What is the role of antifibrotic therapies in the treatment of restrictive lung disease?

Updated: Sep 16, 2020
  • Author: Jonathan Robert Caronia, DO; Chief Editor: John J Oppenheimer, MD  more...
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Answer

These therapies, including colchicine, are suggested for a variety of fibrotic disorders, including IPF.

IPF subjects given high-dose prednisone had an increased incidence of serious adverse effects and shortened survival compared with those given colchicine in a prospective randomized study [58] ; therefore, a trial of therapy with colchicine is reasonable in less symptomatic patients or those who are experiencing adverse effects with steroid therapy.

One study showed that in patients with idiopathic pulmonary fibrosis, interferon gamma-1b did not affect progression-free survival, pulmonary function, or quality of life. No survival benefit was demonstrated in this trial. [59]

Nintedanib, a triple tyrosine kinase inhibitor of fibroblast growth factor (FGF), vascular endothelial growth factor (VEGF), and platelet-derived growth factor (PDGF), has been demonstrated to lead to a reduction in the decline of FVC, led to improved quality of life, and yielded a reduction in acute exacerbations of IPF. [60] In 2014, the INPULSIS studies, two randomized, double-blind, phase 3 trials, were able to demonstrate that nintedanib led to a reduced rate of progression of disease in patients with IPF. [61]

Pirfenidone an oral medication that reduces fibroblast proliferation and collagen deposition, via down-regulation of transforming growth factor (TGF)–β and tumor necrosis factor (TNF)–α, was investigated in 2010 in two phase 3 trials. [62, 63] Results suggested that pirfenidone may reduce the FVC decline associated with IPF. Some conflicting data necessitated an additional phase 3 study. In 2014, the ASCEND trial, a multicenter, randomized control trial, demonstrated a reduction in the composite outcome of FVC decline and all-cause mortality. [61] Additional secondary outcomes demonstrated no significant decrease in all-cause mortality decline in the treatment arm. However, there was a significant improvement in progression-free survival.


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