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INSIDE ▶ Introduction ▶ Recommendations ▶ Tables ▶ Figures Idiopathic Pulmonary Fibrosis and Progressive Pulmonary Fibrosis

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2 Introduction Part I. Update on the Diagnosis and Treatment of IPF ➤ We suggest transbronchial lung cryobiopsy (TBLC) be regarded as an acceptable alternative to surgical lung biopsy for making a histopathological diagnosis in patients with ILD of undetermined type in medical centers with experience performing and interpreting TBLC (conditional recommendation, very low-quality evidence). ➤ We make no recommendation for or against the addition of genomic classifier testing (GCT) for the purpose of diagnosing usual interstitial pneumonia (UIP) in patients with ILD of undetermined type who are undergoing transbronchial forceps biopsy, due to insufficient agreement among the committee members. Remarks: There was consensus that genomic classifier testing should be reconsidered once additional studies are published. ➤ We suggest not treating patients with IPF with antacid medication for the purpose of improving respiratory outcomes (conditional recommendation, very low-quality evidence). Remarks: Antacid medication and other interventions may be appropriate for patients with both IPF and symptoms of gastroesophageal reflux disease (GERD) for the purpose of improving gastroesophageal reflux (GER) -related outcomes in accordance with GER-specific guidelines. ➤ We suggest not referring patients with IPF for anti-reflux surgery for the purpose of improving respiratory outcomes (conditional recommendation, very low-quality evidence). Remarks: Anti-reflux surgery may be appropriate for patients with both IPF and symptoms of GERD for the purpose of improving GER-related outcomes in accordance with GER-specific guidelines. ➤ Progress in the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILDs) manifesting progressive pulmonary fibrosis (PPF) has generated the need for updating guidelines. ➤ These recommendations are not mandates because they cannot account for all unique clinical circumstances and should be revisited as new evidence is published. Recommendations

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