ATS GUIDELINES Bundle

Idiopathic Pulmonary Fibrosis Diagnosis

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3 Diagnosis Table 1. Comparison of ATS/ERS/JRS/ALAT Recommendations for the Diagnosis of IPF in the 2011 and 2018 Guidelines HRCT Pattern of Probable UIP, Indeterminate for UIP, and Alternative Diagnosis HRCT Pattern of UIP 2011 Guideline: Did Not Distinguish among Patients with Different HRCT Patterns BAL cellular analysis We suggest performing BAL cellular analysis (C) We suggest NOT performing BAL cellular analysis (C) BAL cellular analysis should not be performed in the diagnostic evaluation of IPF in the majority of patients, but may be appropriate in a minority of patients. Surgical lung biopsy We suggest performing surgical lung biopsy (C) We recommend NOT performing surgical lung biopsy (S) Surgical lung biopsy is not required for patients with an HRCT pattern consistent with UIP. Transbronchial lung biopsy No recommendation for or against We recommend NOT performing transbronchial lung biopsy (S) Transbronchial biopsy should not be used in the evaluation of IPF in the majority of patients, but may be appropriate in a minority. Lung cryobiopsy No recommendation for or against We recommend NOT performing cryobiopsy (S) Not addressed Medical history of medication use and environmental exposures We recommend taking a detailed history of both medication use and environmental exposures at home, work, and other places the patient frequently visits to exclude potential causes of ILD (motherhood statement) Diagnosis of IPF requires exclusion of other known causes of ILD (e.g., domestic and occupational environmental exposures, connective tissue disease, and drug toxicity). Serological testing to exclude connective tissue disease We recommend serological testing to exclude connective tissue diseases as a potential cause of the ILD (motherhood statement) Multidisciplinary discussion We suggest multidisciplinary discussion for decision-making (C) We recommend that a multidisciplinary discussion should be used in the evaluation of IPF. Serum biomarkers We recommend NOT measuring serum MMP-7, SPD, CCL-18, or KL-6 for the purpose of distinguishing IPF from other ILDs (S) Not addressed e quality of evidence for all recommendations in the 2018 guideline was very low. S= strong recommendation; C = conditional recommendation.

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