Serum biomarkers in idiopathic pulmonary fibrosis.
Summary of "Serum biomarkers in idiopathic pulmonary fibrosis."
Within the group of Idiopathic Interstitial Pneumonias (IIPs), above all Idiopathic Pulmonary Fibrosis (IPF) poses a considerable diagnostic and therapeutic problem. Although genetic profiling indicates that IPF, Non Specific Interstitial Pneumonia (NSIP), and chronic hypersensitivity pneumonitis (HP) are distinctly different diseases, in every day practice these diseases can be difficult to tell apart. Furthermore, treatment of these diseases is notoriously difficult. Serum biomarkers reflect our understanding of the underlying pathogenesis and potentially fulfill a role in establishing a diagnosis, prognosis and therapy. While no single biomarker is currently able to accurately predict the presence or absence of an IIP, a composite of several markers holds promise for the future. Several biomarkers, such as KL-6, surfactant proteins and circulating fibrocytes, appear to contribute to our insight into disease progression and prognosis. It is however uncertain whether these markers give us additional information to common diagnostic tests and their value has as yet to be validated for every day practice. Fortunately, the potential of biomarkers is increasingly recognized and biomarker data are prospectively gathered in current placebo-controlled therapeutic trials.
Affiliation
Department of Pulmonary Medicine, Erasmus MC, 's-Gravendijkwal 230, 3015 CE Rotterdam, the Netherlands.
Journal Details
This article was published in the following journal.
Name: Pulmonary pharmacology & therapeutics
ISSN: 1522-9629
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/20708704
- DOI: http://dx.doi.org/10.1016/j.pupt.2010.08.001
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...
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...
Clinical Trials
Study purpose: The disease course of idiopathic pulmonary fibrosis (IPF) is variable. During the course of the disease some patients will get better, some will stay the same, and others...
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.
Efficacy and Safety of Oral Bosentan in Patients With Idiopathic Pulmonary Fibrosis
Endothelin-1 (ET-1) is expressed in a variety of pulmonary pathological conditions including pulmonary vascular disease and pulmonary fibrosis. Bosentan (an oral dual ET-1 receptor antago...
Clinical Study With QAX576 in Patients With Idiopathic Pulmonary Fibrosis
The purpose of this study is to investigate how QAX576 affects levels of interleukin 13 (IL-13) in patients with idiopathic pulmonary fibrosis (IPF).
