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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease idiopathic pulmonary fibrosis
Comorbidity C1800706|usual interstitial pneumonia
Sentences 24
PubMedID- 20727133 usual interstitial pneumonia (uip) is rare in children [88].
PubMedID- 24886550 Background: the pathological appearance of idiopathic pleuroparenchymal fibroelastosis (ippfe) with hematoxylin-eosin staining is similar to that of usual interstitial pneumonia (uip) in patients with idiopathic pulmonary fibrosis (ipf).
PubMedID- 24205507 usual interstitial pneumonia (uip) and nonspecific interstitial pneumonia (nsip) are the main patterns of ild described in ra although also other forms, including lymphocytic interstitial pneumonia (lip) and organizing pneumonia (op), have been less commonly observed [14, 31, 32].
PubMedID- 26460792 Of numerous types of ild, usual interstitial pneumonia (uip) patterns on chest computed tomography (ct) are reported to be associated with the risk for acute exacerbation of ild in several conditions.
PubMedID- 20502709 A radiographic ct pattern of usual interstitial pneumonia (uip), which is consistent with a diagnosis of ipf, is seen in 74% of cases and a pathologic pattern of uip is seen in 86% of surgical lung biopsies.
PubMedID- 24368713 The diffuse idiopathic interstitial pneumonias describe a spectrum of parenchymal lung diseases sharing clinical, physiological, radiological and pathological similarities, including varying degrees of fibrosis, inflammation and vascular injury.1 idiopathic pulmonary fibrosis (ipf) is associated with usual interstitial pneumonia (uip), poor survival and limited treatment options.2 interstitial lung disease (ild), most typically presenting as non-specific interstitial pneumonitis, is a leading cause of death in systemic sclerosis (ssc)3 and a prominent clinical feature of other connective tissue diseases (ctds), including idiopathic inflammatory myopathy (iim) and sjögren syndrome.
PubMedID- 26528058 usual interstitial pneumonia (uip) is the most common histopathological form of diffuse lung fibrosis occurring in older adults, first described by liebow in 1969 [16].
PubMedID- 24473559 A cox regression analysis showed a high risk of death for the patients with usual interstitial pneumonia and a high percentage of vessel cells immunostained for the angiotensin 2 receptor in the lymphatic vessels.
PubMedID- 22408196 The pattern of usual interstitial pneumonia (uip) (subpleural reticular abnormality and honeycombing, with or without traction bronchiectasis, in the lung basal regions) on chest hrct in the absence of other known causes for uip is typical, thus emphasising the diagnostic role of imaging.
PubMedID- 25922588 usual interstitial pneumonia (uip) is the most common of the idiopathic interstitial pneumonias (iips).1 uip can be idiopathic (idiopathic pulmonary fibrosis, ipf), may be secondary to certain inhalational exposures and medications, or occur in the context of connective tissue diseases (ctds).
PubMedID- 26090479 [12] identified that the subtype of usual interstitial pneumonia/overlap syndromes has around 3.9-fold risk for death in comparison with the subtype of nonspecific interstitial pneumonia/cryptogenic organizing pneumonia.
PubMedID- 24468008 This subject was a 46 year-old man with biopsy-proven usual interstitial pneumonia (uip) who required hospital admission for worsening respiratory status in the setting of a suspected ipf exacerbation.
PubMedID- 20126467 Subjects were also excluded if they had histologic patterns on lung biopsy (or explant) other than usual interstitial pneumonia (uip) or, in one case, end-stage fibrotic lung disease with honeycombing [51].
PubMedID- 23741087 [5] symptomatic pulmonary fibrosis with a usual interstitial pneumonia pattern on high resolution computerized tomography and histopathology was seen in our case 2 years after the influenza infection.
PubMedID- 21265830 The classical histopathological pattern of ipf is one of usual interstitial pneumonia characterized by evidence of patchy epithelial damage including type ii pneumocyte hyperplasia, together with abnormal proliferation of mesenchymal cells, varying degrees of fibrosis and overproduction and disorganized deposition of collagen and ecm – this results in significant distortion of pulmonary architecture and honeycombing (figure 1).
PubMedID- 25477529 Of note, usual interstitial pneumonia, characterised as temporally heterogeneous fibrosis, is not seen in infants.
PubMedID- 24038004 Ra-ild on hrct was classified as a usual interstitial pneumonia (uip) pattern or a non-uip pattern according to recent guideline with slight modification.15 briefly, a uip pattern on hrct was characterised by the following: subpleural and basal predominance; reticular abnormalities; honeycombing with or without traction bronchiectasis and the absence of features listed as inconsistent with a uip pattern; including upper or mid-lung predominance, peribronchovascular predominance, extensive ground-glass abnormalities, profuse micronodules, discrete cysts, diffuse mosaic attenuation/air-trapping and consolidation in bronchopulmonary segment(s)/lobe(s).
PubMedID- 20929558 The histological correlate of ipf is usual interstitial pneumonia (uip), in which areas of interstitial fibrosis of various ages are interspersed with normal lung [1,2].
PubMedID- 26523279 However, the usual interstitial pneumonia (uip) pattern of lung fibrosis, which is the histologic appearance of the fibrotic pattern of ipf, also occurs in a small number of young people.
PubMedID- 22488224 usual interstitial pneumonia (g) showing alternating areas of normal parenchyma (asterisks), honeycombing (triangles) and organizing fibrosis with fibroblastic foci (ff), in detail; anti-d2-40 immunohistochemical stains lymphatics (arrows; h) with a large area or dilated in the peripheral region of honeycombing changes; note fibroblastic foci (ff) overlying the surface of the airspace or enlarged and remodeled airspaces with dense scars in the walls (asterisks).
PubMedID- 24834127 usual interstitial pneumonia (uip), the histological presentation of ipf, is marked by patches of fibrotic tissue in areas of normal appearing lung [1].
PubMedID- 24468083 Every patient had typical features of usual interstitial pneumonia on high-resolution ct. all were treated with corticosteroids and cyclophosphamide, which lead to an improvement in twelve cases.
PubMedID- 23268535 The histological correlate of ipf, usual interstitial pneumonia (uip), is characterized by temporally heterogeneous lung architectural distortion and dense collagen and extracellular matrix (ecm) deposition in the interstitium, alveolar collapse, and the presence of fibroblastic foci.
PubMedID- 22815997 A diagnosis of usual interstitial pneumonia (uip) was confirmed by a lung pathologist in all ipf cases.

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