Typical Usual Interstitial Pneumonia Pattern Research Articles

RADIOLOGICAL EVALUATION OF INTERSTITIAL LUNG DISEASES ON HIGH-RESOLUTION COMPUTED TOMOGRAPHY IN TERTIARY CARE CENTER OF NORTH INDIA

Objective: This study assessed the various high-resolution computed tomography (HRCT) patterns of interstitial lung diseases (ILDs) and to differentially diagnose ILD based on their clinical findings and distribution patterns. Methods: The study was conducted in the Department of Radiodiagnosis, Government Medical College and Rajindra Hospital, Patiala, Punjab, India. Forty-three patients with clinical suspicion of ILD were enrolled in the study. HRCT scans of the chest were done in all the cases taken in the study. Results: The mean age (±he age group of 51–60 years. We found slight female preponderance with females accounting for 53.5% and males accounting for 46.5% of the caseload. The most common presenting complaint was dyspnea on exertion (83.7%) followed by cough in 69.8%. The most frequent HRCT finding was septal thickening (90.7%), followed by tractional bronchiectasis (83.7%), fibrotic changes (72.1%), ground-glass opacities (65.1%), and honeycombing (58.1%). Based on HRCT findings, the most common HRCT pattern was the typical usual interstitial pneumonia pattern (58.1%), followed by the non-specific interstitial pneumonia pattern. The final diagnoses were made based on clinic-radiological findings and by the exclusion of other possibilities. The most common ILD reported was idiopathic pulmonary fibrosis (39.5%), followed by connective tissue disorder-ILD (20.9%), and SR-ILD (13.9%). This is followed by idiopathic non-specific interstitial pneumonia (11.6%), hypersensitivity pneumonitis (6.9%), and cryptogenic organizing pneumonia (2.3%). Conclusion: HRCT is a valuable technique for evaluating various ILDs even when chest X-rays are normal. It can differentially diagnose ILDs based on their clinical findings and distribution patterns.

The Impact of the Envisia Genomic Classifier in the Diagnosis and Management of Patients with Idiopathic Pulmonary Fibrosis.

Rationale: The diagnosis of idiopathic pulmonary fibrosis (IPF) remains challenging and can result in delayed or misdiagnosis. IPF diagnosis is based on the presence of either a radiographic or histologic usual interstitial pneumonia (UIP) pattern in the absence of an identifiable etiology. The Envisia Genomic Classifier is a clinically validated molecular diagnostic test that identifies UIP in transbronchial biopsies. Objectives: To determine the impact of the Envisia Genomic Classifier on physicians' clinical decision-making in the diagnosis and management of IPF. Methods: This prospective randomized decision impact survey was designed to test the hypothesis that including an Envisia UIP-positive result will increase IPF diagnoses, diagnostic confidence, and the recommendation for antifibrotic therapy. The survey included patients from the BRAVE (Bronchial Sample Collection for a Novel Genomic Test) study who had a high-resolution computed tomographic scan without a typical UIP pattern, an Envisia UIP-positive result, and a final diagnosis of IPF by multidisciplinary team discussion. Each case was presented in three different formats: a pre-post cohort, where each case is presented initially without and then with Envisia, and two independent cohorts, where each case is presented without and with Envisia, respectively. Results: U.S.-based pulmonologists from community and academic centers in geographically diverse practices were approached for inclusion in this study. 103 (65%) U.S.-based pulmonologists met the inclusion criteria and provided 605 case reviews of 11 patient cases. The number of IPF diagnoses increased with Envisia by an absolute difference of 39% from 47 (30%) before Envisia to 107 (69%) after Envisia in the pre-post cohort and by 13% in the independent cohorts. High confidence (⩾90%) of interstitial lung disease diagnoses was more commonly seen with Envisia in both the pre-post cohort and in the independent cohorts. Recommendation for antifibrotic treatment increased with Envisia by an absolute difference of 36% from 15 (10%) before Envisia to 72 (46.4%) after Envisia in the pre-post cohort and by 11% in the independent cohorts. Conclusions: This decision impact survey suggests the clinical utility of the Envisia Classifier by demonstrating a significant increase in IPF diagnoses, diagnostic confidence, and recommendation for antifibrotic therapies to assist physicians in effectively managing patients to improve outcomes of patients with IPF.

Use of a Genomic Classifier in Patients with Interstitial Lung Disease: A Systematic Review and Meta-Analysis.

Background: Usual interstitial pneumonia (UIP) is the histopathologic hallmark of idiopathic pulmonary fibrosis (IPF), the prototypical interstitial lung disease (ILD). Diagnosis of IPF requires that a typical UIP pattern be identified by using high-resolution chest computed tomography or lung sampling. A genomic classifier for UIP has been developed to predict histopathologic UIP by using lung samples obtained through bronchoscopy. Objective: To perform a systematic review to evaluate genomic classifier testing in the detection of histopathologic UIP to inform new American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana del Tórax guidelines. Data Sources: Medline, Embase, and the Cochrane Central Register of Controlled Trials were searched through June 2020. Data Extraction: Data were extracted from studies that enrolled patients with ILD and reported the use of genomic classifier testing. Synthesis: Data were aggregated across studies via meta-analysis. The quality of the evidence was appraised by using the Grading of Recommendations, Assessment, Development, and Evaluation approach. Results: Genomic classifier testing had a sensitivity of 68% (95% confidence interval [CI], 55-73%) and a specificity of 92% (95% CI, 81-95%) in predicting the UIP pattern in ILD. Confidence in an IPF diagnosis increased from 43% to 93% in one cohort and from 59% to 89% in another cohort. Agreement levels in categorical IPF and non-IPF diagnoses measured by using a concordance coefficient were 0.75 and 0.64 in the two cohorts. The quality of evidence was moderate for test characteristics and very low for both confidence and agreement. Conclusions: Genomic classifier testing predicts histopathologic UIP in patients with ILD with a specificity of 92% and improves diagnostic confidence; however, sensitivity is only 68%, and testing is not widely available.

Evaluation of Correlations between Genetic Variants and High-Resolution Computed Tomography Patterns in Idiopathic Pulmonary Fibrosis.

Background. Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease (ILD). This prospective observational study aimed at the evaluation of any correlation between genetic variants associated with IPF susceptibility and high-resolution computed tomography (HRCT) patterns. It also aimed at evidencing any differences in the HRTC pattern between the familial and sporadic form at diagnosis and after two years. Methods. A total of 65 IPF patients (mean age at diagnosis 65 ± 10) were enrolled after having given written informed consent. HRCT and genetic evaluations were performed. Results. A total of 19 familial (mean age 62 ± 15) and 46 sporadic (mean age 70 ± 9) IPF patients were enrolled. A statistically significant difference was evidenced in the HRTC pattern at diagnosis between the two groups. Sporadic IPF patients had a predominantly usual interstitial pneumonia (UIP) pattern compared with those patients with familial IPF (60.0% vs. 21.1%, respectively). Moreover, familial IPF patients had more alternative diagnoses than those with sporadic IPF (31.6% vs. 2.2%, respectively). Furthermore, there was a slight increase in the typical UIP pattern in the familial IPF group at two years from diagnosis. Conclusions. Genetic factors play a pivotal role in the risk of developing IPF. However, further studies are required to clarify how these genetic factors may guide clinical treatment decisions.

Comparison of Clinical Measures Among Interstitial Lung Disease (ILD) Patients with Usual Interstitial Pneumonia (UIP) Patterns on High-Resolution Computed Tomography.

Idiopathic Pulmonary Fibrosis is a progressive and fatal interstitial lung disease (ILD) characterized by a typical radiographic or histologic usual interstitial pneumonia (UIP) pattern.In 2018, diagnostic categories of UIP based on computed tomography patterns were revised by the Fleischner Society. The study aimed to describe differences in comorbidities and spirometry in ILD patients that were characterized by high-resolution computed tomography (HRCT) images as having a typical, probable, indeterminate, and alternative diagnosis of UIP. We retrospectively studied 80 ILD patients from 2017 to 2019. Typical UIP was defined using the Fleischner Society diagnostic criteria for IPF. Atypical UIP was reached by consensus after a multidisciplinary clinical-radiological-pathological review of patient data. Baseline characteristics, comorbidities, and spirometry were compared among the four subgroups. Among 80 patients, 59% were male, 61% had a history of smoking, and the mean age was 67.7 ± 10years (SD). A typical UIP pattern was more frequently observed among patients with chronic obstructive pulmonary disease (COPD) (p < 0.001) and pulmonary hypertension (p = 0.03). Of 30 patients with COPD, 14 had emphysema, while 10 had IPF. After adjusting for forced expiratory volume in one second (FEV1) in liters, change of FEV1% from baseline to 6-12months, age, and sex, only COPD remained significantly associated with typical UIP (p = 0.018). Tobacco use was not significantly associated with any radiographic type (p = 0.199). Typical UIP was prevalent among COPD/emphysema patients. Although smoking has a strong association with IPF, we did not find a significant association with smoking and typical UIP in our cohort.

Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg).

(1) Aim of the study: In spite of extensive research, up to 20% of interstitial lung diseases (ILD) patients cannot be safely classified. We analyzed clinical features, progression factors, and outcomes of unclassifiable ILD (uILD). (2) Methods: A total of 140 uILD subjects from the University of Giessen and Marburg Lung Center (UGMLC) were recruited between 11/2009 and 01/2019 into the European Registry for idiopathic pulmonary fibrosis (eurIPFreg) and followed until 01/2020. The diagnosis of uILD was applied only when a conclusive diagnosis could not be reached with certainty. (3) Results: In 46.4% of the patients, the uILD diagnosis was due to conflicting clinical, radiological, and pathological data. By applying the diagnostic criteria of usual interstitial pneumonia (UIP) based on computed tomography (CT), published by the Fleischner Society, 22.2% of the patients displayed a typical UIP pattern. We also showed that forced vital capacity (FVC) at baseline (p = 0.008), annual FVC decline ≥10% (p < 0.0001), smoking (p = 0.033), and a diffusing capacity of the lung for carbon monoxide (DLco) ≤55% of predicted value at baseline (p < 0.0001) were significantly associated with progressive disease. (4) Conclusions: The most important prognostic factors in uILD are baseline level and decline in lung function and smoking. The use of Fleischner diagnostic criteria allows further differentiation and accurate diagnosis.

High resolution computed tomography patterns in interstitial lung disease (ILD): prevalence and prognosis.

ABSTRACTObjectiveTo correlate the prevalence and prognosis of each HRCT pattern of typical, probable, and indeterminate usual interstitial pneumonia (UIP) with the clinical multidisciplinary diagnosis of interstitial lung disease (ILD).MethodsWe included all patients with a multidisciplinary diagnosis of ILD with an HRCT pattern of typical UIP, probable UIP, or indeterminate for UIP. Clinical and histopathological data, pulmonary function tests, and survival status were retrospectively obtained. The final diagnosis was validated by a multidisciplinary team.ResultsA total of 244 patients were included in the study, with a mean age of 68 ±13 years and being 52.5% males. In a total of 106 patients with typical UIP pattern, 62% had the multidisciplinary diagnosis of IPF, 20% had chronic hypersensitivity pneumonitis (CHP), and 10% had connective tissue disease-related ILD (CTD-ILD). Out of the 114 cases with probable UIP, CTD-ILD corresponded to 39%, IPF to 31%, desquamative interstitial pneumonia to 11%, drug-related lung disease to 9%, and CHP to 8%. In the 24 patients with CT indeterminate for UIP, CTD-ILD was the final diagnosis in 33%, followed by desquamative interstitial pneumonia (21%), and IPF (13%). Patients with typical UIP were more likely to die or had lung transplantation in the follow-up (17.9% and 11.3%, respectively).ConclusionIPF, CHP, and CTD-ILD were the main differential diagnoses in patients with HRCT patterns of typical, probable and indeterminate UIP. Patients with HRCT typical UIP pattern were more likely to die or had lung transplantation in the follow-up.

Changes in management of idiopathic pulmonary fibrosis: impact on disease severity and mortality

ABSTRACT Background Idiopathic pulmonary fibrosis (IPF) is a serious interstitial lung disease (ILD) with a median survival of 3-5 years. The aim of the present study was to evaluate disease severity and survival in patients diagnosed with IPF in the era of antifibrotic therapies compared with an earlier IPF cohort. Methods We identified all patients with fibrotic ILD in the hospital electronic case record system between 2011 and 2016, and reviewed each case in order to identify incident patients with IPF. We used the GAP-index to compare disease severity and mortality to previous findings in patients with IPF diagnosed at our center between 2003 and 2009. Results 260 patients were diagnosed with IPF between 2011 and 2016. Mean age was 72.6 years, 79% were male, mean forced vital capacity (FVC) was 80%, and mean diffusing capacity for carbon monoxide (DLco) was 44%. Age, FVC and DLco were significant predictors of mortality, but the presence of a typical usual interstitial pneumonia pattern on HRCT was not. Eighty percent of patients in GAP stage I received antifibrotic therapy, 73% in GAP stage II, and 29% in GAP stage III. The median survival was four years in the 2011-2016 cohort compared with three years in the 2003-2009 cohort. The distribution of patients between GAP stages was unchanged in 2011-2016 compared with 2003-2009, (stage I 34% vs. 32%, stage II 49% vs. 48% and stage III 20% vs. 16%). One-year mortality was 13% in 2011-2016 and 26% in 2003-2009. In severe disease (GAP stage III), one-year mortality was 26% and 54%, respectively, (p=0.019). Conclusion Short-term mortality was significantly lower in the 2011-2016 cohort compared with 2003-2009. This improvement may be linked to changes in treatment strategies towards limited use of corticosteroids. Although early diagnosis of IPF still needs increased focus, the improvement is encouraging.

Radiologic and pathologic characteristics of myeloperoxidase-antineutrophil cytoplasmic antibody-associated interstitial lung disease: a retrospective analysis.

The association between interstitial lung disease (ILD) and myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCAs) has been described, but pathologic characteristics are not well characterized. We assessed the radiologic and pathologic characteristics of ILD in MPO-ANCA-positive patients and the association between ILD and vasculitis, particularly microscopic polyangiitis (MPA). We retrospectively searched electronic health records to identify MPO-ANCA-positive patients with ILD who underwent surgical lung biopsy at our institution from January 1997 through August 2017. Demographic, clinical, imaging, and pathologic characteristics were analyzed. We identified 18 MPO-ANCA-positive patients with ILD. The median (range) age was 58 (43-75) years, and the cohort included 10 men (56%), 10 former smokers (56%), and 11 patients (61%) had clinical evidence of systemic vasculitis (MPA) at the time of diagnosis of ILD. On high-resolution computed tomography, the most common radiologic pattern was "inconsistent with usual interstitial pneumonia" (UIP) (n=14 [78%]); the other 4 patients (22%) fulfilled the radiologic criteria for the UIP pattern. Honeycombing was seen in 15 patients (83%). Ten patients (56%) had the UIP pattern on biopsy: 4 of these patients had additional inflammatory changes that were not typical of UIP (as seen in patients with idiopathic pulmonary fibrosis), and the other 6 patients had other inflammatory patterns or findings. The presence or absence of MPA did not correlate with pathologic findings. MPO-ANCA-positive patients with ILD do not show the typical UIP pattern as seen in patients with idiopathic pulmonary fibrosis on surgical lung biopsy.

Tissue Continues to Be the Issue: Role of Histopathology in the Context of Recent Updates in the Radiologic Classification of Interstitial Lung Diseases

High-resolution computed tomography (HRCT) imaging has an increasingly important role in clinical decision-making in patients with interstitial lung diseases. The recent Fleischner Society white paper on the diagnostic criteria for idiopathic pulmonary fibrosis highlights the advances in our understanding of HRCT imaging in interstitial lung diseases. To discuss the evidence and recommendations outlined in the white paper as it pertains to the radiologic diagnosis of interstitial lung disease, specifically highlighting the current limitations of HRCT in confidently predicting histopathologic findings. The recent Fleischner Society white paper and other studies pertaining to the role of HRCT in predicting histopathology in interstitial lung diseases are reviewed. High-resolution computed tomography is highly predictive of a usual interstitial pneumonia (UIP) pattern on histopathology when the HRCT shows a typical UIP pattern on a "confident" read by the radiologist. A probable UIP pattern is also very predictive of a UIP pattern on histopathology, and histopathologic confirmation is not needed for most patients demonstrating this pattern in the appropriate clinical setting. A UIP pattern may be seen in a substantial proportion of patients with an "indeterminate UIP" pattern on HRCT and in many patients for whom the HRCT suggests an alternative diagnosis; histopathologic confirmation should be considered in patients demonstrating these patterns whenever feasible.

Air trapping in usual interstitial pneumonia pattern at CT: prevalence and prognosis

This study was conducted to evaluate the presence of air trapping in patients with idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILDs) (non-IPF), showing the radiological pattern of usual interstitial pneumonia (UIP). Retrospectively, we included 69 consecutive patients showing the typical UIP pattern on computed tomography (CT), and 15 final diagnosis of IPF with CT pattern “inconsistent with UIP” due to extensive air trapping. Air trapping at CT was assessed qualitatively by visual analysis and quantitatively by automated-software. In the quantitative analysis, significant air trapping was defined as >6% of voxels with attenuation between −950 to −856 HU on expiratory CT (expiratory air trapping index [ATIexp]) or an expiratory to inspiratory (E/I) ratio of mean lung density >0.87. The sample comprised 51 (60.7%) cases of IPF and 33 (39.3%) cases of non-IPF ILD. Most patients did not have air trapping (E/I ratio ≤0.87, n = 53, [63.1%]; ATIexp ≤6%, n = 45, [53.6%]). Air trapping in the upper lobes was the only variable distinguishing IPF from non-IPF ILD (prevalence, 3.9% vs 33.3%, p < 0.001). In conclusion, air trapping is common in patients with ILDs showing a UIP pattern on CT, as determined by qualitative and quantitative evaluation, and should not be considered to be inconsistent with UIP. On subjective visual assessment, air trapping in the upper lobes was associated with a non-IPF diagnoses.

Usual interstitial pneumonia: typical, possible, and "inconsistent" patterns.

ABSTRACTIdiopathic pulmonary fibrosis is a severe and progressive chronic fibrosing interstitial lung disease, a definitive diagnosis being established by specific combinations of clinical, radiological, and pathological findings. According to current international guidelines, HRCT plays a key role in establishing a diagnosis of usual interstitial pneumonia (UIP). Current guidelines describe three UIP patterns based on HRCT findings: a typical UIP pattern; a pattern designated “possible UIP”; and a pattern designated “inconsistent with UIP”, each pattern having important diagnostic implications. A typical UIP pattern on HRCT is highly accurate for the presence of histopathological UIP, being currently considered to be diagnostic of UIP. The remaining patterns require further diagnostic investigation. Other known causes of a UIP pattern include drug-induced interstitial lung disease, chronic hypersensitivity pneumonitis, occupational diseases (e.g., asbestosis), and connective tissue diseases, all of which should be included in the clinical differential diagnosis. Given the importance of CT studies in establishing a diagnosis and the possibility of interobserver variability, the objective of this pictorial essay was to illustrate all three UIP patterns on HRCT.

Bronchoscopic Lung Cryobiopsy Increases Diagnostic Confidence in the Multidisciplinary Diagnosis of Idiopathic Pulmonary Fibrosis

Surgical lung biopsy is often required for a confident multidisciplinary diagnosis of idiopathic pulmonary fibrosis (IPF). Alternative, less-invasive biopsy methods, such as bronchoscopic lung cryobiopsy (BLC), are highly desirable. To address the impact of BLC on diagnostic confidence in the multidisciplinary diagnosis of IPF. In this cross-sectional study we selected 117 patients with fibrotic interstitial lung disease without a typical usual interstitial pneumonia pattern on high-resolution computed tomography. All cases underwent lung biopsies: 58 were BLC, and 59 were surgical lung biopsy (SLB). Two clinicians, two radiologists, and two pathologists sequentially reviewed clinical-radiologic findings and biopsy results, recording at each step in the process their diagnostic impressions and confidence levels. We observed a major increase in diagnostic confidence after the addition of BLC, similar to SLB (from 29 to 63%, P = 0.0003 and from 30 to 65%, P = 0.0016 of high confidence IPF diagnosis, in the BLC group and SLB group, respectively). The overall interobserver agreement in IPF diagnosis was similar for both approaches (BLC overall kappa, 0.96; SLB overall kappa, 0.93). IPF was the most frequent diagnosis (50 and 39% in the BLC and SLB group, respectively; P = 0.23). After the addition of histopathologic information, 17% of cases in the BLC group and 19% of cases in the SLB group, mostly idiopathic nonspecific interstitial pneumonia and hypersensitivity pneumonitis, were reclassified as IPF. BLC is a new biopsy method that has a meaningful impact on diagnostic confidence in the multidisciplinary diagnosis of interstitial lung disease and may prove useful in the diagnosis of IPF. This study provides a robust rationale for future studies investigating the diagnostic accuracy of BLC compared with SLB.