Figures
- FIGURE 1
a) shows idiopathic pulmonary fibrosis (IPF) and b) progressive fibrosing interstitial lung disease (PFILD) antifibrotic treatment algorithms. Other therapies are not shown. Black boxes show current treatments and pathways, red boxes and arrows show possible future treatments and pathways based upon the BI 1015550 and RELIEF trials [3, 7, 8, 39].
Tables
- TABLE 1
Comparison of the diagnostic criteria for progressive pulmonary fibrosis (PPF) in the ATS/ERS 2022 idiopathic pulmonary fibrosis and PPF guideline update and the INBUILD progressive fibrosing interstitial lung disease (PFILD) randomised control trial inclusion criteria
ERS/ATS PPF criteria [3] (any 2 criteria) INBUILD trial PFILD criteria [8] Forced vital capacity Absolute decline ≥5% Relative decline >10%
OR 5–10% and any of the below criteriaSymptoms Worsened respiratory symptoms Worsened respiratory symptoms DLCO Absolute decline ≥10% Not included Radiology Worsened fibrosis on HRCT Worsened fibrosis on HRCT Time-period decline/investigations must be within 12 months 24 months ATS: American Thoracic Society; ERS: European Respiratory Society; DLCO: diffusing capacity of the lung for carbon monoxide; HRCT: high-resolution computed tomography.
- TABLE 2
Summary of pirfenidone and nintedanib trials conducted in progressive fibrosing interstitial lung diseases (PFILDs)
PFILD (non-IPF) Trial Nintedanib/pirfenidone PFILD (CTD-ILD; fHP; autoimmune lung fibrosis; NSIP; unclassifiable IIP; sarcoidosis; others) INBUILD [8] Nintedanib PFILD (CTD-ILD; NSIP; fHP; asbestosis) RELIEF [7] Pirfenidone Sarcoidosis PFILD PirFS [32] Pirfenidone Unclassifiable PFILD Pirfenidone in unclassifiable PF-ILD [33] Pirfenidone Pneumoconiosis PFILD NiPPS (ClinicalTrials.gov identifier: NCT04161014, expected completion 2025) Pirfenidone Pulmonary fibrosis with anti- myeloperoxidase antibodies PFILD PIRFENIVAS [34] Pirfenidone IPF: idiopathic pulmonary fibrosis; CTD-ILD: connective tissue disease associated ILD; NSIP: nonspecific interstitial pneumonia; fHP: fibrosing hypersensitivity pneumonitis; IIP: idiopathic interstitial pneumonia; INBUILD: Efficacy and Safety of Nintedanib in Patients With Progressive Fibrosing Interstitial Lung Disease; RELIEF: Exploring Efficacy and Safety of oral Pirfenidone for progressive, non-IPF Lung Fibrosis; PirFS: Pirfenidone for Progressive Fibrotic Sarcoidosis; NiPPS: The Nintedanib in Progressive Pneumoconiosis Study; PIRFENIVAS: Pilot Study of Pirfenidone in Pulmonary Fibrosis with Anti-myeloperoxidase Antibodies. Notable fibrosing ILD trials, excluded due to lack of progression in inclusion criteria, include the TRAIL 1 (Pirfenidone in rheumatoid arthritis associated ILD) and SENSCIS trials (Nintedanib in systemic sclerosis associated ILD) [35, 36].
