P56 What happens to patients with idiopathic pulmonary fibrosis who are not eligible for antifibrotic treatment due to current NICE guidelines. (12th November 2019)

• Record Type: Journal Article
• Authors: Noor, S; Nawaz, S; Garfoot, T; Greaves, M; Hayton, C; Margaritopoulos, G; Marshall, T; Montoro, A; Morris, H; Newman, K; Rivera-Ortega, P; Stanel, S; Zakis, K; Leonard, C; Chaudhuri, N
• Is Part Of: Thorax. Volume 74(2019)Supplement 2
• Page Start: A119
• Page End: A120
• Publication Date: 2019-11-12
• Subjects: Chest -- Diseases -- Periodicals; Thorax; Chest -- Diseases; Periodicals; Periodicals; 617.54
• DOI: 10.1136/thorax-2019-BTSabstracts2019.199
• Languages: English
• ISSNs: 0040-6376

Abstract

Abstract : Antifibrotic prescribing for Idiopathic Pulmonary Fibrosis (IPF) is limited by the National Institute of Health and Clinical Effectiveness (NICE) to patients with a forced vital capacity (FVC) of 50–80%. 38% of IPF patients on the British Thoracic Society registry have an FVC above 80%. Methods: This is a retrospective single centre cohort study of IPF patients with baseline FVC above 80%, between January 2007 and September 2018. We assessed electronic records to collect data on patient demographics, treatment and lung function changes over time. Data and statistics are described as in Table1. A linear mixed model was performed to assess the change in FVC and DLCO over time. Results: 161 patients with baseline FVC above 80% were included, 74.5% (n=120) were male. The mean age was 72.7 ±7.4. 128 patients initially were treated with antifibrotics through compassionate use programmes (CUP) (42 (26.1%) on pirfenidone and 104 (64.6%) on nintedanib) compared to 33 patients who had no treatment, as the CUP had closed. Patient demographics, duration of treatment, adverse events and reasons for discontinuation are presented in Table 1. Patients without antifibrotic therapy had a statistically higher baseline FVC compared to other groups (3.55 (100%) vs. 2.88 (89%) pirfenidone vs 2.96 (92%) nintedanib) (p<0.001). FVC decline over 12 months was similar regardless of therapy (3.72% untreated vs. 3.24%pirfenidone vs 2.64% nintedanib). Untreated patients died within 2.7 ±1.4SD years post diagnosis (median survival of 2.5 years) compared to 3.6 ±1.8 years of diagnosis (median survival of 3.5 years) on pirfenidone and 3.1 ±1.3 years of diagnosis (median survival of 3 years) on nintedanib. Conclusion: Untreated patients had higher FVC and DLCO compared to treated cohorts, which makes comparison of lung function decline difficult. Despite this, one in five untreated patients with an average FVC of 100% still die within a median of 2.5 years while antifibrotic therapy was associated with a median survival to 3–3.5 years in a cohort with lower baseline lung function. Lung function decline in treated cohorts is similar to that seen in clinical trials (-2.64%nintedanib and -3.24%pirfenidone).