Idiopathic pulmonary fibrosis (IPF) is the most common form of chronic, progressive fibrosing interstitial pneumonia of unknown origin. The histopathology and radiological findings of IPF are the same as those of usual interstitial pneumonia (UIP), and similar to many other cardiopulmonary conditions, the presenting signs and symptoms of IPF are usually nonspecific and include both respiratory and systemic manifestations (eg, shortness of breath, persistent cough, loss of appetite, weight loss).
IPF predominantly affects men, who typically present with chronic cough and progressive respiratory distress of 6 months or longer in duration. Patients with IPF have a complicated clinical course and a poor prognosis. The median survival of patients with IPF is 3-5 years from diagnosis. Although early diagnosis and treatment can relieve symptoms and slow disease progression, many patients eventually succumb to respiratory failure. However, lung transplantation may be curative and is the only interventional modality that has been shown to increase survival time in patients with IPF.
Because of the insidious onset and progression of IPF, diagnosis is often challenging and is often one of exclusion. Unfortunately, many patients with IPF are initially misdiagnosed and receive incorrect and/or delayed therapy before accurate diagnosis.
Are you familiar with key criteria and methods for diagnosing IPF? Test your knowledge with this quick quiz.
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Cite this: Guy W. Soo Hoo. Fast Five Quiz: Diagnosing Idiopathic Pulmonary Fibrosis - Medscape - Feb 04, 2021.