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A Guide to Idiopathic Pulmonary Fibrosis for
Physicians and Healthcare Professionals
Idiopathic pulmonary fibrosis (IPF) is a progressive and generally fatal interstitial lung disease
characterized by a unique pattern of scarring, inflammation, proliferation of fibroblasts, and
deposition of connective-tissue matrix proteins in the lungs. This scarring (fibrosis) and inflammation
result in dyspnea and poor gas exchange, eventually leading to death.
Historically, a lack of agreed-upon guidelines along with clinical features that mimic other pulmonary
diseases have made the correct diagnosis of IPF very difficult. A recently released
international consensus statement (authored by the American Thoracic Society [ATS] and the
European Respiratory Society [ERS] in collaboration with the American College of Chest Physicians [ACCP]
provides physicians with practical, up-to-date guidelines for the diagnosis and treatment of IPF (1). In addition, a better understanding of the mechanisms
behind IPF is driving exploration into novel treatment options—some of which are already in clinical trials.
This section of the IPF Web site, intended for pulmonologists and specialists in related fields, highlights recent
advances in the diagnosis, research, and treatment of IPF. It also provides links to additional resources for
healthcare professionals involved with this debilitating and elusive disease.
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