Diagnosis of IPF

Usual Interstitial Pneumonia on HRCT, Zoom In

Used with permission from McGraw-Hill Companies, Inc. Copyright © 2007 McGraw-Hill Companies, Inc.

Idiopathic pulmonary fibrosis (IPF) is a complex, progressive scarring lung disease of unknown etiology.12 IPF has a variable and unpredictable clinical course and a poor prognosis, with a 5-year survival rate that is worse than several types of cancer.1345 For more information about the clinical course of IPF, see Natural History and Potential Risk Factors.

Because of the poor prognosis, there is a critical need to make an early and accurate diagnosis of IPF. This allows for appropriate management of symptoms and quality-of-life issues in patients with this disease.26 For patients with advanced disease, early diagnosis of IPF allows for timely referral to a lung transplant center.

Evolution of the Diagnostic Criteria

In 2000, the American Thoracic Society (ATS) and the European Respiratory Society (ERS) published the first international consensus statement on the diagnosis and treatment of IPF.7 Over the last decade, accumulating clinical data and observations from clinical studies in IPF have led to the development of evidence-based guidelines for the diagnosis and clinical management of this disease.28

The updated 2011 guidelines, published by the ATS, the ERS, the Japanese Respiratory Society (JRS), and the Latin American Thoracic Association (ALAT), provide new evidence-based criteria that simplify the diagnostic process and facilitate an earlier and more confident clinical diagnosis of IPF, many times without the need for surgical biopsy.689

Current Diagnostic Criteria

According to the 2011 ATS/ERS/JRS/ALAT guideline criteria, the diagnosis of IPF requires the following8:

  • Exclusion of other known causes of interstitial lung disease (ILD)
  • A usual interstitial pneumonia (UIP) pattern on high-resolution computerized tomography (HRCT) in patients without surgical lung biopsy
  • Specific combinations of HRCT and surgical lung biopsy pattern in patients who undergo a surgical lung biopsy

Content contributed by:
Ryan Hadley, MD
University of Michigan