Novel therapeutic approaches for pulmonary fibrosis

Abstract

Pulmonary fibrosis represents the end stage of a number of heterogeneous conditions and is, to a greater or lesser degree, the hallmark of the interstitial lung diseases. It is characterized by the excessive deposition of extracellular matrix proteins within the pulmonary interstitium leading to the obliteration of functional alveolar units and in many cases, respiratory failure. While a small number of interstitial lung diseases have known aetiologies, most are idiopathic in nature, and of these, idiopathic pulmonary fibrosis is the most common and carries with it an appalling prognosis – median survival from the time of diagnosis is less than 3 years. This reflects the lack of any effective therapy to modify the course of the disease, which in turn is indicative of our incomplete understanding of the pathogenesis of this condition. Current prevailing hypotheses focus on dysregulated epithelial–mesenchymal interactions promoting a cycle of continued epithelial cell injury and fibroblast activation leading to progressive fibrosis. However, it is likely that multiple abnormalities in a myriad of biological pathways affecting inflammation and wound repair – including matrix regulation, epithelial reconstitution, the coagulation cascade, neovascularization and antioxidant pathways – modulate this defective crosstalk and promote fibrogenesis. This review aims to offer a pathogenetic rationale behind current therapies, briefly outlining previous and ongoing clinical trials, but will focus on recent and exciting advancements in our understanding of the pathogenesis of idiopathic pulmonary fibrosis, which may ultimately lead to the development of novel and effective therapeutic interventions for this devastating condition. LINKED ARTICLES This article is part of a themed issue on Respiratory Pharmacology. To view the other articles in this issue visit http://dx.doi.org/10.1111/bph.2011.163.issue‐1

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     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

238. 238.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

239. 239.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

240. 240.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

241. 241.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

242. 242.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

243. 243.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

244. 244.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

245. 245.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

246. 246.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

247. 247.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

248. 248.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

249. 249.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

250. 250.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

251. 251.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

252. 252.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

253. 253.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

254. 254.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

255. 255.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

256. 256.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

257. 257.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

258. 258.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

259. 259.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

260. 260.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

261. 261.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

262. 262.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

263. 263.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

264. 264.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

265. 265.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

266. 266.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

267. 267.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

268. 268.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

269. 269.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

270. 270.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

271. 271.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

272. 272.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

273. 273.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

274. 274.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

275. 275.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

276. 276.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

277. 277.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

278. 278.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

279. 279.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

280. 280.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

281. 281.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

282. 282.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

283. 283.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

284. 284.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

285. 285.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

286. 286.

     This reference’s wording can’t be shown under the paper’s licence.

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287. 287.

     This reference’s wording can’t be shown under the paper’s licence.

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288. 288.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

289. 289.

     This reference’s wording can’t be shown under the paper’s licence.

     Not in our library

Cited by papers we hold 7

• Stem Cells, Cell Therapies, and Bioengineering in Lung Biology and Disease 2019 · 2020
  Not checked
• Transforming growth factor beta 1 induces methylation changes in lung fibroblasts. · 2019
  Not checked
• M2 macrophages promote myofibroblast differentiation of LR-MSCs and are associated with pulmonary fibrogenesis · 2018
  No notice
• Differential effects of Nintedanib and Pirfenidone on lung alveolar epithelial cell function in ex vivo murine and human lung tissue cultures of pulmonary fibrosis · 2018
  No notice
• Nrf2 antioxidant pathway suppresses Numb-mediated epithelial–mesenchymal transition during pulmonary fibrosis · 2018
  No notice
• Exploration of a potent PI3 kinase/mTOR inhibitor as a novel anti-fibrotic agent in IPF · 2016
  No notice
• Diverse functions of matrix metalloproteinases during fibrosis · 2014
  No notice

Reads

A person counts once a day per paper. We keep no cookie and no address: a reader is a hash that changes every day. Any month under five reads shows no number.