Idiopathic Pulmonary Fibrosis Diagnosis

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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

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