Interstitial lung diseases (ILDs) represent a heterogeneous group of clinical entities among which disease of unknown causes may mimic ILDs due to known causes [ 1 ]. Clinical, radiographic and histopathology presentation can largely overlap between different entities and a multidisciplinary approach is proven to be essential in composing the puzzle to reach the most likely clinical diagnosis in each single patients [ 2 ]. The defi nition of specifi c radiographic (on chest high-resolution computed tomography, HRCT) and histopathology (on lung surgical lung biopsy, SLB) patterns has provided a common terminology in the fi eld of ILDs in the tentative of classifying entities presenting with distinctive features. These patterns have been proposed in the classifi cation of idiopathic interstitial pneumonias (IIPs) [ 3 ], and then have been applied to describe ILDs due to secondary known causes, particularly those related to connective-tissue diseases. Among all these patterns, the usual interstitial pneumonia (UIP) pattern has received more emphasis, in particular since it identifies patients with idiopathic pulmonary fibrosis (IPF), as outlined in the most recent evidence based international guideline [ 4 ]. In this document, specific HRCT and SLB criteria for the defi nition of a defi nite UIP pattern have been proposed and since then they have been widely used as reference standard both in clinical practice and in the defi nition of eligibility criteria for randomized clinical trials. However, while a defi nite UIP pattern can be diagnostic for IPF in the proper clinical context, it is well known that the same pattern can be present in fibrotic lung diseases other than IPF, with important consequences in terms of therapeutic management and prognosis. This chapter will provide an overview on how the UIP pattern is defined, both on radiologist’s view and on pathologist’s view, along with elements that might be helpful in distinguishing an idiopathic UIP pattern from similar appearance in secondary diseases.
|Data di pubblicazione:||2015|
|Titolo:||Pulmonary fibrosis and the many faces of UIP|
|Autori:||Cerri, Stefania; Sgalla, Giacomo; Rossi, Giulio; Della Casa, Giovanni; Richeldi, Luca|
|Digital Object Identifier (DOI):||10.1007/978-1-4471-2401-6_21|
|Titolo del libro:||Orphan lung diseases. A clinical guide to rare lung disease|
|Tutti i curatori:||Cottin, Vincent; Cordier, Jean-Francois; Richeldi, Luca|
|Appare nelle tipologie:||Capitolo/Saggio|
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