ATS GUIDELINES Bundle

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

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5 Indeterminate for UIP CT findings suggestive of an alternative diagnosis Provisional low confidence (51–69%) Low to very low confidence (≤50%) • Diffuse distribution without subpleural predominance • Peribronchovascular predominant with subpleural sparing (consider non-specific interstitial pneumonia [NSIP]) • Peri-lymphatic distribution (consider sarcoidosis) • Upper or mid lung (consider fibrotic hypersensitivity pneumonitis [HP], connective tissue disease-associated ILD [CTD-ILD], sarcoidosis) • Subpleural sparing (consider NSIP or smoking- related interstitial pneumonia [IP]) • CT features of lung fibrosis that do not suggest any specific etiology • Lung findings: ▶ Cysts (consider lymphangioleiomyomatosis [LAM], pulmonary Langerhan's cell histiocytosis [PLCH], lymphocytic interstitial pneumonia [LIP], desquamative interstitial pneumonia [DIP]) ▶ Mosaic attenuation or 3-density sign (consider HP) ▶ Predominant GGO (consider HP, smoking- related disease, drug toxicity, acute exacerbation of fibrosis) ▶ Profuse centrilobular micronodules (consider HP or smoking-related disease) ▶ Nodules (consider sarcoidosis) ▶ Consolidation (consider organizing pneumonia, etc.) • Mediastinal findings: ▶ Pleural plaques (consider asbestosis) ▶ Dilated esophagus (consider connective tissue disease [CTD])

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