POS0123 ASSOCIATION OF LUNG IMAGING PATTERN WITH PROGNOSIS AND IMMUNOSUPPRESSION RESPONSE IN CONNECTIVE TISSUE DISEASE ASSOCIATED INTERSTITIAL LUNG DISEASE

Background Prognosis in connective tissue disease associated interstitial lung disease (CTD-ILD) is influenced by the underlying diagnosis and chest imaging pattern. Usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), and fibrotic hypersensitivity pneumonitis (fHP) patterns can be found across all CTD-ILD subtypes although their impact on disease evolution and treatment response is unclear. Objectives Our goal was to examine the association of lung imaging pattern with CTD-ILD progression, mortality, and immunosuppression response. Methods 615 patients with CTD-ILD enrolled in the Canadian Registry for Pulmonary Fibrosis had high-resolution chest computed tomography (HRCT) from their first ILD clinic visit reviewed in standardized multidisciplinary discussion. All CTD diagnoses were rheumatologist-confirmed. Experienced chest radiologists blinded to clinical data categorized each case into five groups: UIP, NSIP, organizing pneumonia (OP), fHP, and other patterns. Longitudinal percent-predicted forced vital capacity (FVC) and transplant-free survival were compared between imaging groups using linear mixed effects and Cox proportional hazards models adjusted for age, sex, smoking pack-years, and baseline FVC. Linear mixed effects models were used to compare pre- and post-treatment rate of FVC decline in patients with ≥6 months follow-up before and after treatment with mycophenolate, azathioprine, rituximab, cyclophosphamide, and/or tocilizumab. UIP was the reference group for all comparisons. Results The most frequent CTD subtypes were systemic sclerosis (SSc) (33%), rheumatoid arthritis (RA) (20%), and idiopathic inflammatory myopathy (IIM) (16%) with NSIP pattern present in 54% of all CTD-ILD (Table 1). On multivariable analyses among all CTD-ILD patients, NSIP was associated with a slower rate of FVC decline by 1.1%/year (0.2, 1.9) and a lower mortality HR (95%CI) of 0.65 (0.45, 0.93) compared to UIP. OP was also associated with a slower rate of FVC decline by 3.5%/year (2.0, 4.9) and a lower mortality HR (95%CI) of 0.18 (0.05, 0.57) compared to UIP. In contrast, fHP had a higher mortality HR (95%CI) of 1.58 (1.01, 2.40). The rate of FVC decline after treatment was not significantly different compared to pre-treatment in the UIP group but was slower in the NSIP group by 2.1%/year (1.4, 2.8). Subgroup analyses in RA-ILD and SSc-ILD showed the persistence of fHP having a higher mortality compared to UIP in RA-ILD. Conclusion The presence of an NSIP pattern was associated with improved outcomes and immunosuppression response compared to UIP in the overall CTD-ILD group. The findings of fHP associated with worse survival compared to UIP in CTD-ILD and in the RA-ILD are novel. These findings need to be further confirmed in disease specific cohorts and randomized trials of immunosuppression in patients with CTD-ILD. REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests Boyang Zheng: None declared, Daniel-Costin Marinescu: None declared, cameron hague: None declared, Nestor Muller: None declared, darra murphy: None declared, Andrew Churg: None declared, Joanne Wright: None declared, Amna Al-Arnawoot: None declared, Ana-Maria Bilawich: None declared, patrick bourgouin: None declared, Gerald Cox: None declared, celine durand: None declared, Tracy Elliot: None declared, Jen Ellis: None declared, Jolene Fisher Consultant of: Boehringer-Ingelheim, AstraZeneca, Derek Fladeland: None declared, Amanda Grant-Orser: None declared, Gillian Goobie Grant/research support from: Boehringer Ingelheim, Zachary Guenther: None declared, Ehsan Haider: None declared, Nathan Hambly Speakers bureau: Boehringer Ingelheim, Grant/research support from: Boehringer Ingelheim, Janssen, Roche, James Huynh: None declared, Kerri Johannson Consultant of: Boehringer-Ingelheim, Hoffman-La Roche Ltd, geoff karjala: None declared, Nasreen Khalil: None declared, Martin Kolb Speakers bureau: Roche, Novartis, Boehringer Ingelheim, Grant/research support from: Boehringer Ingelheim, Pieris, Roche, Jonathon Leipsic Speakers bureau: GE Healthcare, Philips Healthcare, Stacey Lok Speakers bureau: Boehringer Ingelheim, sarah macisaac: None declared, micheal mcinnis: None declared, Helene Manganas Grant/research support from: Boehringer Ingelheim Canada, Hoffmann La Roche, Galapagos, BMS, Veronica Marcoux Grant/research support from: Astra Zeneca,Roche,Boehringer Ingelheim, John Mayo: None declared, julie morisset Speakers bureau: Roche, Boehringer Ingelheim, Ciaran Scallan: None declared, Tony Sedlic: None declared, shane shapera Consultant of: AstraZeneca, Boehringer Ingelheim, Hoffman LaRoche, Kelly Sun: None declared, victoria tan: None declared, Alyson Wong: None declared, Christopher Ryerson Speakers bureau: Boehringer Ingelheim, Hoffmann-La Roche, Astra Zeneca, Consultant of: Boehringer Ingelheim, Hoffmann-La Roche, Astra Zeneca.

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