POS1307 NINTEDANIB IN RHEUMATIC DISEASE-ASSOCIATED INTERSTITIAL LUNG DISEASE – A MULTICENTRE NATIONWIDE COHORT STUDY

BackgroundInterstitial lung disease (ILD) is a severe manifestation of inflammatory rheumatic diseases (IRD). Conventional treatments have shown modest and short-lived success in the control of ILD [1]. Nintedanib (Nib) was recently approved for systemic sclerosis (SSc)-associated ILD and fibrosing ILD with a progressive phenotype [1]. However, its experience in other IRD-associated ILD is still scarce.ObjectivesTo describe the clinical features and outcomes of patients with IRD-ILD treated with Nib across ten national rheumatology departments.MethodsRetrospective cohort study, with chart review of the medical records for inclusion of patients with IRD-ILD treated with Nib. Data are presented as frequencies and mean±standard deviation (range) for categorical and continuous variables, respectively.ResultsA total of 64 patients (70% women) were identified (Table 1). The mean age at IRD and ILD diagnosis was 56.2±11.7 and 59.7±10.8 years (y), respectively, and the mean time for Nib initiation after ILD diagnosis was 5.1±3.9 y. The most frequently underlying IRD were SSc (55%) and rheumatoid arthritis (28%). ILD was diagnosed prior to the IRD in only 7 patients (11%). Computed tomography (CT) identified usual interstitial pneumonia (UIP) in 36 cases (56%), fibrotic nonspecific interstitial pneumonia (NSIP) in 27 (42%) and organizing pneumonia in one (2%). Patients with UIP took longer to start Nib (5.5±4.4 y) compared to patients with NSIP (4.7±2.4 y). Before Nib, respiratory function tests (RFTs) showed a low single-breath diffusing capacity of the lungs for CO (DLCO) in all patients, with a mean predicted value of 39±18%. All patients had previously received immunosuppressive therapy, most commonly glucocorticoids (70%) and mycophenolate mofetil (50%). Patients were exposed to Nib for a mean of 18.5±15.9 months (m), 17 were treated for more than 24m. After 6m, there was a statistical improvement in DLCOc SB (p=0.041) and DLCOc/VA (p=0.029) compared to baseline, which was not observed at 12 or 24m (p>0.05). No differences were found between patients with NSIP and UIP. Nevertheless, the latter showed a trend for an overall lower benefit on RFTs (Figure 1), mainly in the first 12m of treatment (NSIP: forced vital capacity [FVC] p=0.173 and DLCOc SB p=0.199; UIP: FVC p=0.278 and DLCOc SB p=0.267). In patients with <3y of ILD duration (n=20 [8 NSIP; 12 UIP]) a significant improvement in DLCOc SB and DLCOc/VA was observed at 24m (p=0.003 and p=0.017). Regarding tolerance: 38 (59%) patients were able to maintain a daily dose of 150 mg bid, 24 (38%) required reduction to 100 mg bid due to gastrointestinal (GI) symptoms and 2 (3%) discontinued Nib.ConclusionAn initial benefit was observed in the DLCO of patients treated with Nib at 6m. Only 1/3 of patients were started on Nib with less than 3y of ILD duration, possibly reflecting the recent approval of Nib for these diseases. In this subset of patients, a significant improvement in DLCO was observed at 24m, suggesting that early initiation of treatment may halt ILD progression.Reference[1]Khanna D, et al. Arthritis Rheumatol. 2022;74(1):13-27Acknowledgements:NIL.Disclosure of InterestsNone Declared.