POS0864 ELIGIBILITY FOR ANTI-FIBROTIC TREATMENT WITH NINTEDANIB OF PATIENTS WITH SYSTEMIC SCLEROSIS RELATED INTERSTITIAL LUNG DISEASE
Authors: E. De Lorenzis, G. Natalello, L. Verardi, L. Gigante, M. A. D’agostino, S. L. Bosello
Journal: Annals of the Rheumatic Diseases (2021)

Background: Pulmonary involvement is the main determinant of disease-related mortality in systemic sclerosis (SSc). The SENSCIS and INBUILD trials have documented the efficacy of the antifibrotic drug Nintedanib (NTN) in the treatment of SSc-related interstitial lung disease (SSc-ILD) and progressive forms of ILD other than Idiopathic Pulmonary Fibrosis 1,2 . The criteria for the use of this drug in patients with SSc-ILD in clinical practice are currently being defined. Objectives: To evaluate the proportion of patients eligible for NTN treatment based on the enrollment criteria fof the SENSCIS and INBUILD studies in a real-life cohort of SSc-ILD patients. Methods: We considered consecutive patients with ILD extension on CT ≥10% and disease duration ≤7 years with at least one visit in the period between September 2016 and December 2019. Data of the clinical visits were retrospectively evaluated. For each patient, we examined the visits during which a well-tolerated immunosuppressive therapy was modified because of progression of SSc-ILD and the most recent follow-up visit. Eligibility of patients for NTN was defined according to the inclusion criteria of the SENSCIS and INBUILD trials. Patients with more than 2 acral ulcers at the time of evaluation, history of digital amputation, pulmonary hypertension (functional class III-IV) and increased hemorrhagic or thrombotic risk were judged not eligible to NTN in trials. Results: A total of 177 visits regarding 78 patients were examined (females 80.8%, diffuse skin disease 51.3%, anti-Scl70 antibodies positivity 55.7%, age 54.8±16.0 years, disease duration 4.0±2.4 years). Considering the visits in which indication for a therapeutic change was given, 54 patients (54.5%) were eligible for NTN according to SENSCIS criteria and of these 31 (31.3%) also according to INBUILD criteria (Figure 1). In this group, 25 patients were treated with mycophenolate mofetil, 11 with azathioprine, 10 with cyclophosphamide, 7 with methotrexate and 8 with rituximab (2 in combination). At the latest available evaluation, 42 patients (62.8%) were eligible for NTN according to SENSCIS criteria and of these 12 (15.4%) also according to INBUILD criteria (Figure 1). In this group, 30 patients were in mycophenolate mofetil (6 in combination with biologic treatment), 5 in azathioprine, 1 in cyclophosphamide, 1 in methotrexate, 6 in rituximab, 2 in tocilizumab and 1 in pirfenidone. Overall, the factors limiting NTN start according to the trial enrollment criteria would have been: uncompromised (19.2%) or too low (6.4%) DLco values, too low FVC (3.4%), severe acral disease with ulcers (16.9%), severe pulmonary arterial hypertension (6.2%), increased thrombotic or haemorrhagic risk (6.2%). In the scenario of eligibility, skin progression was detectable in 43.4% of all visits. Conclusion: Treatment that can modify the progression of SSc-ILD are currently limited. Based on our retrospective analysis, the use of NTN in accordance with current clinical evidence could be considered in a significant percentage of patients with SSc-ILD. References: [1]Flaherty KR, et al. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med. 2019. [2]Distler O, et al. Nintedanib for Systemic Sclerosis-Associated Interstitial Lung Disease. N Engl J Med. 2019. Disclosure of Interests: None declared