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Mimics of Idiopathic Pulmonary Fibrosis

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Idiopathic Pulmonary Fibrosis

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Abstract

Making an accurate and confident diagnosis of idiopathic pulmonary fibrosis (IPF) requires a careful evaluation of the patient combined with appropriate thoracic imaging, which in many cases may be sufficient to attain a confident diagnosis. In other cases, however, sampling of lung tissue might be necessary to confirm a usual interstitial pneumonia (UIP) histopathologic pattern if high-resolution computed tomography (HRCT) does not reveal a UIP pattern with adequate confidence. An ultimate diagnosis relies on probability when clinical, radiologic, and histopathologic data are combined in accordance with the currently used clinical practice guidelines for the diagnosis of IPF. This frequently involves using a multidisciplinary discussion, which can be essential to making an ultimate diagnosis. Nonetheless, IPF can be difficult to differentiate from other forms of idiopathic interstitial pneumonia (IIP) such as non-specific interstitial pneumonia (NSIP) and non-IIP forms of interstitial lung disease (ILD). Specifically, connective tissue disorders (CTDs) with lung involvement or chronic hypersensitivity pneumonitis (HP) can have UIP patterns on both imaging and lung tissue biopsy specimens. Additionally, other disease entities may occasionally have UIP or UIP-like patterns on both HRCT imaging and lung tissue histopathology. This chapter discusses conditions that are often confused with and/or difficult to differentiate from IPF.

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Correspondence to Keith C. Meyer .

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Meyer, K.C., Nathan, S.D. (2019). Mimics of Idiopathic Pulmonary Fibrosis. In: Meyer, K., Nathan, S. (eds) Idiopathic Pulmonary Fibrosis. Respiratory Medicine. Humana Press, Cham. https://doi.org/10.1007/978-3-319-99975-3_14

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  • DOI: https://doi.org/10.1007/978-3-319-99975-3_14

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