of patients with Sjögren’s disease (SjD), leading to increased morbidity and mortality. Early detection is crucial for optimizing disease management and improving patient outcomes. Current guidelines recommend screening SjD patients at-risk for ILD using high-resolution computed tomography (HRCT). To date, risk factor-based estimation tools for ILD in SjD patients are there are no criteria for referral to HRCT in clinical practice.
Objectives: To develop a decision support tool for estimating the risk of ILD in SjD patients, guiding HRCT referrals in clinical practice.
Methods: We included all SjD patients fulfilling the 2016 American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) classification criteria from three European expert rheumatology centers (Zurich, Oslo and Vienna) with available HRCT images of the chest. All HRCT images were reviewed by expert radiologists at each site for the presence of ILD. Available data included demographic, serological and laboratory data, clinical SjD manifestations, and other comorbidities. To create the decision support tool, we developed a nomogram based on a logistic regression model (with Odds ratio (OR) and 95% confident interval (CI)) to estimate patient-specific probabilities, translating multivariable relationships into a graphical tool for clinical application. Predictor variables were selected based on clinical relevance, statistical significance and the area under the ROC curve (AUC) of the final multivariable logistic model.
Results: We included 547 SjD patients with available HRCT, of which 163 (29.8%) had ILD. Demographic and clinical characteristics are presented in Table 1. In multivariable regression, age (OR 1.01, 95%CI 1.01-1.07, p<0.001), erythrocyte sedimentation rate (ESR) (OR 1.03, 95%CI 1.01-1.04, p=0.003), and complement C3 (OR 0.13, 95%CI 0.05-0.35, p<0.001) were significantly associated with the presence of ILD, adjusted for male sex, polyneuropathy, lymphoma, hypergammaglobulinemia and SSB antibodies. The model was had good discriminatory ability (AUC 0.8). Based on this multivariable model, we build a nomogram which is a practical decision support tool estimating the probability of ILD in SjD patients, informing HRCT referrals based on individual risk. An example patient who is female, 50 years old, positive for anti-La/SSB, has hypergammaglobulinemia, an ESR of 40 mm/hour, and C3 of 0.8g/L has a total score of 15.5, which corresponds to a 50% predicted risk of having ILD.
Conclusion: Utilizing data from a large, multicenter international cohort of SjD patients with HRCT scans, we have developed a decision support tool for assessing ILD risk in SjD patients. Based on the availability of HRCT resources in different countries, a score of 15.5 corresponds to a 50% probability of ILD, warranting HRCT referral for the early detection of ILD in these patients.
Table 1. Baseline characteristics of patients with SjD and HRCT scans segregated by the presence of ILD.
Nomogram estimating the ILD probability in SjD patients, informing HRCT referrals based on individual risk.
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Acknowledgements: NIL.
Disclosure of Interests: Anna-Maria Hoffmann-Vold Boehringer Ingelheim, Janssen, Medscape, Merck Sharp & Dohme, Novartis and Roche, Abbvie, ARXX, Boehringer Ingelheim, Bristol Myers Squibb, Genentech, Janssen, Medscape, Merck Sharp & Dohme, Pliant therapeutics, Roche and Werfen, Boehringer Ingelheim, Janssen, Kastriot Kastrati: None declared, Marco Sprecher Abbvie, Emily Langballe Boehringer Ingelheim, Phuong Phuong Diep Boehringer-Ingelheim, Boehringer-Ingelheim, NordicInfu Care AB, Håvard Fretheim Boehringer Ingelheim, payments made to my institution, Helena Andersson: None declared, Paul Studenic AbbVie, Bojana Müller-Durovic: None declared, Cathrine Brunborg: None declared, Dr. Cosimo Bruni Boehringer Ingelheim, Novartis Foundation for medical-biological research, EMDO Foundation, Iten-Kohaut Foundation. Educational grants from Wellcome Trust. Congress support from Boehringer-Ingelheim, Hartmann-Muller Foundation, Christian Clarenbach Boehringer Ingelheim and Roche, Boehringer Ingelheim and Roche, Thomas Frauenfelder Bayer, Trond Mogens Aaløkken: None declared, Natasha Moe Boehringer Ingelheim, Helmut Prosch AstraZeneca, BMS, Boehringer Ingelheim, Bracco, Daiichi Sankyo, Janssen, MSD, Novartis, Roche, Sanofi, Siemens Healthineers, Takeda, BMS, Boehringer Ingelheim, Janssen, MSD, Roche, Sanofiim, Travel grants: Boehringer Ingelheim Research support: Boehringer Ingelheim, AstraZeneca, Siemens Healthineers and the Christian Doppler Research Association, EU Commission (EU4Health, Horizon Europe Health), Øyvind Molberg: None declared, Oliver Distler 4P-Pharma, Abbvie, Acceleron, Acepodia Biotech, Aera, Alcimed, Altavant, Amgen, AnaMar, Anaveon AG, Argenx, AstraZeneca, Blade, Bayer, Boehringer Ingelheim, Calluna (Arxx), Cantargia AB, Catalyze Capital, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, Horizon, Janssen, Kymera, Lupin, Medscape, MSD Merck, Miltenyi Biotec, Mitsubishi Tanabe, Nkarta Inc, Novartis, Orion, Pilan, Prometheus, Quell, Redxpharma, Roivant, EMD Serono, Topadur and UCB, 4P-Pharma, Abbvie, Acceleron, Acepodia Biotech, Aera, Alcimed, Altavant, Amgen, AnaMar, Anaveon AG, Argenx, AstraZeneca, Blade, Bayer, Boehringer Ingelheim, Calluna (Arxx), Cantargia AB, Catalyze Capital, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, Horizon, Janssen, Kymera, Lupin, Medscape, MSD Merck, Miltenyi Biotec, Mitsubishi Tanabe, Nkarta Inc, Novartis, Orion, Pilan, Prometheus, Quell, Redxpharma, Roivant, EMD Serono, Topadur and UCB, Patent issued “mir-29 for the treatment of systemic sclerosis” (US8247389, EP2331143). Co-founder of CITUS AG. Research Grants: BI, Kymera, Mitsubishi Tanabe, UCB.
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