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12 Diagnosis Patient suspected to have IPF Patients with suspected IPF (i.e., unexplained symptomatic or asymptomatic bilateral pulmonary infiltrates on a chest radiograph or chest CT scan, bibasilar inspiratory crackles, and age older than 60 yr), unexplained dyspnea on exertion, and/or cough with evidence of ILD should be carefully evaluated for potential and/or identifiable causes of ILD, such as domestic and occupational environmental exposures, CTD, or drug toxicity. Middle-aged adults (>40 yr and <60 yr), especially patients with risks for familial pulmonary fibrosis, can rarely present with the otherwise same clinical scenario as the typical patient >60 years. If a potential cause for ILD is identified, the patient should undergo a thorough evaluation to confirm or exclude other known causes, such as hypersensitivity pneumonitis, CTD, pneumoconiosis, and iatrogenic causes (e.g., drug toxicity, irradiation). If a specific diagnosis is not made or no potential cause for ILD is identified, further evaluation is influenced by the patterns of HRCT images of the chest and supportive clinical findings surfaced in the course of multidisciplinary discussion to ascertain or exclude the diagnosis of IPF. IPF is diagnosed if the appropriate combination of HRCT patterns and histopathological patterns are present. * Surgical lung biopsy is not indicated in patients at high risk for intra-, peri-, or postoperative complications (e.g., severe hypoxemia at rest and/or severe pulmonary hypertension with a diffusion capacity less than 25% aer correction for hematocrit). Surgical lung biopsy may be unnecessary in some familial cases. e panel has no recommendation for or against conventional transbronchial biopsy and/ or cryobiopsy. However, if performed, histopathology may be sufficient in selected patients. Potential cause/associated condition NO YES Further evaluation (including HRCT) Chest HRCT pattern NO Specific diagnosis YES probable UIP, indeterminate for UIP, alternative diagnosis BAL Surgical lung biopsy* Alternative diagnosis MDD UIP MDD IPF per Figure 8 Not IPF Figure 9. Diagnostic Algorithm for IPF