Idiopathic pulmonary fibrosis diagnostic study of choice

The diagnostic criteria for IPF includes major criteria and minor criteria. The major criteria must include exclusion of other causes of interstitial lung diseaes, pulmonary function tests that is evident of lung fibrosis, bibasilar reticular abnormalities in CT lung, and lung biopsy shows fibrosis. The minor criteria should include 3 of 4 criterion as age > 50, duration of illness more than 3 months, bibasilar inspiratory crackles, and insidious onset of exertional dyspnea.

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.^[1] 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

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