Sildenafil in idiopathic pulmonary fibrosis.
Summary of "Sildenafil in idiopathic pulmonary fibrosis."
To the Editor: Pulmonary hypertension develops in 37 to 59% of patients with end-stage idiopathic pulmonary fibrosis.(1),(2) Although it is not explicitly stated, the Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis (STEP-IPF) (Aug. 12 issue)(3) was designed as a study of the treatment of pulmonary hypertension in patients with end-stage idiopathic pulmonary fibrosis. Despite this fact, enrollment was determined by the severity of lung physiology and not by any measure of pulmonary hypertension. Although this pragmatic approach to case selection obviated the need for right heart catheterization, it resulted in a negative primary outcome. However, sildenafil therapy . . .
Affiliation
Journal Details
This article was published in the following journal.
Name: The New England journal of medicine
ISSN: 1533-4406
Pages: 2169-71
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/21105805
- DOI: http://dx.doi.org/10.1056/NEJMc1010408
Medical and Biotech [MESH] Definitions
Idiopathic Interstitial Pneumonias
A group of interstitial lung diseases with no known etiology. There are several entities with varying patterns of inflammation and fibrosis. They are classified by their distinct clinical-radiological-pathological features and prognosis. They include IDIOPATHIC PULMONARY FIBROSIS; CRYPTOGENIC ORGANIZING PNEUMONIA; and others.
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.
Cardiomyopathy, Restrictive
A form of CARDIAC MUSCLE disease in which the ventricular walls are excessively rigid, impeding ventricular filling. It is marked by reduced diastolic volume of either or both ventricles but normal or nearly normal systolic function. It may be idiopathic or associated with other diseases (ENDOMYOCARDIAL FIBROSIS or AMYLOIDOSIS) causing interstitial fibrosis.
Pulmonary Fibrosis
A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.
Lung Diseases, Interstitial
A diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of PULMONARY ALVEOLI that extends to the interstitium and beyond leading to diffuse PULMONARY FIBROSIS. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features.
PubMed Articles
Sildenafil in idiopathic pulmonary fibrosis.
To the Editor: Pulmonary hypertension develops in 37 to 59% of patients with end-stage idiopathic pulmonary fibrosis.(1),(2) Although it is not explicitly stated, the Sildenafil Trial of Exercise Perf...
Sildenafil in idiopathic pulmonary fibrosis.
To the Editor: Pulmonary hypertension develops in 37 to 59% of patients with end-stage idiopathic pulmonary fibrosis.(1),(2) Although it is not explicitly stated, the Sildenafil Trial of Exercise Perf...
Sildenafil in idiopathic pulmonary fibrosis.
To the Editor: Pulmonary hypertension develops in 37 to 59% of patients with end-stage idiopathic pulmonary fibrosis.(1),(2) Although it is not explicitly stated, the Sildenafil Trial of Exercise Perf...
Pulmonary complications, including pulmonary fibrosis (PF) and pulmonary arterial hypertension (PAH), are the leading cause of mortality in patients with systemic sclerosis (SSc). The aim of this stud...
Treatment of pulmonary fibrosis : New substances and new interventions.
Idiopathic pulmonary fibrosis is a chronic progressive lung disease with poor prognosis. The IFIGENIA trial showed that antioxidative therapy with N-acetylcysteine versus placebo for patients under tr...
Clinical Trials
Targeting Vascular Reactivity in Idiopathic Pulmonary Fibrosis
The purpose of this study is to determine whether combination therapy with sildenafil and losartan can improve function and exercise tolerance in patients with idiopathic pulmonary fibros...
Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that affects an individual's ability to breathe. This study will evaluate the effectiveness of sildenafil, a medication that i...
Sildenafil Study to Treat Idiopathic Pulmonary Fibrosis
Medicines that decrease blood pressure in the lungs may help IPF patients function better. This study will test whether sildenafil improves the ability to exercise in patients with pulmona...
To assess the long-term safety and efficacy of oral pirfenidone in doses of up to 40 mg/kg/d in a limited number of patients with pulmonary fibrosis/idiopathic pulmonary fibrosis (PF/IPF)
Evaluation of Patients With Idiopathic Pulmonary Fibrosis (IPF) Through an IPF Registry
The purpose of this study is to create a database of demographics and samples in idiopathic pulmonary fibrosis.
