Pulmonary fibrosis is a lung condition characterized by extensive scarring of the lung. This usually includes extensive deposition of collagen and other components of the extracellular matrix, loss of normal lung architecture and change in the functions of the cells in the lung that lead to shortness of breath and ultimately, respiratory failure. Pulmonary fibrosis can be triggered or worsened by environmental or occupational exposures: tobacco smoke, infection, drugs or can be secondary to autoimmune and inflammatory disorders. Idiopathic pulmonary fibrosis (IPF) is a disease in which progressive lung scarring occurs without an obvious cause. Idiopathic pulmonary fibrosis represents a significant health problem as it is estimated that close to 200,000 patients are affected in the US and over 5 million patients worldwide. The disease has a mortality rate of 50% in 3-5 years after diagnosis, but the course is variable. Based on current guidelines the diagnosis of idiopathic pulmonary fibrosis requires a multidisciplinary approach and is usually based on the patient’s history, pulmonary functions, high-resolution computed tomographic images and sometimes lung biopsy.
Exclusion of known causes of pulmonary fibrosis is required as therapeutic approaches may differ. Over the last decades there has been significant progress in idiopathic pulmonary fibrosis research. Using animal models of lung fibrosis, studies have identified pathways that lead to pulmonary fibrosis and promising new targets for therapeutic interventions. Significant progress has been made in understanding the role of gene variants in causing pulmonary fibrosis. The identification of genes that cause the less common familial form of pulmonary fibrosis provided significant insights into mechanisms of disease. Using advanced genomic technologies, in patients with the more common sporadic idiopathic pulmonary fibrosis, investigators have identified gene variants that are associated with idiopathic pulmonary fibrosis in more than a third of cases, providing support for a genetic predisposition for the disease in the general population.
Using novel technologies investigators identified genes and proteins in the blood stream that are characteristics of the disease and predictive outcomes that could potentially serve as biomarkers. A series of ambitious drug studies led to significant changes in the management of patients with idiopathic pulmonary fibrosis. A commonly prescribed immunosuppressive regimen was proven to be dangerous for patients and is no longer in use and replaced by two drugs (Pirfenidone and Nintedanib) that have been proven to slow down the progression of the disease, leading to their subsequent approval by the FDA. The scientific achievements of the recent decade have increased interest and awareness to idiopathic pulmonary fibrosis research and many exciting developments will follow. There are multiple additional drug studies that are under way and will be completed in the next few years. The National Heart, Lung and Blood Institute of the National Institutes of Health (NIH), despite limited funding, continues to fund translational clinical research in pulmonary fibrosis, and most recently in their Centers for Advanced Diagnostics and Experimental Therapeutics (CADET II) included several studies that focus on novel therapies for idiopathic pulmonary fibrosis. The American Thoracic Society in partnership with patient advocacy groups and medical professionals continue to both fund pulmonary fibrosis research as well as lobby for increased funding for research. The road to curing idiopathic pulmonary fibrosis is long but the breakthroughs in recent years indeed transformed our perception of idiopathic pulmonary fibrosis, from hopeless to hopeful.
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Article from American Thoracic Society