Idiopathic pulmonary fibrosis diagnostic study of choice

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Criteria

The diagnosis of IPF can be made by demonstrating UIP pattern on lung biopsy in a patient without clinical features suggesting an alternate diagnosis (see clinical features, above). Establishing the diagnosis of IPF without a lung biopsy has been shown to be reliable when expert clinicians and radiologists concur that the presenting features are typical of IPF. Based on this evidence, the 2002 ATS/ERS Multidisciplinary Consensus Statement on the Idiopathic Interstitial Pneumonias proposes the following criteria for establishing the diagnosis of IPF without a lung biopsy:

Major criteria (all 4 required):

• Exclusion of other known causes of interstitial lung disease (drugs, exposures, connective tissue diseases)
• Abnormal pulmonary function tests with evidence of restriction (reduced vital capacity) and impaired gas exchange (pO₂, p(A-a)O₂, DLCO)
• Bibasilar reticular abnormalities with minimal ground glass on high-resolution CT scans
• Transbronchial lung biopsy or bronchoalveolar lavage (BAL) showing no features to support an alternative diagnosis

Minor criteria (3 of 4 required):

• Age > 50
• Insidious onset of otherwise unexplained exertional dyspnea
• Duration of illness > 3 months
• Bibasilar inspiratory crackles