2
Key Points
➤ Adult patients with newly detected interstitial lung disease (ILD)
of apparently unknown cause are clinically suspected of having
idiopathic pulmonary fibrosis (IPF) if they have unexplained
symptomatic or asymptomatic patterns of bilateral fibrosis on a
chest radiograph or chest computed tomography (CT) scan, bibasilar
inspiratory crackles, and an age typically older than 60 years.
➤ Rarely, middle-aged adults (>40 yr and <60 yr), especially those with
risks for familial pulmonary fibrosis, may otherwise manifest the
same clinical scenario as the typical patient older than 60 years.
➤ The recommendations in this guideline are for the patterns and
distributions of images obtained by high-resolution CT (HRCT)
imaging and, thus, require that patients be subjected to HRCT of the
chest for evaluation.
Diagnosis
Diagnostic Criteria for IPF
1. Exclusion of other known causes of ILD (e.g., domestic and
occupational environmental exposures, CTD, drug toxicity), and
either #2 or #3:
2. The presence of the HRCT pattern of UIP (Table 3)
3. Specific combinations (Figure 8) of HRCT patterns (Table 3) and
histopathology patterns (Table 4) in patients subjected to lung
tissue sampling
