Upfront combination therapy with nintedanib and anti-inflammatory agents for progressive pulmonary fibrosis: a multicentre, single-arm phase 2 study (TOP-ILD).

ABSTRACT:

Objective

Progressive pulmonary fibrosis (PPF) is a chronic interstitial lung disease (ILD) characterised by fibrotic progression and poor prognosis, with effective treatment strategies for previously untreated patients remaining unclear. This study evaluated the efficacy and safety of upfront combination therapy with anti-inflammatory and antifibrotic agents in previously untreated PPF patients.

Methods

This multicentre, single-arm phase 2 study enrolled 34 patients with ILD (including unclassifiable idiopathic interstitial pneumonia, idiopathic nonspecific interstitial pneumonia, fibrotic hypersensitivity pneumonitis and rheumatoid arthritis-associated ILD) all with evidence of PPF. Tacrolimus (0.0375 mg·kg^-1 twice daily) and prednisolone (10 mg once daily) were

SUBMITTER: Tsubouchi K 

PROVIDER: S-EPMC12907810 | biostudies-literature | 2026 Jan

REPOSITORIES: biostudies-literature