Usual interstitial pneumonia, abbreviated UIP, is a relatively common pattern in diffuse lung diseases. Overall, it is an uncommon pathology. Idiopathic pulmonary fibrosis (abbreviated IPF ) redirects here Terminology. UIP is the name of morphological pattern in histology and radiology, and also seen in other etiologies such as connective tissue diseases and hypersensitivity pneumonia. IPF was previously called chronic interstitial pneumonitis or cryptogenic fibrosing alveolitis
The histology of UIP pattern. The key histology features of the UIP pattern are: 1) spatial heterogeneity due to patchy parenchymal involvement; 2) architectural distortion; and 3) temporal heterogeneity [62, 63] IPF may exhibit a range of UIP patterns on histopathologic samples1; UIP pattern can indicate other pathologies in addition to IPF3; The observed histopathologic pattern should be considered together with the HRCT features, medical history, and clinical observations in the context of an MDD to produce a diagnosis1,3 16. 1
. The histological features of UIP are beautifully described in recent papers.34, 35, 36 The diagnostic keys are: 1) a patchwork appearance resulting from alternating areas of scarred and normal lung; 2) architectural distortion; and 3) fibroblastic foci Idiopathic pulmonary fibrosis (IPF) is a fibrotic disease with the histology of usual interstitial pneumonia (UIP). Although the pathologist's visual inspection is central in histologic assessments, three-dimensional microcomputed tomography (microCT) assessment may complement the pathologist's scoring The MA-UIP cohort included myositis or anti-synthetase syndrome patients with interstitial lung disease while manifesting UIP on high-resolution CT chest and/or a lung biopsy revealing UIP histology. IPF subjects met American Thoracic Society criteria and similarly had UIP histopathology
Histopathology Features of the UIP Pattern Histopathology Patterns Diagnostic Criteria for IPF Diagnostic Interventions Question 1: Should Patients with Newly Detected ILD of Unknown Cause Who Are Clinically Suspected of Having IPF Undergo a Detailed, Prompted History of Medication Use an prevalence of histological UIP, the positive predictive value (PPV) was 84% and the negative predictive value (NPV) was 77%. When the analysis was repeated on the 42 patients for which SLB was recommended by guidelines, i.e. excluding those with a definite HRCT UIP pattern, a PPV of 80% for UIP was found. When comparing the outcomes of two separat Histologic Fleischner Society White Paper  Pattern Clinical-Radiologic-Pathologic-Diagnosis (Histopathology) UIP UIP UIP IPF UIP UIP DIP DIP DIP DIP Probable UIP Probable UIP RB-ILD RB RB-ILD Possible UIP Possible UIP LIP LIP LIP Not UIP Alternative diagnosis GIP (HRCT) (HRCT) BIP BOOP OP COP UIP Typical UIP AIP DAD AIP Possible UIP Probable UIP This Review provides an updated approach to the diagnosis of idiopathic pulmonary fibrosis (IPF), based on a systematic search of the medical literature and the expert opinion of members of the Fleischner Society. A checklist is provided for the clinical evaluation of patients with suspected usual interstitial pneumonia (UIP). The role of CT is expanded to permit diagnosis of IPF without.
Omote et al. studied 23 patients with histologic UIP pattern and CTD features and reported no significant deterioration in lung function during 12 months (baseline FVC, 80.6%; 1-year FVC, 78.4%; p. CT imaging shows a pattern of typical or probable UIP (usual interstitial pneumonia) • If the clinical context is indeterminate for IPF, or the CT pattern is not indicative of typical or probable UIP, biopsy should be considered to confirm the presence of a UIP histological pattern, and a confident diagnosis of IPF coul Generally less severe than UIP; Overall 80=90% 5 year survival Responds to steroids; High resolution computed tomography (HRCT) Predominantly ground glass opacities ; Changes bilateral, predominantly basal and peripheral; Reticular, linear patterns may also be seen in 50%; Focal honeycombing only seen in occasional cases; Histopathologic feature
Prognostic evaluation in idiopathic pulmonary fibrosis (IPF) may be important as it can guide management decisions, but the potential role of honeycomb changes in providing information about outcome and survival of patients with IPF, particularly if diagnosed using cryobiopsy, has not been evaluated. Aim of this study was to determinate whether a relationship exists between honeycombing on. The current usual interstitial pneumonitis (UIP)/idiopathic pulmonary fibrosis CT scan classification system excludes probable UIP as a diagnostic category. We sought to determine the predictive effect of probable UIP on CT scan on histology and the effect of the promoter polymorphism in MUC5B (rs35705950) on histologic and CT scan UIP diagnosis Background Histology is a key element for the multidisciplinary diagnosis of fibrotic diffuse parenchymal lung diseases (f-DPLD) when the clinical-radiological picture is nondiagnostic. Transbronchial lung cryobiopsy (TBLC) have been shown to be useful for obtaining large and well-preserved biopsies of lung parenchyma, but experience with TBLC in f-DPLD is limited 3- Histopathology UIP patterns . Both guidelines recommend categorizing histopathologic findings of biopsies into four categories: UIP, probable UIP, indeterminate for UIP, and alternative diagnosis 1 / features most consistent with an alternative diagnosis 2 terstitialpneumonia(UIP).Prone axial CT through the lung bases in a patient with histologically proven UIP shows predominantly peribronchovascular ground-glass/ reticular opacities with traction bronchiectasis. Under the ATS/ ERS/JRS/ALATStatement(8),this would be classiﬁed as inconsistent with UIP. Histologic features: ( B.
The histologic distinction between UIP and NSIP (especially fibrotic NSIP) carries significant inter and intraobserver variability among pathologists. (low interobserver kappa of 0.57) Relatively low agreement between the two pathologists The histologic finding of usual interstitial pneumonia, a term coined by the Yale pathologists Averill Liebow and Charles Carrington, has long been considered the histologic sine qua non of idiopathic pulmonary fibrosis (IPF). In a previous blog post, I noted that their 1969 histologic description of that pattern actually seems to describe the organizing phas The role of histology in idiopathic pulmonary fibrosis: An update. Respiratory Medicine, 2010. Massimiliano Paci. Cristiano Carbonelli. Alberto Cavazza. Giulio Rossi. Massimiliano Paci. Cristiano Carbonelli
The characteristic histological pattern of IPF is UIP. Interestingly, a UIP pattern is associated with a significantly better survival in CTD-related disease compared to the idiopathic variety. Prognosis in IPF is dismal, with a median survival since diagnosis of 2 3 years. No treatment regimen has been shown to improve survival in IPF UIP in RA shares a number of radiological and histopathological features with IPF [90, 91, 92]. An additional histological feature of UIP in RA is frequent germinal center formation . RA-related UIP also begins within basal, subpleural peripheral areas as does IPF. Recently, the MUC5B promoter variant was reported in RA-related UIP
For example, when lower zone-, posterior-, subpleural-predominant reticular opacities with traction bronchiectasis and honeycombing are identified on HRCT in the absence of significant ground glass opacities, the certainty of finding a histologic UIP pattern by SLBx exceeds 96%. 10 In fact, when those features are present, the HRCT is considered to display a radiologic UIP pattern The histologic characteristics of air space enlargement with fibrosis (AEF) are compared with usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP) and centrilobular emphysema (CLE) to determine similarities and differences Histologic UIP may be associated with atypical HRCT appearances, and in such cases in which surgical biopsy shows UIP, multi-disciplinary discussion should be performed in an effort to ascertain a final diagnosis [1, 4, 5] Histologic differential diagnoses of UIP include other patterns of interstitial pneumonias, including fibrosing nonspecific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP. Radiological versus histological diagnosis in UIP and NSIP: survival implications. Thorax 2003;58(2):143-148. Crossref, Medline, Google Scholar; 20. Aaløkken TM, Naalsund A, Mynarek G et al. Diagnostic accuracy of computed tomography and histopathology in the diagnosis of usual interstitial pneumonia. Acta Radiol 2012;53(3):296-302
Though HRCT and histopathology are concordant for honeycombing in the majority of cases, there is a minority of patients (approximately 30%) in whom HRCT and histopathology are discordant. A HRCT pattern highly suggestive of UIP was almost always associated with a UIP pattern on histopathology Which histologic findings are characteristic of usual interstitial pneumonia (UIP)? Updated: Jan 02, 2020 Author: Eunhee (Joanne) S Yi, MD; Chief Editor: Philip T Cagle, MD more.. We developed a classifier using RNA sequencing data that identifies the usual interstitial pneumonia (UIP) pattern for the diagnosis of idiopathic pulmonary fibrosis. We addressed significant challenges, including limited sample size, biological and technical sample heterogeneity, and reagent and assay batch effects. We identified inter- and intra-patient heterogeneity, particularly within the.
a non-UIP pattern.1 This new group would draw in patients from what would previously have been classified as possible UIP or inconsistent with UIP. We have concerns regarding the language used to describe the indeterminate for UIP category; Lynch and colleagues suggest that inconspicuous features suggestive of a non-UIP pattern would warrant Despite their similarities, the UIP pattern of CTD and the UIP pattern of IPF show several histologic differences (Figure 2, C through H) that can enable their distinction. 10-12,24 At low magnification, RA-associated ILD often shows a mixed pattern of fibrosis that is difficult to classify as either UIP or NSIP, with both patterns represented in the biopsy Other studies show that up to 60% of patients demonstrating typical UIP histopathology had CT imaging findings that were not typical for UIP. 1,10,11 Areas of decreased attenuation or mosaic attenuation, both suggesting an alternative diagnosis such as hypersensitivity pneumonitis, can be relatively common (43% in one study 12 and 27% in another 9 ) in patients with biopsy-proven UIP. 1 In the. A usual interstitial pneumonia (UIP) pattern on chest CT scans is highly suggestive of UIP pathologic findings; the most common cause of UIP is idiopathic pulmonary fibrosis (IPF) [1-5].Under current guidelines, a UIP pattern on CT images is specific for IPF after a thorough clinical and serologic workup has excluded other causes of interstitial lung disease (ILD) 
So if Leibow and Carrington didn't actually describe the histology of UIP/IPF, who did? December 21, 2018 roberthomer The histologic finding of usual interstitial pneumonia, a term coined by the Yale pathologists Averill Liebow and Charles Carrington, has long been considered the histologic sine qua non of idiopathic pulmonary fibrosis (IPF) Background/Aims —The pathogenesis of idiopathic pulmonary fibrosis (IPF)/usual interstitial pneumonia (UIP), a chronic and incurable human respiratory disease, is not well established. This study was designed to investigate whether the apoptosis of type II pneumocytes could be the precipitating factor in the pathogenesis of IPF. Methods —Nineteen specimens obtained by retrospective review.
No major complications were seen. Mean specimen diameter in the histological biopsies was 6.9 ± 4.4 mm (Range 2 - 22 mm). A correlation between clinical and histopathological diagnoses was found in 79% of cases (41/52). In the case of UIP (usual interstitial pneumonia) pattern, the concordance was 10/15 (66%) Smoking-related interstitial fibrosis should not show architectural distortion due to scarring or lung remodeling in the form of microscopic honeycomb change, both of which are histologic features of UIP. 8 Although rare fibroblastic foci can occasionally be identified in SRIF, their presence should raise concern for UIP and prompt careful review of any available clinical and radiologic data. Patients with histological UIP with no HRCT diagnosis of probable or definite UIP fared better than patients with histological UIP and an HRCT diagnosis of definite or probable UIP (HR 0.49, 95% CI 0.25 to 0.98; median survival 5.76 v 2.08 years) and worse than those with a histological diagnosis of NSIP (HR 5.42, 95% CI 1.25 to 23.5; median. Idiopathic interstitial pneumonia (IIP), or noninfectious pneumonia are a class of diffuse lung diseases.These diseases typically affect the pulmonary interstitium, although some also have a component affecting the airways (for instance, cryptogenic organizing pneumonitis).There are seven recognized distinct subtypes of IIP
The presence of a UIP pattern on histology is strictly correlated with peculiar features on CT scan, namely radiological UIP pattern . Therefore, intensivists should be able to promptly recognize the UIP pattern at the CT scan, as it is the main determinant of the squishy-ball behavior of the fibrotic lung subject to MV The histological lung injury in RA-ILD can be any of the interstitial pneumonia patterns, including overlaps. Most studies report UIP and NSIP as being the most common patterns, and coexisting UIP and NSIP are common in RA-ILD The typical histological finding in AE-IPF is diffuse alveolar damage superimposed on the underlying UIP pattern. 54 The incidence of AE-IPF is still unclear, varying from 5% to 15% per year in retrospective studies on the placebo arm populations enrolled in clinical trials 15, 55, 56 and might increase with time, reaching 20.7% at 3 years from diagnosis. 52 Lower FVC and DL CO have been shown.
Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown cause, primarily occurring in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP).  It causes lung scarring, which, over time, results in reduced oxygen intake Noteworthy, the histological UIP pattern observed in IPAF patients has been reported as non-typical, with diffuse lymphoplasmacytic infiltration, interstitial lymphoid aggregates, or histological involvement of the airways (10, 18) classical (or deﬁnite) UIP pattern on the HRCT scan has a very high positive predictive value (between 90% and 100%) for a histological diagnosis of UIP,12 and has been therefore considered sufﬁcient for a diagnosis of IPF to be made. If honeycombing on HRCT is absent, then the diagnosis of IPF is regarde
ABSTRACT Background and objective Lobar and temporal histological variability in chronic bird‐related hypersensitivity pneumonitis (BRHP) has not been clearly elucidated. This study was designed to.. Low-power histology demonstrating subpleural and interstitial fibrosis with temporal heterogeneity seen with a usual interstitial pneumonia (UIP) pattern. View Media Gallery Top image: Typical histologic finding of spatial heterogeneity in usual interstitial pneumonia (UIP) with an abrupt transition from honeycomb change (left) to normal lung (right) (trichrome stain, x40) histologic features for LD-CTD were observed in 15 patients with histologic UIP (h-UIP) and 11 patients with histologic NSIP (h-NSIP). Fift een patients with h-UIP (60%) showed an inconsistent UIP pattern on HRCT scan. Aft er multidisciplinary discussion (MDD), 18 patients with h-UIP were labeled as having unclassifi able IIP Histologic Features. The histologic hallmark of UIP is the presence of scattered ﬁbroblastic foci (Fig 2). Typically, the lung involvement is heterogeneous and areas of normal lung alternate with interstitial inﬂamma-tion and honeycombing (1). Owing to the patchy lung involvement, histologic evaluation of mul
UIP, indeterminate, probable NSIP, or definite NSIP. Cox regression analysis examined the relationships between histopathological and radiological diagnoses and mortality, controlling for patient age, sex, and smoking status. Results: All 27 patients with definite or probable UIP on HRCT had histological UIP; 18 of 44 patient Introduction. Pleuroparenchymal fibroelastosis (PPFE) is characterized by upper lobe predominant pulmonary fibrosis affecting the visceral pleura and the subpleural lung parenchyma .The term PPFE was introduced by Frankel and colleagues in 2004 to describe an apparently rare upper lobe predominant pulmonary fibrotic disorder .In 2013 PPFE was inserted in the American Thoracic Society. include varying histologic criteria for making this diagnosis and substantial overlap of histologic and radiologic findings with those of other condi-tions, including UIP, COP, hypersensitivity pneu-monitis, and DIP. The goal of this article is to review the com-mon clinical, pathologic, and radiologic findings seen in NSIP Histological characteristics of pulmonary involvement in RDs: 1) lymphoid hyperplasia, which is visible at low magnification, and inflammation (more represented in the UIP pattern of RDs than the UIP pattern of IPF) , 2) honeycombing, emphysema, fibroblastic foci (less represented in the UIP pattern of RDs than the UIP pattern of IPF) [4,24], 3) pleural fibrosis Possible UIP is considered if there is patchy or diffuse involvement of lung parenchyma with or without interstitial inflammation, if there are no findings of high-confidence UIP on histopathology, and if there are no findings suggestive of an alternative diagnosis