American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS) and Latin American Thoracic Association (ALAT) Management Guidelines state that a diagnosis of IPF requires the following:
Exclusion of other causes
Other known causes of interstitial lung disease (ILD) have to be excluded. These can be, for example, domestic and occupational environmental exposures, drug toxicity or connective tissue disease.1
- A thorough medical, occupational, environmental, and family history must be obtained in order to exclude known causes of ILD. A physical examination, physiological testing and laboratory evaluation also have to take place.1
- A multidisciplinary discussion between pulmonologists, radiologists and pathologists is helpful in excluding alternative aetiologies.1
UIP pattern on HRCT
In patients who did not undergo surgical lung biopsy, a usual interstitial pneumonia (UIP) pattern has to be present on high-resolution computed tomography (HRCT).1
- Surgical lung biopsy is not considered essential anymore; rather, the presence of a UIP pattern on HRCT is sufficient for the diagnosis of IPF in the majority of patients.1
Combining HRCT and lung biopsy results1
In patients subjected to surgical lung biopsy, specific combinations of HRCT and surgical lung biopsy patterns are required for a diagnosis of IPF.1
- The risks of a biopsy procedure must be weighed against the benefit of conclusively diagnosing IPF on a patient-by-patient basis.1
Diagnostic Algorithm for IPF
Reprinted with permission of the American Thoracic Society. Copyright© 2015 American Thoracic Society.
Raghu G, et al. An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management, Am J Respir Crit Care Med 2011;183;788-824. Official Journal of the American Thoracic Society. This document was published in March 2011 and is currently in revision. Certain aspects of this document may be out of date and caution should be used when applying these in clinical practice or other usages.
- Raghu G, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788–824.