Published in Am J Respir Crit Care Med on September 27, 2007
Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med (2012) 6.51
Strategies for treating idiopathic pulmonary fibrosis. Nat Rev Drug Discov (2010) 3.20
The SF-36 and SGRQ: validity and first look at minimum important differences in IPF. Respir Med (2009) 2.18
Interstitial lung abnormalities and reduced exercise capacity. Am J Respir Crit Care Med (2012) 1.80
Bosentan in pulmonary hypertension associated with fibrotic idiopathic interstitial pneumonia. Am J Respir Crit Care Med (2014) 1.73
Identification of early interstitial lung disease in smokers from the COPDGene Study. Acad Radiol (2009) 1.63
Study design implications of death and hospitalization as end points in idiopathic pulmonary fibrosis. Chest (2014) 1.51
Subclinical interstitial lung disease: why you should care. Am J Respir Crit Care Med (2012) 1.46
The 6 minute walk in idiopathic pulmonary fibrosis: longitudinal changes and minimum important difference. Thorax (2009) 1.46
Lung function in idiopathic pulmonary fibrosis--extended analyses of the IFIGENIA trial. Respir Res (2009) 1.34
Predicting pulmonary fibrosis disease course from past trends in pulmonary function. Chest (2014) 1.25
Novel therapeutic approaches for pulmonary fibrosis. Br J Pharmacol (2011) 1.24
Patient-reported dyspnea in COPD reliability and association with stage of disease. Chest (2009) 1.23
Endothelin-1 and transforming growth factor-beta1 independently induce fibroblast resistance to apoptosis via AKT activation. Am J Respir Cell Mol Biol (2009) 1.20
A review of current and novel therapies for idiopathic pulmonary fibrosis. J Thorac Dis (2013) 1.18
Determinants of initiation and progression of idiopathic pulmonary fibrosis. Respirology (2009) 1.16
Development and validity testing of an IPF-specific version of the St George's Respiratory Questionnaire. Thorax (2010) 1.14
Undifferentiated connective tissue disease-associated interstitial lung disease: changes in lung function. Lung (2010) 1.11
Suspected acute exacerbation of idiopathic pulmonary fibrosis as an outcome measure in clinical trials. Respir Res (2013) 1.10
Interstitial Lung Disease in Idiopathic Inflammatory Myopathy. Curr Rheumatol Rev (2010) 1.07
The clinical significance of 5% change in vital capacity in patients with idiopathic pulmonary fibrosis: extended analysis of the pirfenidone trial. Respir Res (2011) 1.06
Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction. Chest (2013) 1.05
New insights into the pathogenesis and treatment of idiopathic pulmonary fibrosis. Drugs (2011) 1.03
New therapeutic targets in idiopathic pulmonary fibrosis. Aiming to rein in runaway wound-healing responses. Am J Respir Crit Care Med (2014) 1.02
Echocardiographic and hemodynamic predictors of mortality in idiopathic pulmonary fibrosis. Chest (2013) 1.02
Interstitial lung disease in connective tissue diseases: evolving concepts of pathogenesis and management. Arthritis Res Ther (2010) 1.02
Treatment of idiopathic pulmonary fibrosis with losartan: a pilot project. Lung (2012) 0.98
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Severe pulmonary hypertension in idiopathic nonspecific interstitial pneumonia. Pulm Circ (2012) 0.94
Cellular mechanisms of tissue fibrosis. 8. Current and future drug targets in fibrosis: focus on Rho GTPase-regulated gene transcription. Am J Physiol Cell Physiol (2014) 0.92
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Update on therapeutic management of idiopathic pulmonary fibrosis. Ther Clin Risk Manag (2015) 0.91
Fibrocytes and the pathogenesis of diffuse parenchymal lung disease. Fibrogenesis Tissue Repair (2012) 0.89
Innovative approaches to the therapy of fibrosis. Curr Opin Rheumatol (2009) 0.88
Patient-reported outcomes in idiopathic pulmonary fibrosis research. Chest (2012) 0.88
The psychometric properties of the St George's Respiratory Questionnaire (SGRQ) in patients with idiopathic pulmonary fibrosis: a literature review. Health Qual Life Outcomes (2014) 0.87
Using bosentan to treat paraquat poisoning-induced acute lung injury in rats. PLoS One (2013) 0.87
Idiopathic pulmonary fibrosis: diagnostic pitfalls and therapeutic challenges. Multidiscip Respir Med (2012) 0.87
Idiopathic Pulmonary Fibrosis: Gender-Age-Physiology Index Stage for Predicting Future Lung Function Decline. Chest (2016) 0.86
Optimizing the 6-min walk test as a measure of exercise capacity in COPD. Chest (2012) 0.86
The role of endothelin-1 in the pathogenesis of idiopathic pulmonary fibrosis. BioDrugs (2010) 0.86
The treatment of idiopathic pulmonary fibrosis. F1000Prime Rep (2014) 0.86
The current position of surgical lung biopsy in the diagnosis of idiopathic pulmonary fibrosis. Respir Res (2013) 0.85
Pharmacological Treatment of Idiopathic Pulmonary Fibrosis: Current Approaches, Unsolved Issues, and Future Perspectives. Biomed Res Int (2015) 0.83
Plasma endothelin-1 and vascular endothelial growth factor levels and their relationship to hemodynamics in idiopathic pulmonary fibrosis. Respiration (2012) 0.83
Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias. PLoS One (2015) 0.83
Pirfenidone treatment of idiopathic pulmonary fibrosis. Ther Clin Risk Manag (2011) 0.83
Systemic Sclerosis-Associated Interstitial Lung Disease: Lessons from Clinical Trials, Outcome Measures, and Future Study Design. Curr Rheumatol Rev (2010) 0.82
Pulmonary hypertension and right heart dysfunction in chronic lung disease. Biomed Res Int (2014) 0.82
Pharmacologic therapies for idiopathic pulmonary fibrosis, past and future. Ann Med (2015) 0.81
Treatment of pulmonary hypertension in idiopathic pulmonary fibrosis: shortfall in efficacy or trial design? Drug Des Devel Ther (2014) 0.81
The efficiency of a urotensin II antagonist in an experimental lung fibrosis model. Inflammation (2012) 0.80
Treatment of rheumatoid arthritis-associated interstitial lung disease: a perspective review. Ther Adv Musculoskelet Dis (2015) 0.80
Health-related quality of life and 6 min walk distance in patients with idiopathic pulmonary fibrosis. Can Respir J (2011) 0.80
Diagnostic workup for diffuse parenchymal lung disease: schematic flowchart, literature review, and pitfalls. Lung (2012) 0.80
Quality of life and dyspnoea in patients treated with bosentan for idiopathic pulmonary fibrosis (BUILD-1). Eur Respir J (2009) 0.80
Idiopathic Pulmonary Fibrosis: Adaptive Multiple Features Method Fibrosis Association with Outcomes. Am J Respir Crit Care Med (2016) 0.79
Treatment of idiopathic pulmonary fibrosis: a network meta-analysis. BMC Med (2016) 0.79
Grape seed pro-anthocyanidins ameliorates radiation-induced lung injury. J Cell Mol Med (2014) 0.79
Quantitative assessment of lung ventilation and microstructure in an animal model of idiopathic pulmonary fibrosis using hyperpolarized gas MRI. Acad Radiol (2010) 0.79
Managing comorbidities in idiopathic pulmonary fibrosis. Int J Gen Med (2015) 0.79
New perspectives on management of idiopathic pulmonary fibrosis. Ther Adv Chronic Dis (2016) 0.79
Macitentan inhibits the transforming growth factor-β profibrotic action, blocking the signaling mediated by the ETR/TβRI complex in systemic sclerosis dermal fibroblasts. Arthritis Res Ther (2015) 0.79
Targeted therapy for systemic sclerosis: how close are we? Nat Rev Rheumatol (2010) 0.78
Effect and safety of mycophenolate mofetil in idiopathic pulmonary fibrosis. Pulm Med (2011) 0.78
Clinical trials and tribulations--lessons from pulmonary fibrosis. QJM (2012) 0.78
Novel Therapeutic Strategies for Reducing Right Heart Failure Associated Mortality in Fibrotic Lung Diseases. Biomed Res Int (2015) 0.78
The diagnosis and treatment of idiopathic pulmonary fibrosis. Dtsch Arztebl Int (2013) 0.78
Idiopathic pulmonary fibrosis: new insights on functional characteristics at diagnosis. Can Respir J (2014) 0.78
Pulmonary hypertension in parenchymal lung disease. Pulm Med (2012) 0.77
Patient considerations and drug selection in the treatment of idiopathic pulmonary fibrosis. Ther Clin Risk Manag (2016) 0.77
Macitentan for the treatment of pulmonary arterial hypertension. Vasc Health Risk Manag (2014) 0.77
Advances in the management of idiopathic pulmonary fibrosis. F1000 Med Rep (2010) 0.77
Exercise peripheral oxygen saturation (SpO2) accurately reflects arterial oxygen saturation (SaO2) and predicts mortality in systemic sclerosis. Thorax (2009) 0.76
Cardiorespiratory function before and after aerobic exercise training in patients with interstitial lung disease. J Cardiopulm Rehabil Prev (2014) 0.76
Idiopathic pulmonary fibrosis is associated with circulating antiepithelial antibodies. Lung (2012) 0.76
Scleroderma therapy: clinical overview of current trends and future perspective. Rheumatol Int (2012) 0.76
Understanding and optimizing health-related quality of life and physical functional capacity in idiopathic pulmonary fibrosis. Patient Relat Outcome Meas (2016) 0.75
Bosentan for the treatment of idiopathic pulmonary fibrosis. Expert Opin Investig Drugs (2008) 0.75
Clinical Adverse Effects of Endothelin Receptor Antagonists: Insights From the Meta-Analysis of 4894 Patients From 24 Randomized Double-Blind Placebo-Controlled Clinical Trials. J Am Heart Assoc (2016) 0.75
Bosentan: a rapidly expanding role in the management of cardio-thoracic diseases. Rheumatol Int (2008) 0.75
Pirfenidone in the treatment of idiopathic pulmonary fibrosis: an evidence-based review of its place in therapy. Core Evid (2016) 0.75
Pulmonary Hypertension Associated with Idiopathic Pulmonary Fibrosis: Current and Future Perspectives. Can Respir J (2017) 0.75
Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations. Clin Med Insights Circ Respir Pulm Med (2016) 0.75
Reduced hypoxia risk in a systemic sclerosis patient with interstitial lung disease after long-term pulmonary rehabilitation. Clin Med Insights Case Rep (2011) 0.75
An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med (2011) 22.39
Bosentan therapy for pulmonary arterial hypertension. N Engl J Med (2002) 11.18
Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med (2014) 10.62
A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med (2014) 9.40
An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med (2013) 6.82
Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med (2011) 6.74
Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med (2007) 6.71
Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med (2012) 6.51
A common MUC5B promoter polymorphism and pulmonary fibrosis. N Engl J Med (2011) 6.40
An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med (2015) 6.03
A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med (2004) 6.02
Adult-onset pulmonary fibrosis caused by mutations in telomerase. Proc Natl Acad Sci U S A (2007) 5.95
Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet (2011) 5.94
Induction of epithelial-mesenchymal transition in alveolar epithelial cells by transforming growth factor-beta1: potential role in idiopathic pulmonary fibrosis. Am J Pathol (2005) 5.88
Telomere shortening in familial and sporadic pulmonary fibrosis. Am J Respir Crit Care Med (2008) 5.82
An official American Thoracic Society clinical practice guideline: the clinical utility of bronchoalveolar lavage cellular analysis in interstitial lung disease. Am J Respir Crit Care Med (2012) 5.37
Idiopathic pulmonary fibrosis: clinically meaningful primary endpoints in phase 3 clinical trials. Am J Respir Crit Care Med (2012) 5.33
Clinical and pathologic features of familial interstitial pneumonia. Am J Respir Crit Care Med (2005) 4.25
The clinical course of patients with idiopathic pulmonary fibrosis. Ann Intern Med (2005) 4.25
The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med (2005) 4.15
Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med (2008) 3.97
Inhaled iloprost for severe pulmonary hypertension. N Engl J Med (2002) 3.90
Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med (2003) 3.89
Short telomeres are a risk factor for idiopathic pulmonary fibrosis. Proc Natl Acad Sci U S A (2008) 3.86
Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med (2011) 3.85
Sarcoidosis. Lancet (2003) 3.60
Diagnosis and management of cough executive summary: ACCP evidence-based clinical practice guidelines. Chest (2006) 3.59
A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma. Arthritis Rheum (2006) 3.45
Gene expression profiling of familial and sporadic interstitial pneumonia. Am J Respir Crit Care Med (2006) 3.32
BUILD-3: a randomized, controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med (2011) 3.23
Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet (2009) 3.21
Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med (2008) 3.19
Genome-wide association study identifies multiple susceptibility loci for pulmonary fibrosis. Nat Genet (2013) 3.17
Clazosentan, an endothelin receptor antagonist, in patients with aneurysmal subarachnoid haemorrhage undergoing surgical clipping: a randomised, double-blind, placebo-controlled phase 3 trial (CONSCIOUS-2). Lancet Neurol (2011) 3.08
Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med (2003) 3.07
Histopathologic subsets of fibrosing alveolitis in patients with systemic sclerosis and their relationship to outcome. Am J Respir Crit Care Med (2002) 3.06
Rheumatoid arthritis-interstitial lung disease-associated mortality. Am J Respir Crit Care Med (2010) 3.05
Expression of human herpesvirus 8 in primary pulmonary hypertension. N Engl J Med (2003) 3.00
Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med (2004) 2.97
A polymorphism in the CTGF promoter region associated with systemic sclerosis. N Engl J Med (2007) 2.95
Pulmonary fibrosis associated with aluminum trihydrate (Corian) dust. N Engl J Med (2014) 2.88
Idiopathic pulmonary fibrosis: a composite physiologic index derived from disease extent observed by computed tomography. Am J Respir Crit Care Med (2003) 2.78
Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease. J Rheumatol (2013) 2.74
Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med (2007) 2.66
Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med (2008) 2.65
Sarcoidosis-related mortality in the United States from 1988 to 2007. Am J Respir Crit Care Med (2011) 2.62
Forced vital capacity in patients with idiopathic pulmonary fibrosis: test properties and minimal clinically important difference. Am J Respir Crit Care Med (2011) 2.62
Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med (2011) 2.54
Connective tissue disease-associated interstitial lung disease: a call for clarification. Chest (2010) 2.51
Human pulmonary chimerism after hematopoietic stem cell transplantation. Am J Respir Crit Care Med (2003) 2.50
Role of surfactant proteins A, D, and C1q in the clearance of apoptotic cells in vivo and in vitro: calreticulin and CD91 as a common collectin receptor complex. J Immunol (2002) 2.43
Pigment epithelium-derived factor in idiopathic pulmonary fibrosis: a role in aberrant angiogenesis. Am J Respir Crit Care Med (2004) 2.42
Six-minute-walk test in idiopathic pulmonary fibrosis: test validation and minimal clinically important difference. Am J Respir Crit Care Med (2010) 2.40
Fibroblast foci are not discrete sites of lung injury or repair: the fibroblast reticulum. Am J Respir Crit Care Med (2006) 2.34
Endothelin-1 promotes myofibroblast induction through the ETA receptor via a rac/phosphoinositide 3-kinase/Akt-dependent pathway and is essential for the enhanced contractile phenotype of fibrotic fibroblasts. Mol Biol Cell (2004) 2.29
Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA (2013) 2.20
Telomere lengths, pulmonary fibrosis and telomerase (TERT) mutations. PLoS One (2010) 2.19
Elastase-mediated phosphatidylserine receptor cleavage impairs apoptotic cell clearance in cystic fibrosis and bronchiectasis. J Clin Invest (2002) 2.19
The SF-36 and SGRQ: validity and first look at minimum important differences in IPF. Respir Med (2009) 2.18
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax (2006) 2.10
Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest (2004) 2.00
Prognostic implications of histologic patterns in multiple surgical lung biopsies from patients with idiopathic interstitial pneumonias. Chest (2004) 1.95
Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis? Am J Respir Crit Care Med (2007) 1.94
High-resolution computed tomography in idiopathic pulmonary fibrosis: diagnosis and prognosis. Am J Respir Crit Care Med (2005) 1.90
All-cause mortality rate in patients with idiopathic pulmonary fibrosis. Implications for the design and execution of clinical trials. Am J Respir Crit Care Med (2014) 1.89
Constitutive ALK5-independent c-Jun N-terminal kinase activation contributes to endothelin-1 overexpression in pulmonary fibrosis: evidence of an autocrine endothelin loop operating through the endothelin A and B receptors. Mol Cell Biol (2006) 1.87
High incidence of ganciclovir-resistant cytomegalovirus infection among lung transplant recipients receiving preemptive therapy. J Infect Dis (2001) 1.85
Increased risk of pulmonary embolism among US decedents with sarcoidosis from 1988 to 2007. Chest (2011) 1.85
Brain natriuretic peptide and exercise capacity in lung fibrosis and pulmonary hypertension. Am J Respir Crit Care Med (2004) 1.84
A detailed evaluation of acute respiratory decline in patients with fibrotic lung disease: aetiology and outcomes. Respirology (2010) 1.80
Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med (2014) 1.80
The relationship between individual histologic features and disease progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med (2002) 1.80