In 2000, IPF was defined as a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults and limited to the lungs. Usual interstitial pneumonia (UIP) is the histopathological pattern of IPF. IPF is characterized by progressive worsening of dyspnea and lung function and is associated with a poor prognosis.
Diagnostic Criteria for Idiopathic Pulmonary Fibrosis (IPF)
Diagnosis of IPF requires the following:
- Exclusion of other known causes of interstitial lung disease (ILD) (e.g., domestic and occupational environmental exposures, connective tissue disease (CTD), drug toxicity), and either #2 or #3:
- The presence of the HRCT pattern of UIP (Table 1)
- Specific combinations of HRCT patterns (Table 1) and histopathology patterns (Table 2) in patients subjected to lung tissue sampling
Table 1. High-Resolution Computed Tomography (HRCT) Scanning Patterns
|Indeterminate for UIP||
|Alternative Diagnosis||Findings suggestive of another diagnosis, including:
Table 2. Histopathology Patterns and Features
|Indeterminate for UIP||
*Granulomas, hyaline membranes (other than when associated with acute exacerbation of IPF, which may be the presenting manifestation in some patients), prominent airway-centered changes, areas of interstitial inflammation lacking associated fibrosis, marked chronic fibrous pleuritis, organizing pneumonia. Such features may not be overt or easily seen to the untrained eye and often need to be specifically sought.
†Features that should raise concerns about the likelihood of an alternative diagnosis include a cellular inflammatory infiltrate away from areas of honeycombing, prominent lymphoid hyperplasia including secondary germinal centers, and a distinctly bronchiolocentric distribution that could include extensive peribronchiolar metaplasia.
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Created May 15, 2019