The American Thoracic Society has released new official clinical guidelines on the diagnosis and management of idiopathic pulmonary fibrosis (IPF). The statement replaces ATS guidelines published in 2000, and reviews current knowledge in the epidemiology, etiology, diagnosis and management of IPF, as well as available treatment options, including pharmacologic and non-pharmacologic therapies and palliative care. The statement appears in the March 15, 2011 issue of American Journal of Respiratory and Critical Care Medicine. IPF is a chronic, progressive, fatal form of fibrotic lung disease, characterized by shortness of breath during exertion, which occurs primarily in relatively older adults. The etiology of IPF is unclear. The disease occurs when injury to the lungs is triggered by an unknown cause, resulting in the formation of scar tissue that causes the lungs to become thickened and stiff. IPF may progress slowly over several years and may be punctuated by episodes of acute respiratory decline. Lung transplantation is a feasible treatment option in highly selected patients. A subgroup of patients with IPF has a genetic predisposition to the disease. "In the decade since the publication of the previous statement on IPF, studies have used the criteria for the diagnosis of IPF and recommendations published in the previous consensus-based statement to further our understanding of the clinical manifestations and course of IPF, and there has been an increasing body of evidence pertinent to its clinical management," said Dr. Ganesh Raghu, director of the Interstitial Lung Disease/Sarcoid/Pulmonary Fibrosis Program at the University of Washington Medical Center in Seattle and chair of the collaborative committee that drafted the statement.
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