Cases Of Usual Interstitial Pneumonia Research Articles

Fine-tuning characterization of patients with interstitial pneumonia and an underlying autoimmune disease in real-world practice: We get closer with Nailfold videocapillaroscopy.

To assess performance of interstitial pneumonia (IP) with autoimmune features (IPAF) criteria in clinical practice and describe the utility of additional workup in identifying patients with underlying connective tissue diseases (CTD). We set a retrospective study of our patients with autoimmune IP, who were allocated to CTD-IP, IPAF or undifferentiated autoimmune IP (uAIP) subgroups according to the updated classification criteria. Presence of the process-related variables comprising IPAF defining domains was scrutinized in all patients, and, when available, the results of nailfold videocapillaroscopy (NVC) were recorded. Thirty nine out of 118 patients, accounting for 71% of former undifferentiated cases, fulfilled IPAF criteria. Arthritis and Raynaud's phenomenon were prevalent in this subgroup. While systemic sclerosis-specific autoantibodies were restricted to CTD-IP patients, anti-tRNA synthetase antibodies were also present in IPAF. In contrast, rheumatoid factor, anti-Ro antibodies and ANA nucleolar patterns could be found in all subgroups. Usual interstitial pneumonia (UIP) / possible UIP were the most frequently observed radiographic patterns Therefore, the presence of thoracic multicompartimental findings as also performance of open lung biopsies were useful in characterizing as IPAF those UIP cases lacking a clinical domain. Interestingly, we could observe NVC abnormalities in 54% of IPAF and 36% of uAIP tested patients, even though many of them did not report Raynaud's phenomenon. Besides application of IPAF criteria, distribution of IPAF defining variables along with NVC exams help identify more homogeneous phenotypic subgroups of autoimmune IP of potential relevance beyond clinical diagnosis.

Exploring the Value of Features of Lung Texture in Distinguishing Between Usual and Nonspecific Interstitial Pneumonia

This article aims to explore the potential use of lung texture assessed in CT images in distinguishing between the usual interstitial pneumonia and the nonspecific interstitial pneumonia. A retrospective analysis of 96 cases of interstitial pneumonia was performed. Among these cases, there were 40 cases of usual interstitial pneumonia (UIP) and 56 cases of the nonspecific interstitial pneumonia (NSIP) . All of the patients underwent computed tomography (CT) scans. A lung intelligence kit (LK) was utilized to perform lung segmentation and texture feature extraction. The significant variables were determined by variance analysis, least absolute shrinkage and selection operator (LASSO) and multivariate logistic regression. Finally, a multivariate logistic regression model was established to distinguish between the two types of interstitial pneumonia. Receiver operating characteristic (ROC) curves, area under the curve (AUC) values, sensitivity, and specificity were used to evaluate the performance of the established model. A total of 100 texture features were extracted from the whole lung that was segmented by LK, and 8 features remained after feature reduction. The AUC, sensitivity, and specificity of the multivariate logistic regression model in the training group and the test group were 0.952 and 0.838, 0.821 and 0.667, and 0.949 and 0.824, respectively. It is possible to distinguish between UIP and NSIP using lung texture features obtained from CT images.

Diagnostic value and safety of medical thoracoscopy under local anesthesia for unexplained diffuse interstitial lung disease: A retrospective study.

ObjectiveWe aimed to explore the safety and diagnostic value of medical thoracoscopic lung biopsy in patients with unexplained diffuse interstitial lung disease (ILD) in a single center pilot study.MethodWe retrospectively analyzed clinical and pathological diagnostic data from 52 patients with diffuse ILD undergoing medical thoracoscopic lung biopsy.ResultsForty-four cases of diffuse ILD were confirmed pathologically, giving a diagnostic rate of 84.6%. Among these 44 patients, 11 patients were diagnosed with cancer, including eight patients with lung adenocarcinoma, three patients with metastases; two from a gastrointestinal malignancy, and one from a granulosa cell tumor of the ovary. There were 17 cases of idiopathic interstitial pneumonia, including nine cases of usual interstitial pneumonia (UIP), four cases of non-specific interstitial pneumonia (NSIP), three cases of cryptogenic organizing pneumonia (COP), and one case of acute interstitial pneumonia (AIP). There were 12 cases of rare interstitial pneumonias, which included six cases of pulmonary alveolar proteinosis, one case each of pulmonary Langerhans cell histiocytosis (LCH) and pulmonary lymphangiomyomatosis, two cases of nodular sarcoidosis, and two cases of chronic eosinophilic pneumonia. We recorded various complications, including bleeding, infection, and pneumothorax. A total of 28 patients (53.8%) experienced at least one of the above complications, but there were no deaths associated with biopsy.ConclusionsMedical thoracoscopic lung biopsy appears a safe and effective method for diagnosing diffuse ILD of unknown cause but further prospective studies, with larger numbers, including comparison with other established techniques are required.

Content-based Image Retrieval by Using Deep Learning for Interstitial Lung Disease Diagnosis with Chest CT.

Background Evaluation of interstitial lung disease (ILD) at CT is a challenging task that requires experience and is subject to substantial interreader variability. Purpose To investigate whether a proposed content-based image retrieval (CBIR) of similar chest CT images by using deep learning can aid in the diagnosis of ILD by readers with different levels of experience. Materials and Methods This retrospective study included patients with confirmed ILD after multidisciplinary discussion and available CT images identified between January 2000 and December 2015. Database was composed of four disease classes: usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia, and chronic hypersensitivity pneumonitis. Eighty patients were selected as queries from the database. The proposed CBIR retrieved the top three similar CT images with diagnosis from the database by comparing the extent and distribution of different regional disease patterns quantified by a deep learning algorithm. Eight readers with varying experience interpreted the query CT images and provided their most probable diagnosis in two reading sessions 2 weeks apart, before and after applying CBIR. Diagnostic accuracy was analyzed by using McNemar test and generalized estimating equation, and interreader agreement was analyzed by using Fleiss κ. Results A total of 288 patients were included (mean age, 58 years ± 11 [standard deviation]; 145 women). After applying CBIR, the overall diagnostic accuracy improved in all readers (before CBIR, 46.1% [95% CI: 37.1, 55.3]; after CBIR, 60.9% [95% CI: 51.8, 69.3]; P < .001). In terms of disease category, the diagnostic accuracy improved after applying CBIR in UIP (before vs after CBIR, 52.4% vs 72.8%, respectively; P < .001) and NSIP cases (before vs after CBIR, 42.9% vs 61.6%, respectively; P < .001). Interreader agreement improved after CBIR (before vs after CBIR Fleiss κ, 0.32 vs 0.47, respectively; P = .005). Conclusion The proposed content-based image retrieval system for chest CT images with deep learning improved the diagnostic accuracy of interstitial lung disease and interreader agreement in readers with different levels of experience. © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Wielpütz in this issue.

Vessel-related structures predict UIP pathology in those with a non-IPF pattern on CT.

To determine if a quantitative imaging variable (vessel-related structures [VRS]) could identify subjects with a non-IPF diagnosis CT pattern who were highly likely to have UIP histologically. Subjects with a multidisciplinary diagnosis of interstitial lung disease including surgical lung biopsy and chest CT within 1 year of each other were included in the study. Non-contrast CT scans were analyzed using the Computer-Aided Lung Informatics for Pathology Evaluation and Rating (CALIPER) program, which quantifies the amount of various abnormal CT patterns on chest CT. Quantitative data were analyzed relative to pathological diagnosis as well as the qualitative CT pattern. CALIPER-derived volumes of reticulation (p = 0.012), honeycombing (p = 0.017), and VRS (p < 0.001) were associated with a UIP pattern on pathology on univariate analysis but only VRS was associated with a UIP pathology on multivariable analysis (p = 0.013). Using a VRS cut-off of 173 cm3, the sensitivity and specificity for pathological UIP were similar to those for standard qualitative CT assessment (55.9% and 80.4% compared to 60.6% and 80.4%, respectively). VRS differentiated pathological UIP cases in those with a non-IPF diagnosis CT category (p < 0.001) but not in other qualitative CT patterns (typical UIP, probable UIP, and indeterminate for UIP). The rate of pathological UIP in those with VRS greater than 173 cm3 (84.2%) was nearly identical to those who had a qualitative CT pattern of probable UIP (88.9%). VRS may be an adjunct to CT in predicting pathology in patients with interstitial lung disease. • Volume of vessel-related structures (VRS) was associated with usual interstitial pneumonia (UIP) on pathology. • This differentiation arose from those with CT scans with a non-IPF diagnosis imaging pattern. • Higher VRS has similar diagnostic ramifications for UIP as probable UIP, transitively suggesting in patients with high VRS, pathology may be obviated.

Concordance of Bronchoalveolar Lavage Cytology with Transbronchial Lung Biopsies in Non-neoplastic Pulmonary Diseases

Introduction: In many pulmonary diseases, despite radiological &amp; clinical investigations, laboratory tests and function studies, the diagnosis becomes difficult. Bronchoalveolar Lavage (BAL) is a minimally invasive method in which cells are collected from bronchial and alveolar spaces for cytology. This is facilitated by using a flexible bronchoscope with which a biopsy is taken following BAL. Bronchoscopy with BAL when used appropriately can offer correct diagnosis which in turn aids in proper management of the patient. Aim: To find the concordance of BAL findings with the histopathological features of Transbronchial Lung Biopsy (TBLB) in non-neoplastic lung diseases. Materials and Methods: It was a retrospective study conducted in Kempegowda Institute of Medical Sciences, Bengaluru, Karnataka. A total of 40 patients presenting with clinico-radiological findings, suggesting a non-neoplastic lung disease in the year 2019, undergoing bronchoscopy with BAL and concurrent TBLB were chosen. The BAL fluid was processed and differential count of cells was done to classify according to the American Thoracic Society Guidelines. Concordance was checked between the diagnoses made on TBLB and BAL analysis. Results: In the present study, a total of 40 cases were included of which 13 (32.5%) cases showed neutrophilic, 16 (40%) cases showed lymphocytic, 5 (12.5%) cases showed eosinophilic and 6 (15%) cases showed normal cellular distribution on BAL cytology. Diagnoses on studying TBLB included nine cases of Nonspecific Interstitial Pneumonia, seven cases of Usual Interstitial Pneumonia, six cases of Bronchiolitis Obliterans Organising Pneumonia, three cases of Bronchiolitis, two cases each of pulmonary tuberculosis and granulomatous inflammation. There was one case each of actinomycosis, sarcoidosis, lung abscess and mucor mycosis. Normal histology was noted in seven cases. The sensitivity of BAL fluid analysis was found to be 84.84% and the concordance was 80%. The Kappa value obtained was 0.71 indicating good agreement/concordance between BAL cytology and TBLB. Conclusion: The data from the current study suggest that differential cell counts in BAL provide diagnostic information of fundamental importance in frequently occurring non-neoplastic lung diseases in the community.

Senescence and autophagy in usual interstitial pneumonia of different etiology

Idiopathic pulmonary fibrosis (IPF) is a disease with a dismal prognosis. Currently, the causing agent(s) are poorly understood. Recent data suggest that senescence and autophagy might play a role in its development, as well as changes in metabolism due to hypoxic conditions. In this study, the expression of senescence markers in 23 cases of usual interstitial pneumonia (UIP)/IPF and UIP/chronic autoimmune diseases (UIP/AuD) was investigated. The status of autophagy was evaluated with respect to either antiinflammatory or antihypoxia function. Formalin-fixed paraffin-embedded tissues of UIP were selected for immunohistochemistry with antibodies for p21, p16, and β-galactosidase (senescence); for LC3, SIRT1, MAP1S, and pAMKα (autophagy); and for LDH and GLUT1 (metabolism). Epithelial cells in cystic remodeled areas of UIP stained for p16 and p21, p16 being more specific compared with p21. Myofibroblasts were negative in all cases. An upregulation of all four autophagy markers was seen not only in epithelia within remodeled areas and proliferating myofibroblasts, but also in bronchial epithelia and pneumocytes. Upregulated autophagy points to a compensatory mechanism for hypoxia; therefore, LDH and GLUT1 were investigated. Their expression was present in epithelia within cystic remodeling and in myofibroblasts. The cells within the remodeled areas stained for cytokeratin 5, but coexpressed TTF1, confirming their origin from basal cells of bronchioles. Within this population, senescent cells arise. Our results indicated that autophagy in UIP very likely helps cells to survive in hypoxic condition. By phagocytosis of cellular debris, they supplement their need for nutrition, and by upregulating LDH and GLUT1, they compensate for local hypoxia.

Chest High-resolution Computed Tomography Imaging Features of Lung Involvement in Rheumatoid Arthritis

Objective To explore the chest high-resolution computed tomography (HRCT) features in patients with rheumatoid arthritis (RA) complicated with pulmonary involvement. Methods Totally 161 patients with RA with lung involvement were collected from June 2014 to May 2018. The chest HRCT findings were retrospectively analyzed. According to the imaging features as well as the results of history taking,pulmonary function test,pathology,and bronchoalveolar lavage fluid test,RA-related lung diseases (RA-LD) were classified and their clinical characteristics were compared. Results These 161 RA-LD patients (56 males and 105 females) whose mean age at diagnosis was (60.7±12.8) years (14-85 years) included 87 cases of usual interstitial pneumonia (UIP) (including 16 cases of possible UIP),44 cases of non-specific interstitial pneumonia (NSIP),10 cases of organizing pneumonia,7 cases of lymphocytic interstitial pneumonia,9 cases of small airway disease (SAD) (including 8 cases of bronchiolitis obliterans and 1 case of follicular bronchiolitis),and 4 other lung manifestations (including 3 cases of diffuse alveolar hemorrhage and 1 case of rheumatoid nodules). The UIP group had the oldest average age [(63.3±12.1) years old] and the highest smoking rate (41.4%). The SAD group had the youngest average age [(54.7±15.1) years old] and there was no smoking history. There were significant differences between these two groups (P=0.020,P<0.001). Seventy patients (43.5%) with RA-LD were complicated with pleural lesions. Conclusions RA involving the lung is common and has varied imaging manifestations,with interstitial lung diseases (mainly UIP and NSIP) being the most important manifestations. RA patients should undergo lung HRCT as early as possible to identify the lung involvement and related types.

WHEN TO BAL? A CASE OF CHRONIC EOSINOPHILIC PNEUMONIA PRESENTING WITH PROBABLE USUAL INTERSTITIAL PNEUMONIA PATTERN ON HIGH RESOLUTION CT SCAN AND CLINICAL FEATURES INITIALLY CONSISTENT WITH IDIOPATHIC PULMONARY FIBROSIS

SESSION TITLE: Wednesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Usual interstitial pneumonia (UIP) is a common radiographic pattern seen in interstitial lung disease, and is traditionally required for the diagnosis of Idiopathic pulmonary fibrosis (IPF). In older adults without clinical suspicion for other forms of interstitial lung disease, IPF diagnosis does not require BAL when UIP is present. In probable UIP, expert recommendations on the utility of BAL are conflicting. We present a patient with probable UIP on High resolution CT (HRCT), favorable clinical features for ILD, and negative serologies, who was diagnosed with chronic eosinophilic pneumonia after BAL. CASE PRESENTATION: This is 69 year old male with a past medical history of HTN, HLD, GERD, and a remote history of bladder cancer status post local resection. Over the past 1.5 years, he had been seen in our clinic for dyspnea, with a decline in functional status. His FVC was now moderately severe, with a reduced DLCO. His HRCT of the chest was consistent with probable UIP, and had progressed. He had recently begun home oxygen therapy, was referred for pulmonary rehabilitation, and had started pirfenidone. Multidisciplinary discussion with an advanced lung disease center was pursued. He had no systemic signs or symptoms of other causes of interstitial lung disease. He had not started any other new medications and was a life-long non-smoker. The only other finding was a low level peripheral eosinophilia. He underwent Bronchoscopy and BAL, which showed a 19% eosinophilic predominance, but with a low total yield. The BAL was repeated 4 months later, and now showed a 35% eosinophilic predominance. Plans were made to start him on prednisone therapy, and he has shown steady improvement since that time. DISCUSSION: IPF is the most common etiology for new UIP in the over 60 population. The association is strong enough, that the 2018 ATS executive summary on diagnosis of IPF recommends against performing BAL in UIP cases with a strong clinical picture for IPF and negative serologies. This recommendation was based on potential risks of bronchoscopy for IPF patients, and a small chance of revealing meaningful diagnostic information. In probable UIP, BAL is recommended but overall evidence is remarked to be weak. Occasionally more prognostically favorable forms of ILD may appear similar, and diagnostic clues to prompt BAL may be subtle or absent. Functional status prior to diagnosis is an important consideration, since these patients have the most to gain when other etiologies are revealed. CONCLUSIONS: UIP is most likely to be IPF in the correct clinical setting. However, thresholds for pursuing BAL and cellular fluid analysis that include features other than clinical status and radiographic pattern may prevent underdiagnoses of more treatable forms of ILD. Previous functional status and comorbidities should be factored into this decision as well. Reference #1: Raghu et al., "Diagnosis of Idiopathic Pulmonary Fibrosis: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline." ATS Documents, 2018 Reference #2: Cottin et al., "Eosinophilic Lung Diseases." Clin Chest Med. Sep, 2016. Reference #3: Oyama et al., "Efficacy of Short-Term Prednisone Treatment in Patients with Chronic Eosinophilic Pneumonia." Eur Respir J. JUN, 2015. DISCLOSURES: My spouse/partner as a Employee No relevant relationships by Jason Unger, source=Web Response

Computer-Aided Diagnosis of Pulmonary Fibrosis Using Deep Learning and CT Images.

ObjectivesThe objective of this study is to assess the performance of a computer-aided diagnosis (CAD) system (INTACT system) for the automatic classification of high-resolution computed tomography images into 4 radiological diagnostic categories and to compare this with the performance of radiologists on the same task.Materials and MethodsFor the comparison, a total of 105 cases of pulmonary fibrosis were studied (54 cases of nonspecific interstitial pneumonia and 51 cases of usual interstitial pneumonia). All diagnoses were interstitial lung disease board consensus diagnoses (radiologically or histologically proven cases) and were retrospectively selected from our database. Two subspecialized chest radiologists made a consensual ground truth radiological diagnosis, according to the Fleischner Society recommendations. A comparison analysis was performed between the INTACT system and 2 other radiologists with different years of experience (readers 1 and 2). The INTACT system consists of a sequential pipeline in which first the anatomical structures of the lung are segmented, then the various types of pathological lung tissue are identified and characterized, and this information is then fed to a random forest classifier able to recommend a radiological diagnosis.ResultsReader 1, reader 2, and INTACT achieved similar accuracy for classifying pulmonary fibrosis into the original 4 categories: 0.6, 0.54, and 0.56, respectively, with P > 0.45. The INTACT system achieved an F-score (harmonic mean for precision and recall) of 0.56, whereas the 2 readers, on average, achieved 0.57 (P = 0.991). For the pooled classification (2 groups, with and without the need for biopsy), reader 1, reader 2, and CAD had similar accuracies of 0.81, 0.70, and 0.81, respectively. The F-score was again similar for the CAD system and the radiologists. The CAD system and the average reader reached F-scores of 0.80 and 0.79 (P = 0.898).ConclusionsWe found that a computer-aided detection algorithm based on machine learning was able to classify idiopathic pulmonary fibrosis with similar accuracy to a human reader.

The role of video-assisted thoracoscopic surgery in the diagnosis of interstitial lung disease.

When a clinical context is indeterminate for idiopathic pulmonary fibrosis (IPF), or a chest high-resolution computed tomography (HRCT) pattern is not indicative of typical or probable usual interstitial pneumonia (UIP) in patients with interstitial lung disease (ILD), surgical lung biopsy should be considered to make a confident diagnosis on the basis of multidisciplinary diagnosis (MDD). The aim of this study was to evaluate the role and safety of video-assisted thoracoscopic surgery (VATS) in patients with ILD. A total of 143 patients with ILD underwent VATS at Toho University Medical Center Omori Hospital between March 2004 and April 2017. We conducted a retrospective study on the usefulness and safety of VATS in the diagnosis of ILD under MDD. The 30-day mortality was 0%. The postoperative complication rate was 12.6%, which included 5 cases of pneumothorax after discharge (3.5%), 4 cases of prolonged air leakage (2.8%), and 2 cases of acute exacerbation (1.4%). Three of 9 cases (33.3%) complicated by pneumothorax after discharge or prolonged air leakage were resected specimens of pleuroparenchymal fibroelastosis (PPFE). Two patients had acute exacerbation, who were ultimately diagnosed as having idiopathic unclassifiable IP and had histologically significant irregular dense fibrosis and numerous fibroblastic foci. The comparison between chest HRCT and histopathological findings revealed 55 cases of possible UIP [UIP (45%), NSIP (25%), and unclassifiable IP (29%)] and 21 cases of inconsistent with UIP [UIP (10%), NSIP (33%), organizing pneumonia (10%), unclassifiable IP (24%), and PPFE (24%)]. VATS can be safely performed to obtain a confident diagnosis for appropriate treatment strategies in patients with ILD.

CT Features of the Usual Interstitial Pneumonia Pattern: Differentiating Connective Tissue Disease-Associated Interstitial Lung Disease From Idiopathic Pulmonary Fibrosis.

A substantial proportion of cases of usual interstitial pneumonia (UIP) are due to connective tissue disease (CTD)-associated interstitial lung disease (ILD). The purpose of this study was to determine whether specific CT findings can help differentiate a UIP pattern of CTD-ILD from a UIP pattern of idiopathic pulmonary fibrosis (IPF) and whether these signs are associated with survival. Adults visiting an ILD clinic from 2006 to 2015 enrolled in a research registry with a multidisciplinary diagnosis of CTD-ILD or IPF and a UIP pattern at high-resolution CT were included in the study. In these subjects with CT findings of UIP due to either IPF or CTD-ILD, three CT findings anecdotally associated with CTD-ILD were assessed for diagnostic accuracy: the "straight-edge" sign, the "exuberant honeycombing" sign, and the "anterior upper lobe" sign. Survival assessments were performed with univariate and multivariable techniques. The subjects included 63 patients who had CTD-ILD and 133 patients who had IPF with a UIP pattern at CT. All three CT signs were significantly more common in subjects with CTD-ILD than those with IPF (prevalence, 22.2-25.4% for CTD-ILD, 6.0-12.8% for IPF; p = 0.028 to < 0.001). The highest specificity (94.0%) and sensitivity (25.4%) were seen for the straight-edge sign. No CT sign was associated with survival in multivariable analysis. Although UIP is usually associated with IPF, the index of suspicion for CTD-ILD should be raised in the care of patients with any of the three CT signs. A thorough workup for CTD-ILD should be pursued, including referral to the rheumatology department.

Sex steroid receptor expression in idiopathic pulmonary fibrosis

Usual interstitial pneumonia (UIP) is characterized by progressive scarring of the lungs and is associated with high morbidity and mortality despite therapeutic interventions. Sex steroid receptors have been demonstrated to play an important role in chronic lung conditions; however, their significance is unknown in patients with UIP. We retrospectively reviewed 40 idiopathic UIP cases for the expression of hormonal receptors. Forty cases including 10 normal lung, 10 cryptogenic organizing pneumonia, 10 idiopathic organizing diffuse alveolar damage, 7 hypersensitivity pneumonitis, and 3 nonspecific interstitial pneumonitis served as controls. Immunohistochemistry for estrogen receptor α, progesterone receptor (PR), and androgen receptor was performed in all groups. Expression of these receptors was assessed in 4 anatomic/pathologic compartments: alveolar and bronchiolar epithelium, arteries/veins, fibroblastic foci/airspace organization, and old scar. All UIPs (100%) stained positive for PR in myofibroblasts in the scarred areas, whereas among the control cases, only 1 nonspecific interstitial pneumonitis case stained focally positive and the rest were negative. PR was positive in myocytes of the large-sized arteries within the fibrotic areas in 31 cases (77.5%). PR was negative within the alveolar and bronchial epithelium, airspace organization, and center of fibroblastic foci; however, weak PR positivity was noted in the peripheral fibroblasts of the fibroblastic foci where they merged with dense fibrous connective tissue scar. All UIP and control cases were negative for androgen receptor and estrogen receptor α. This is the first study to show the expression of PR within the established fibrotic areas of UIP, indicating that progesterone may have profibrotic effects in UIP patients. Hormonal therapy by targeting PR could be of potential benefit in patients with UIP/IPF.

Pathologic Findings and Prognosis in a Large Prospective Cohort of Chronic Hypersensitivity Pneumonitis

The ability of specific histopathologic features to predict mortality or lung transplantation in patients with chronic hypersensitivity pneumonitis (HP) is unknown. Patients with chronic HP diagnosed by surgical lung biopsy were identified from an ongoing longitudinal cohort. The surgical lung biopsy slides were evaluated prospectively by an experienced thoracic pathologist using a standardized checklist to differentiate the major pathologic patterns and score the presence of specific histopathologic features. Cox proportional hazard analysis was used to identify independent predictors of transplant-free survival, and Kaplan-Meier analysis was used to visualize outcomes. One hundred nineteen patients were identified. Patients with a fibrotic nonspecific interstitial pneumonia (f-NSIP) pattern, bronchiolocentric fibrosis (BF) pattern, or usual interstitial pneumonia (UIP) pattern had significantly worse transplant-free survival than did those with a cellular NSIP (c-NSIP) pattern or peribronchiolar inflammation with poorly formed granulomas (PI-PFG) pattern. No survival difference among patients with an f-NSIP pattern, a BF pattern, or a UIP pattern was found. Fibroblastic foci were identified in a subset of biopsy samples from all pathologic patterns. Peribronchiolar fibrosis was noted in all UIP cases. Independent predictors of time to death or transplantation included the presence of fibroblast foci or dense collagen fibrosis. Histopathologic patterns of c-NSIP and PI-PFG had a better transplant-free survival than did patterns of UIP, f-NSIP, and BF. The presence of fibroblast foci or dense collagen fibrosis correlated with progression to death or lung transplantation. Identification of fibroblast foci on biopsy samples, regardless of the underlying histopathologic pattern, may be a clinically useful predictor of survival in patients with HP.

Usual interstitial pneumonia in asbestos-exposed cohorts - concurrent idiopathic pulmonary fibrosis or atypical asbestosis?

To determine whether usual interstitial pneumonia (UIP) pattern fibrosis is seen in asbestosis. The occurrence of UIP pattern fibrosis was studied in four asbestos cohorts systematically referred following postmortem to the UK Pneumoconiosis Unit, Cardiff. The combined exposed workforce comprised >25 000 persons. Over the 17-year period, 233 subjects were identified; 210 had degrees of interstitial fibrosis with a fibrotic non-specific interstitial pneumonia pattern and subpleural accentuation, and three showed UIP pattern fibrosis. All three of these cases showed grade 4 fibrosis (honeycombing) with no asbestos fibre dose-response correlation. A Poisson distribution of probability analysis indicated that the observed cases of UIP in this workforce could be wholly accounted for by the prevalence of idiopathic pulmonary fibrosis (IPF) in the population. UIP pattern fibrosis is rarely observed in asbestos-exposed subjects, and shows no dose-response correlation with asbestos fibres on mineral analysis; this points to an alternative disease, such as IPF. The results indicate that UIP pattern fibrosis should not be regarded as genuine asbestosis, irrespective of the status of asbestos biomarkers, and this impacts upon the postmortem handling of asbestos-related deaths.

Could prominent airway-centered fibroblast foci in lung biopsies predict underlying chronic microaspiration in idiopathic pulmonary fibrosis patients?

Chronic occult aspiration of small droplets (microaspiration) due to gastroesophageal reflux disease (GERD) and/or hiatal hernia is postulated to be a contributing factor in the pathogenesis of idiopathic pulmonary fibrosis (IPF). Usual interstitial pneumonia (UIP) is the histopathologic correlate of IPF. We hypothesized that chronic microaspiration may manifest as prominent airway-centered fibroblastic foci (FFs) in IPF. UIP cases diagnosed by wedge biopsies over a 6-year period (2006-2011) were identified and scored (1-3) for the prominence of airway-centered FFs by 2 authors blinded for clinical history. Relevant clinical information was obtained. Thirty-seven patients (22 men) were diagnosed with IPF by multidisciplinary approach. Thirteen cases (35.1%) demonstrated high airway-centered FF score (score 3). Twenty (54.1%) patients carried a clinical diagnosis of GERD, and 3 patients (8.1%) had hiatal hernia. High airway-centered FF score was significantly associated with hiatal hernia diagnosis (P=.037) but not with a diagnosis of GERD or the use of proton pump inhibitors/histamine-2 receptor antagonists. High airway-centered FF score was associated with airway-centered acute inflammation (P=.028) and peribronchiolar granulomas (P=.042). In summary, IPF cases with hiatal hernia were more likely to have a prominent airway-centered FF. Given the strong association between hiatal hernia and GERD and their risk for developing chronic microaspiration, the prominent airway-centered FF in UIP might predict the presence of chronic microaspiration, acknowledging that GERD and proton pump inhibitor/histamine-2 receptor antagonist use failed to demonstrate a significant association. Larger studies are warranted for further investigation.

Transbronchial biopsy is useful in predicting UIP pattern

BackgroundUsual interstitial pneumonia (UIP), is a necessary feature pathologically or radiologically for the diagnosis of idiopathic pulmonary fibrosis (IPF). The predictive value of transbronchial biopsy (TBB) in identifying UIP is currently unknown. The objective of this study is to assess the accuracy with which histopathologic criteria of usual interstitial pneumonia (UIP) can be identified in transbronchial biopsy (TBB) and to assess the usefulness of TBBx in predicting a the diagnosis of UIP pattern. We conducted a retrospective blinded and controlled analysis of TBB specimens from 40 established cases of UIP and 24 non-UIP interstitial lung diseases.ResultsAdequate TBB specimens were available in 34 UIP cases (85% of all UIP cases). TBB contained histopathologic criteria to suggest a UIP pattern (ie. at least one of three pathologic features of UIP present; patchy interstitial fibrosis, fibroblast foci, honeycomb changes) in 12 cases (30% of all UIP cases). Sensitivity, specificity, positive and negative predictive values for the two pathologists were 30% (12/40), 100% (24/24), 100% (12/12), 46% (24/52) and 30% (12/40), 92% (22/24), 86% (12/14), 55% (22/40) respectively. Kappa coefficient of agreement between pathologists was good (0.61, 95% CI 0.31-0.91). The likelihood of identifying UIP on TBB increased with the number and size of the TBB specimens.ConclusionAlthough sensitivity is low our data suggest that even modest amount of patchy interstitial fibrosis, fibroblast foci, honeycomb changes detected on TBB can be highly predictive of a UIP pattern. Conversely, the absence of UIP histopathologic criteria on TBB does not rule out UIP.

Diffuse Parenchymal Lung Disease-Diagnostic Approach by Radiology and Histopathology

Aims and objective:This study aims at evaluating the patients of diffuse parenchymal lung disease (DPLD), by comparing radiological and cyto-histopathological findings to determine the importance of histopathology in the diagnosis of DPLD. Materials and methods:Seventy patients with chief complaints of dyspnoea & cough and bilateral diffuse shadows on chest radiography were selected. High resolution computed tomography (HRCT) and bronchoscopy was done for further assessment. Results:Out of70 patients, 44 were finally diagnosed on the basis of bronchoscopic findings, as usual interstitial pneumonia (UIP) 24 cases (54.5%) and as non UIP in 20 cases (45.5%). Two cases of UIP additionally suffered from bronchogenic carcinoma. Conclusion:A combination of clinical, radiological, cytological and histological findings is necessary to evaluate the cases of DPLD and transbronchial needle biopsy (TBNB) gives a definitive edge over other diagnostic measures.

A study on the efficacy of glucocorticoid therapy for idiopathic nonspecific interstitial pneumonia

To investigate the efficacy of glucocorticoid therapy in idiopathic nonspecific interstitial pneumonia (INSIP). Twenty-nine cases of INSIP confirmed by clinical- radiological-pathological (CRP) diagnosis were collected and classified into 2 groups according to the degree of fibrosis: INSIP-1 (including 9 cases of cellular type) and INSIP-2 (20 cases of mixed and fibrotic type). Thirty cases of usual interstitial pneumonia (UIP) confirmed by CRP diagnosis served as the control. Clinical and pathological features, therapeutic effects of glucocorticoids and the follow-up results were retrospectively analyzed and the survival curves were evaluated by Kaplan-Meier method. The mean age at onset of INSIP-1 group [(48 ± 5) years] was significantly younger than INSIP-2 group [(52 ± 11) years] and the UIP group [(57 ± 14) years]. The course of disease in INSIP-1 group [(60 ± 28) months] was longer than that in INSIP-2 group [(48 ± 33) months] and that in the UIP group [(44 ± 23) months], but the differences were not statistically significant (F = 1.22, all P > 0.05). The efficacy rate of glucocorticoid treatment in INSIP-1 group (9/9) was higher than that in INSIP-2 group (11/20) and that in the UIP group (2/30), the differences being statistically significant (all P < 0.05). The follow-up period for INSIP-1 group [(56 ± 27) months] was significantly longer than for INSIP-2 group [(23 ± 18) months] and for the UIP group [(25 ± 17) months], and the rate of significant improvement (6/9) was higher than that of the INSIP-2 group (9/20) and the UIP group (0/30), the differences being statistically significant (F = 9.224, all P < 0.05). The mortality of INSIP-1 group (0/9) was lower than that in INSIP-2 group (4/20) and the UIP group (16/30), the difference being statistically significant (exact probability value 0.000 - 0.005, P < 0.05). The degree of fibrosis of INSIP is closely correlated with the effect of glucocorticoid therapy and prognosis. The cellular type has a favorable reaction to glucocorticoid therapy and a better prognosis as compared to the fibrotic type.

Etiological examination of idiopathic interstitial pneumonia and lung cancer in autopsy cases

In order to investigate the etiology of usual interstitial pneumonia (UIP) and UIP with lung cancer (LC), autopsy findings in 18 cases of UIP with LC and 11 cases of uncomplicated UIP were clinicopathologically compared with the environmental factors of smoking habits and occupation. UIP with LC was highly correlated with smoking, especially heavy smoking and with occupations in which dust is inhaled, such as electrical installation and ceramic production, indicating that these environmental factors are important background factors in the complication of UIP with IC. Pathologic examination of cases of UIP with LC (6 squamous cell carcinomas, 5 small cell carcinomas, 4 adenocarcinomas, and 3 large cell carcinomas, 2 of which showed pulmonary double carcinoma revealing a slight correlation between fibrosis and primary site of LC and a slightly greater correlation of squamous cell carcinoma and small cell carcinoma to smoking habits and inhalation of dust. In terms of the correlation between UIP and LC among autopsy cases, the environmental factors proved to be more significant than the fibrotic findings. These environmental factors are thought to merit consideration as common predisposing factors in the development of LC and its complication with UIP.