Idiopathic Pulmonary Fibrosis

A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.

Also Known As:

Networked: 2004 relevant articles (63 outcomes, 224 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Pulmonary Fibrosis (Hamman Rich Syndrome)
2.	Interstitial Lung Diseases (Interstitial Lung Disease)
3.	Lung Diseases (Lung Disease)
4.	Sarcoidosis (Schaumann Disease)
5.	Vascular Diseases (Vascular Disease)

Experts

1.	Kaminski, Naftali: 29 articles (12/2015 - 07/2004)
2.	Martinez, Fernando J: 27 articles (12/2015 - 01/2003)
3.	Brown, Kevin K: 27 articles (11/2015 - 01/2004)
4.	Flaherty, Kevin R: 26 articles (11/2015 - 01/2003)
5.	Azuma, Arata: 24 articles (11/2015 - 02/2005)
6.	Taniguchi, Hiroyuki: 21 articles (11/2015 - 06/2005)
7.	King, Talmadge E: 21 articles (08/2015 - 09/2002)
8.	Colby, Thomas V: 21 articles (06/2015 - 03/2002)
9.	Costabel, Ulrich: 18 articles (04/2015 - 06/2003)
10.	Sugiyama, Yukihiko: 17 articles (11/2015 - 10/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Idiopathic Pulmonary Fibrosis:

1.	pirfenidoneIBA 01/01/2014 - "Pirfenidone is a new antifibrotic drug, which has been proven beneficial in idiopathic pulmonary fibrosis and is able to inhibit or reverse BIP in animal models. " 01/01/2011 - "A phase III trial in Japan showed that pirfenidone is effective for idiopathic pulmonary fibrosis (IPF). " 01/01/2015 - "Pirfenidone targets the myofibroblast and is effective in the treatment of idiopathic pulmonary fibrosis. " 06/01/2011 - "As a novel treatment option for idiopathic pulmonary fibrosis (IPF), pirfenidone has been granted marketing authorization in the European Union for the treatment of mild to moderate IPF. " 11/01/2015 - "Efficacy of pirfenidone and disease severity of idiopathic pulmonary fibrosis: Extended analysis of phase III trial in Japan."
2.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 10/01/1992 - "Intermittent, intravenous cyclophosphamide therapy was associated with improved pulmonary function and reduced corticosteroid dosage in patients with idiopathic pulmonary fibrosis who survived at least six months after institution of therapy." 10/01/1992 - "To determine the safety and efficacy of intravenous cyclophosphamide for patients with idiopathic pulmonary fibrosis. " 06/01/2004 - "Combined corticosteroid and cyclophosphamide therapy does not alter survival in idiopathic pulmonary fibrosis." 06/01/2000 - "Cyclophosphamide therapy is of limited efficacy in patients with idiopathic pulmonary fibrosis who fail to respond or who experience adverse effects from corticosteroid treatment, and adverse effects often complicate its use." 06/01/2000 - "To prospectively examine the role of cyclophosphamide in patients with idiopathic pulmonary fibrosis that is unresponsive to or intolerant of high-dose steroid treatment. "
3.	Acetylcysteine (Siran)FDA LinkGeneric 05/29/2014 - "Acetylcysteine has been suggested as a beneficial treatment for idiopathic pulmonary fibrosis, although data from placebo-controlled studies are lacking. " 04/01/2012 - "Efficacy of inhaled N-acetylcysteine monotherapy in patients with early stage idiopathic pulmonary fibrosis." 01/01/2010 - "Long-term efficacy of inhaled N-acetylcysteine in patients with idiopathic pulmonary fibrosis." 05/29/2014 - "Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis." 08/15/2005 - "Recent evidence, including the observations that the patients with idiopathic pulmonary fibrosis have higher levels of oxidant stress than control patients, and a recent multicenter European study examining the effect of the antioxidant N-acetylcysteine on the progression of idiopathic pulmonary fibrosis suggest that the cellular redox state may play a significant role in the progression of this disease. "
4.	Prednisone (Sone)FDA LinkGeneric 01/01/1999 - "A 65 year old male was diagnosed with "cryptogenic fibrosing alveolitis (CFA)" and treated successfully with Prednisone. " 01/01/2015 - "With lack of therapeutic response to a course of prednisone, both patients underwent a surgical lung biopsy that revealed a pattern consistent with Usual Interstitial Pneumonia (UIP) with honeycombing and fibroblastic foci. " 10/01/1989 - "Association of activated cytolytic lung lymphocytes with response to prednisone therapy in patients with idiopathic pulmonary fibrosis." 05/24/2012 - "In this randomized, double-blind, placebo-controlled trial, we assigned patients with idiopathic pulmonary fibrosis who had mild-to-moderate lung-function impairment to one of three groups -- receiving a combination of prednisone, azathioprine, and NAC (combination therapy), NAC alone, or placebo -- in a 1:1:1 ratio. " 07/01/1998 - "Twenty-six symptomatic subjects with clinical evidence plus either high-resolution computed tomography (HRCT, n = 25) or open-lung biopsy (OLB, n = 1) patterns typical for idiopathic usual interstitial pneumonia (idiopathic UIP) were entered into a randomized prospective treatment trial using high-dose prednisone (n = 12) versus colchicine (n = 14). "
5.	CollagenIBA 02/01/1983 - "Collagen immunofluorescence studies were performed on biopsy specimens from 25 patients with cryptogenic fibrosing alveolitis. " 02/01/1983 - "Cryptogenic fibrosing alveolitis: prediction of fibrogenic activity from immunohistochemical studies of collagen types in lung biopsy specimens." 12/01/1976 - "Although biopsies in idiopathic pulmonary fibrosis seem to show increased amounts of fibrotic tissue, biochemical studies suggest that the disease is probably one of collagen rearrangement rather than collagen increase. " 09/15/2015 - "Reduced FoxO3a expression causes low autophagy in idiopathic pulmonary fibrosis fibroblasts on collagen matrices." 08/15/2015 - "Increased abundance and stiffness of the extracellular matrix, in particular collagens, is a hallmark of idiopathic pulmonary fibrosis (IPF). "
6.	Bleomycin (Blenoxane)FDA LinkGeneric 01/01/2014 - "The aim of this study was to evaluate differences in the effects of Ephedra sinica Stapf and Fructus Schisandrae Chinensis on angiogenesis in the treatment of bleomycin-induced rat idiopathic pulmonary fibrosis. " 01/01/2015 - "Accumulation of Thy1-negative (Thy1(-)) myofibroblasts was shown in the lungs of humans with idiopathic pulmonary fibrosis (IPF) and of bleomycin-treated mice. " 01/01/2013 - "Lungs from saline- or bleomycin-treated C57BL/6 J mice and lung specimens from patients with idiopathic pulmonary fibrosis were analyzed. " 01/01/2013 - "Here we show that Elovl6 expression is significantly downregulated after an intratracheal instillation of bleomycin (BLM) and in human lung with idiopathic pulmonary fibrosis. " 01/01/2013 - "Bleomycin induces molecular changes directly relevant to idiopathic pulmonary fibrosis: a model for "active" disease."
7.	Adrenal Cortex Hormones (Corticosteroids)IBA 05/01/1984 - "Idiopathic pulmonary fibrosis is a progressive disease and is usually symptomatic at the time of presentation, so it is reasonable to give all patients a therapeutic trial with corticosteroids.(ABSTRACT TRUNCATED AT 250 WORDS)" 08/01/2015 - "Systemic corticosteroids are also used for idiopathic pulmonary fibrosis, a less prevalent chronic respiratory disease. " 08/01/2015 - "Idiopathic pulmonary fibrosis (IPF) patients with acute respiratory worsening are often initially treated with high dose corticosteroids and antimicrobials; however, there are no clear data to support these therapies, and the short-term mortality of AE-IPF is ~50%. " 06/01/2012 - "In 2003, Cochrane reviews of data for treatment with corticosteroids in idiopathic pulmonary fibrosis (IPF) found no evidence supporting their use; similarly, reviews of immunomodulatory agents found very little evidence to support their use. " 07/01/2010 - "Factors associated with the use of corticosteroids in the initial management of idiopathic pulmonary fibrosis."
8.	SteroidsIBA 01/01/2010 - "Randomised studies comparing non-steroid drugs with placebo or steroids in adults with idiopathic pulmonary fibrosis. " 03/01/2012 - "Steroids in idiopathic pulmonary fibrosis acute exacerbation: defenders or killers?" 03/01/2001 - "Steroids in idiopathic pulmonary fibrosis: a prospective assessment of adverse reactions, response to therapy, and survival." 11/01/2002 - "This study was performed to find the rationale for administering steroids to patients with idiopathic interstitial pneumonia (IIP), which was unlikely to be usual interstitial pneumonia (UIP) but was not surgically biopsied. " 03/01/2012 - "Traditionally, a subset of patients diagnosed as having idiopathic pulmonary fibrosis had positive results on cellular biopsies (prominent lymphoplasmacytic inflammation), bronchoalveolar lavage lymphocytosis, a clinical response to steroids, and a better long-term prognosis. "
9.	ColchicineFDA LinkGeneric 11/01/1998 - "Experimental and clinical evidences suggest that colchicine can be effective in the treatment of patients with idiopathic pulmonary fibrosis. " 11/01/1998 - "Patients with clinically diagnosed idiopathic pulmonary fibrosis were treated with colchicine in doses of 0.5 to 1 mg/day, according to tolerance and followed for periods ranging from 7 to 40 months. " 11/01/1998 - "To assess the effect of colchicine in the treatment of idiopathic pulmonary fibrosis. " 11/01/1998 - "[Treatment of idiopathic pulmonary fibrosis with colchicine]." 01/01/1997 - "Antiinflammatory and antifibrotic properties of colchicine: implications for idiopathic pulmonary fibrosis."
10.	Cyclosporine (Ciclosporin)FDA LinkGeneric 03/01/1990 - "Efficacy of cyclosporin A for idiopathic pulmonary fibrosis." 11/01/2015 - "It has been reported that cyclosporine A (CYA) treatment may benefit idiopathic pulmonary fibrosis (IPF) patients. " 11/01/2015 - "Cyclosporine A combined with low-dose corticosteroid treatment in patients with idiopathic pulmonary fibrosis." 01/01/2010 - "Cyclosporin A in the treatment of acute exacerbation of idiopathic pulmonary fibrosis." 07/01/2003 - "Cyclosporin A followed by the treatment of acute exacerbation of idiopathic pulmonary fibrosis with corticosteroid."

Therapies and Procedures

1.	Lung Transplantation 10/01/2006 - "Lung transplantation is effective in improving the survival of selected patients with idiopathic pulmonary fibrosis." 08/01/2003 - "Lung transplantation is effective in improving the survival of selected patients affected by idiopathic pulmonary fibrosis." 07/01/2015 - "Functional improvement in patients with idiopathic pulmonary fibrosis undergoing single lung transplantation." 04/01/2006 - "Lung transplantation has been the only therapy shown to improve survival of those with idiopathic pulmonary fibrosis." 04/01/2014 - "Expected results are: increasing the physicians' knowledge on ILDs, informing correctly the patients, bringing up to light new cases previously not diagnosed, building up a database for research (prevalence studies, risk factor studies, selection of patients for clinical trials), creating a base for a future national health programme dedicated to idiopathic pulmonary fibrosis patients, preparing future projects for development of a Romanian centre for lung transplantation."
2.	Drug Therapy (Chemotherapy) 08/16/2001 - "Although no drug therapy has clearly been demonstrated to benefit patients with idiopathic pulmonary fibrosis, a number of novel investigational agents hold promise for future study. " 10/01/2015 - "The National Heart, Lung, and Blood Institute-sponsored IPF Clinical Research Network (IPFnet) studies enrolled subjects with idiopathic pulmonary fibrosis (IPF) to evaluate drug therapies in treatment trials. " 01/01/2013 - "Idiopathic pulmonary fibrosis: recent trials and current drug therapy." 03/15/2010 - "Challenges in translating preclinical studies to effective drug therapies in idiopathic pulmonary fibrosis." 03/01/2015 - "Acute exacerbation of idiopathic pulmonary fibrosis-a review of current and novel pharmacotherapies."
3.	Transplantation (Transplant Recipients) 10/01/2009 - "Studies uniformly suggest that transplantation extends survival for patients with idiopathic pulmonary fibrosis. " 08/14/2015 - "Idiopathic pulmonary fibrosis (46.9%) represented the most common pulmonary diagnosis leading to transplantation. " 01/01/2015 - "Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible disease of the lung that has no lasting option for therapy other than transplantation. " 09/01/2014 - "In a recent registry report worldwide, 23% of all lung transplant recipients had a diagnosis of idiopathic pulmonary fibrosis. " 09/15/2012 - "The minor allele of rs2305619 was significantly associated with higher plasma PTX3 levels measured pretransplantation (P = 0.014) and at 24 hours (P = 0.047) after transplantation in patients with idiopathic pulmonary fibrosis. "
4.	Transplants (Transplant) 11/01/2015 - "What Is the Optimal Transplant for Older Patients With Idiopathic Pulmonary Fibrosis?" 09/01/2015 - "Idiopathic pulmonary fibrosis is associated with increased impedance measures of reflux compared to non-fibrotic disease among pre-lung transplant patients." 10/01/2014 - "According to our results, unilateral lung transplant for idiopathic pulmonary fibrosis is an alternative to bilateral lung transplant and may affect the allocation process." 10/01/2014 - "Lung transplant is a safe and curative treatment for idiopathic pulmonary fibrosis. " 10/01/2014 - "It is unknown if uni- or bilateral lung transplant is best for treatment of usual idiopathic pulmonary fibrosis. "
5.	Hemoperfusion 01/01/2013 - "Association between cytokine removal by polymyxin B hemoperfusion and improved pulmonary oxygenation in patients with acute exacerbation of idiopathic pulmonary fibrosis." 01/01/2006 - "This study was aimed to investigate the effect of polymyxin B-immobilized fiber column (PMX) hemoperfusion treatment on the acute exacerbation of idiopathic pulmonary fibrosis (IPF). " 01/01/2015 - "Treatment of acute exacerbation of idiopathic pulmonary fibrosis with direct hemoperfusion using a polymyxin B-immobilized fiber column improves survival." 11/01/2007 - "[Direct hemoperfusion with a polymyxin B-immobilized fiber column eliminates neutrophils and improves pulmonary oxygenation--a comparison of two cases with acute exacerbation of idiopathic pulmonary fibrosis]." 01/01/2006 - "Beneficial effect of polymyxin B-immobilized fiber column (PMX) hemoperfusion treatment on acute exacerbation of idiopathic pulmonary fibrosis."