Idiopathic Pulmonary Fibrosis

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1141 relevant articles (31 outcomes, 127 trials/studies) found for this Disease

Description: 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:

Usual Interstitial Pneumonia; Cryptogenic Fibrosing Alveolitides; Fibrosing Alveolitides, Cryptogenic; Fibrosing Alveolitis, Cryptogenic; Idiopathic Pulmonary Fibroses; Interstitial Pneumonia, Usual; Interstitial Pneumonias, Usual; Pulmonary Fibroses, Idiopathic; Pulmonary Fibrosis, Idiopathic; Usual Interstitial Pneumonias; Cryptogenic Fibrosing Alveolitis

Relationship Network

Disease Context: Research Results

Respiratory Tract Diseases: 377

Lung Diseases: 8942

Pulmonary Fibrosis: 2947

Idiopathic Pulmonary Fibrosis: 1141

Interstitial Lung Diseases: 2904

Idiopathic Interstitial Pneumonias: 136

Idiopathic Pulmonary Fibrosis: 1141

Related Diseases

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

Drugs and Biologics

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

1.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 10/01/1992 - "STUDY OBJECTIVE: To determine the safety and efficacy of intravenous cyclophosphamide for patients with idiopathic pulmonary fibrosis. " 10/01/1992 - "CONCLUSIONS: 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." 06/01/2000 - "STUDY OBJECTIVES: To prospectively examine the role of cyclophosphamide in patients with idiopathic pulmonary fibrosis that is unresponsive to or intolerant of high-dose steroid treatment. " 06/01/2004 - "Combined corticosteroid and cyclophosphamide therapy does not alter survival in idiopathic pulmonary fibrosis." 06/01/2000 - "CONCLUSIONS: 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." Order ALL the reference details at left...
2.	Prednisone (Sone)FDA LinkGeneric 01/01/1999 - "A 65 year old male was diagnosed with "cryptogenic fibrosing alveolitis (CFA)" and treated successfully with Prednisone. " 10/01/1989 - "Association of activated cytolytic lung lymphocytes with response to prednisone therapy in patients with idiopathic pulmonary fibrosis." 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). " 08/01/1991 - "Twenty-seven newly diagnosed patients with idiopathic pulmonary fibrosis (IPF) who were previously untreated for IPF were enrolled in a prospective, double-blind, randomized, placebo-controlled study to compare the therapeutic effect of combined prednisone/azathioprine (n = 14) with prednisone plus placebo (n = 13). " 07/01/1998 - "Colchicine versus prednisone in the treatment of idiopathic pulmonary fibrosis. " Order ALL the reference details at left...
3.	Colchicine FDA LinkGeneric 11/01/1998 - "BACKGROUND: Experimental and clinical evidences suggest that colchicine can be effective in the treatment of patients with idiopathic pulmonary fibrosis. " 11/01/1998 - "PATIENTS AND METHODS: 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 - "AIM: 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." Order ALL the reference details at left...
4.	Cyclosporine (Ciclosporin)FDA LinkGeneric 03/01/1990 - "Efficacy of cyclosporin A for idiopathic pulmonary fibrosis." 07/01/2003 - "Cyclosporin A followed by the treatment of acute exacerbation of idiopathic pulmonary fibrosis with corticosteroid." 06/01/1997 - "There are few reports on the use of cyclosporine in idiopathic pulmonary fibrosis, in experimental animals or humans. " 08/01/1991 - "Three of the patients with cryptogenic fibrosing alveolitis and all five patients with lymphocytic infiltration responded with a reduction in dyspnoea or an increase in vital capacity, or both; cyclosporin A appeared to be effective, or at least to have a corticosteroid potentiating effect. " 07/01/1989 - "Advanced cryptogenic fibrosing alveolitis: preliminary report on treatment with cyclosporin A." Order ALL the reference details at left...
5.	Technetium (Technetium 99m)IBA 07/01/1989 - "We measured the clearance of technetium 99m-labelled diethylene triamine penta-acetate (99m Tc-DTPA) with time from the lungs to the blood, in 11 normal, non-smoking subjects (mean age 30 years), ten non-smoking patients with cryptogenic fibrosing alveolitis (CFA, mean age 57 years) and ten patients who were non-smokers with obliterative bronchiolitis (mean age 51 years). " 12/01/2001 - "BACKGROUND: Clearance of inhaled technetium 99m-labelled diethylenetriamine penta-acetic acid ((99m)Tc-DTPA) from the lungs is a potential indicator of disease progression in patients with idiopathic pulmonary fibrosis (IPF). " Order ALL the reference details at left...
6.	Org 31540 (PENTA)IBA 07/01/1989 - "We measured the clearance of technetium 99m-labelled diethylene triamine penta-acetate (99m Tc-DTPA) with time from the lungs to the blood, in 11 normal, non-smoking subjects (mean age 30 years), ten non-smoking patients with cryptogenic fibrosing alveolitis (CFA, mean age 57 years) and ten patients who were non-smokers with obliterative bronchiolitis (mean age 51 years). " 12/01/2001 - "BACKGROUND: Clearance of inhaled technetium 99m-labelled diethylenetriamine penta-acetic acid ((99m)Tc-DTPA) from the lungs is a potential indicator of disease progression in patients with idiopathic pulmonary fibrosis (IPF). " Order ALL the reference details at left...
7.	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. " 03/01/2009 - "The pathological hallmarks of idiopathic pulmonary fibrosis include proliferating fibroblasts and myofibroblasts, as well as excessive collagen matrix deposition. " 01/01/2009 - "Idiopathic pulmonary fibrosis (IPF) is characterized by progressive (myo)fibroblast accumulation and collagen deposition. " Order ALL the reference details at left...
8.	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)" 04/01/2009 - "Authors present the case of a 30-year-old man treated with corticosteroids for idiopathic pulmonary fibrosis, having restrictive ventilatory disfunction and bilateral perihilar interstitial infiltrates, ground-glass opacity on CT of the lungs. " 08/01/2007 - "Idiopathic pulmonary fibrosis (IPF) is a deadly disease, largely unresponsive to treatment with corticosteroids and immunosuppressives. " 01/01/2006 - "The patient was following up as a diagnosis of usual interstitial pneumonia and treating with corticosteroids and azothiopurine. " 02/01/2004 - "[Idiopathic pulmonary fibrosis: should corticoids still be used? " Order ALL the reference details at left...
9.	Transforming Growth Factor beta (TGF-beta)IBA 07/01/2009 - "AIM:The pro-fibrogenic cytokine transforming growth factor-beta 1 (TGF-beta1) has attracted much attention for its potential role in the etiology of idiopathic pulmonary fibrosis (IPF). " 01/01/2009 - "Recent advances in molecular targets and treatment of idiopathic pulmonary fibrosis: focus on TGFbeta signaling and the myofibroblast." 08/15/2003 - "Genetic factors in idiopathic pulmonary fibrosis: transforming growth factor-beta implicated at last." 07/01/2001 - "Circulating transforming growth factor-beta(1): a potential marker of disease activity during idiopathic pulmonary fibrosis." 08/01/1991 - "Increased production and immunohistochemical localization of transforming growth factor-beta in idiopathic pulmonary fibrosis." Order ALL the reference details at left...
10.	Recombinant Interferon-gamma (Interferon gamma, Recombinant)FDA Link 07/18/2009 - "We assessed whether treatment with interferon gamma-1b improved survival compared with placebo in patients with idiopathic pulmonary fibrosis and mild-to-moderate impairment of pulmonary function. " 07/18/2009 - "INTERPRETATION: We cannot recommend treatment with interferon gamma-1b since the drug did not improve survival for patients with idiopathic pulmonary fibrosis, which refutes previous findings from subgroup analyses of survival in studies of patients with mild-to-moderate physiological impairment of pulmonary function. " 07/18/2009 - "Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial." 01/08/2004 - "METHODS: In a double-blind, multinational trial, we randomly assigned 330 patients with idiopathic pulmonary fibrosis that was unresponsive to corticosteroid therapy to receive subcutaneous interferon gamma-1b or placebo. " 01/08/2004 - "A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis." Order ALL the reference details at left...

Therapies and Procedures

1.	Lung Transplantation 10/01/2006 - "CONCLUSION: Lung transplantation is effective in improving the survival of selected patients with idiopathic pulmonary fibrosis." 08/01/2003 - "CONCLUSIONS: Lung transplantation is effective in improving the survival of selected patients affected by idiopathic pulmonary fibrosis." 04/01/2006 - "Lung transplantation has been the only therapy shown to improve survival of those with idiopathic pulmonary fibrosis." 09/01/2005 - "The aim of the study was to evaluate the quality of life and its changes in patients with idiopathic pulmonary fibrosis (IPF), waiting for lung transplantation. " 08/01/2003 - "METHODS: Forty-six patients accepted for lung transplantation during a 12-year period with a diagnosis of idiopathic pulmonary fibrosis form the basis of this study. " Order ALL the reference details at left...
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. " 08/01/2008 - "Pharmacotherapy in patients with idiopathic pulmonary fibrosis." 01/01/2006 - "Idiopathic pulmonary fibrosis : new concepts in pathogenesis and implications for drug therapy." 08/01/2004 - "Idiopathic pulmonary fibrosis: emerging concepts on pharmacotherapy." 04/01/2009 - "RATIONALE: The clinical management of idiopathic pulmonary fibrosis (IPF) remains a major challenge due to lack of effective drug therapy or accurate indicators for disease progression. " Order ALL the reference details at left...
3.	Transplants (Transplant) 08/01/2009 - "Patients in the high-risk group (LAS >46) were more likely to have idiopathic pulmonary fibrosis (IPF; 52.9% vs 23.8%, p < 0.001) and diabetes (25.8% vs 16.8%, p < 0.001) and to require mechanical ventilatory support (15.4% vs 2.2%, p < 0.001) at the time of transplant as compared with patients in the low-risk group. " 12/01/2008 - "Our aim was to evaluate post-transplant morbidity and survival of patients with sarcoid compared to recipients transplanted for idiopathic pulmonary fibrosis (IPF). " 03/15/2008 - "MEASUREMENTS AND MAIN RESULTS: We found that 58.8% (10/17) of patients with idiopathic pulmonary fibrosis, and 15.8% (6/38) of patients without idiopathic pulmonary fibrosis tested while on the wait list for a lung transplant were col(V) DTH positive. " 02/01/2006 - "Racial and ethnic disparities in survival in lung transplant candidates with idiopathic pulmonary fibrosis." 10/01/2000 - "The authors are revising the evolution of a female patient with idiopathic pulmonary fibrosis which suffered an unilateral lung transplant. " Order ALL the reference details at left...
4.	Coronary Artery Bypass (Coronary Artery Bypass Surgery) 11/01/2001 - "In this study we report a patient with end-stage idiopathic pulmonary fibrosis and pulmonary hypertension who received INO after coronary artery bypass surgery, with significant improvement in arterial oxygenation and pulmonary arterial pressure. " Order ALL the reference details at left...
5.	Aftercare (After-Treatment) 03/01/1981 - "5. Changes in the beta:alpha ratio were noted in five of six patients with cryptogenic fibrosing alveolitis after treatment, but on no occasion when two healthy subjects were assessed a total of nine times. " 12/01/2002 - "OBJECTIVE: We attempted to evaluate the feasibility (therapeutic efficacy, tolerance, and clinical courses after treatment) of pirfenidone, an anti-fibrotic drug for patients with chronic pulmonary fibroses such as idiopathic pulmonary fibrosis (IPF). " 06/01/2004 - "Th1 cytokine pattern (IL-12 and IL-18) in bronchoalveolar lavage fluid (BALF) before and after treatment with interferon gamma-1b (IFN-gamma-1b) or colchicine in patients with idiopathic pulmonary fibrosis (IPF/UIP)." Order ALL the reference details at left...

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