Idiopathic pulmonary fibrosis: Recent advances on pharmacological therapy

Abstract

Idiopathic pulmonary fibrosis (IPF) is the most common and lethal of the idiopathic interstitial pneumonias with an estimated 5-year survival of approximately 20%. In the last two decades our understanding of disease pathogenesis has substantially evolved and novel compounds have been developed consequent to the increasing knowledge of the mechanisms underlying disease pathobiology. The disease appears to be driven - following chronic injury - by abnormal/dysfunctional alveolar epithelial cells that promote fibroblast recruitment and proliferation, resulting in scarring of the lung and irreversible loss of function. With very few exceptions, clinical trials evaluating novel potential therapies have provided disappointing results. More recently, pirfenidone and nintedanib, two compounds with pleiotropic mechanisms of action, have proven effective in slowing functional decline and disease progression in IPF patients with mild to moderate functional impairment, highlighting the importance of timely diagnosis and administration of treatment in early stages of disease. However, due to the complexity and uncertainties intrinsic to IPF, it is essential that each therapeutic strategy be tailored to the individual patient, after evaluation of potential benefits and risks. This article provides an overview of the most recent clinical trials in IPF and discusses how their results are going to change the clinical and clinical research landscape in IPF. A number of agents with high potential are currently being tested and many more are ready for clinical trials. Their completion is critical for achieving the ultimate goal of curing patients with IPF

Deficiency of α7 Nicotinic Acetylcholine Receptor Attenuates Bleomycin-Induced Lung Fibrosis in Mice

“…We took advantage of fibroblast culture and BLM-induced lung fibrosis mouse models to investigate (1) whether deletion of Chrna 7 would reduce expression of fibrogenic genes in the early stage of the BLM-induced lung fibrosis mouse model, (2) whether deletion of Chrna 7 would attenuate collagen deposition (Masson′s trichrome staining) in BLMinduced lung fibrosis, and (3) whether activation of α7 nAChR would regulate TGF-β signaling and transcription of fibrogenic genes. The results of this study will provide novel therapeutic targets for combating lung fibrosis.…”

Saffron Protection against Bleomycin-Induced Pulmonary Fibrosis in Rats

“…Recent clinical trials showed disappointing results in treatment of IPF due to its complications highlighting the importance of early diagnosis and treatment of patients [10][11][12]. At present, it is not clear how saffron affects lung fibrosis according to its antioxidant and radical scavenging activity properties [13].…”

Salvianolic Acid B Attenuates Experimental Pulmonary Fibrosis through Inhibition of the TGF-β Signaling Pathway

“…Pulmonary fibrosis represents a group of devastating and largely irreversible disorders characterized by chronic inflammation and excessive deposition of collagen12. Pulmonary fibrosis is a very common end-stage manifestation of several diseases, including idiopathic pulmonary fibrosis (IPF), pulmonary hypertension, and scleroderma345.…”