Safety and Efficacy Study of Pirfenidone to Treat Idiopathic Pulmonary Fibrosis(IPF)
Status:
Unknown status
Trial end date:
2013-03-01
Target enrollment:
Participant gender:
Summary
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive form of lung disease
characterized by fibrosis of the supporting framework (interstitium) of the lungs. By
definition, the term is used only when the cause of the pulmonary fibrosis is unknown
("idiopathic"). Microscopically, lung tissue from patients shows a characteristic set of
histologic/pathologic features known as usual interstitial pneumonia (UIP). UIP is therefore
the pathologic counterpart of IPF.Idiopathic pulmonary fibrosis is characterized by
radiographically evident interstitial infiltrates predominantly affecting the lung bases and
by progressive dyspnea and worsening of pulmonary function. No therapy has been clearly shown
to prolong survival. The current strict definition of idiopathic pulmonary fibrosis provides
a new focus for basic and clinical research that will improve insight into the pathogenesis
of this disorder and stimulate the development of novel therapies.
Pirfenidone has proven antifibrotic and anti-inflammatory properties in various in vitro
systems and animal models of pulmonary fibrosis, although its precise mechanism of action
remains unclear. It attenuates fibroblast proliferation, production of fibrosis-associated
proteins and cytokines, and the increased biosynthesis and accumulation of extracellular
matrix in response to cytokines such as transforming growth factor-β. It is also shown to
slow tumor cell proliferation by inhibiting fibroblast growth factor, epidermal growth factor
and platelet-derived growth factor.
Pirfenidone has not been widely approved for clinical use in China, in this study, safety and
efficacy were evaluated to see if pirfenidone has a significant advantage over placebo in
terms of improving lung function and life quality etc. (see primary and secondary criteria)
or slows down the deterioration of lung function in Chinese subjects diagnosed with IPF.