Concordance between sequential transbronchial lung cryobiopsy and surgical lung biopsy in patients with diffuse interstitial lung disease

BACKGROUND: Increasing evidence indicates the utility of transbronchial lung cryobiopsy (TBLC) for the diagnosis of interstitial lung disease (ILD). However, only one study has compared TBLC and surgical lung biopsy (SLB) performed on the same patients. METHODS: We identified seven patients with ILD with TBLC and SLB. We evaluated the clinical characteristics and made a pathological diagnosis based on the official ATS/ERS/JRS/ALAT clinical practice guideline of idiopathic pulmonary fibrosis with both TBLC and SLB. RESULTS: Six cases were diagnosed as Usual interstitial pneumonia (UIP) in both TBLC and SLB. One case was diagnosed as indeterminate for UIP with TBLC and probable UIP with SLB. Etiological diagnosis with TBLC and SLB were concordant in 2 cases of idiopathic pulmonary fibrosis (IPF) but discordant for other diagnoses. Major histological findings of UIP including dense fibrosis, peripheral distribution, and fibroblastic foci showed high concordance between TBLC and SLB, which implies that TBLC can reliably detect these features. In contrast, loose fibrosis, cellular infiltration, and airway disease showed poor concordance between the two methods. CONCLUSION: Our study showed that TBLC is useful for UIP diagnosis but not for other ILD. With a multidisciplinary approach, diagnosis of IPF may be determined by TBLC, whereas ILD other than IPF may require SLB

A case report of pulmonary amyloidosis recognized by detection of AA amyloid exclusively in alveolar macrophages

Amyloidosis is a rare condition in which tissue deposits of inert fibrillar protein result in organ damage and dysfunction. There are several types of amyloid fibrils. Some of the most common forms are AL (amyloid light chain) protein and AA (amyloid-associated) type of amyloid fibril protein. Pulmonary amyloidosis is relatively common but is usually asymptomatic. Thus, the diagnosis may be easily overlooked. A 78-year-old male with a history of multiple myeloma followed by systemic amyloidosis presented with abnormal chest CT showing diffuse interlobular thickening in the whole lung field with bilateral pleural effusion. Bronchoalveolar lavage and transbronchial biopsy were performed. Due to the patient’s poor condition and hemorrhage, only one fragment was available from forceps biopsy. Histologically, there was no amyloid deposition in the lung parenchyma;however, some histiocytes showed eosinophilic granular contents which prompted us to perform additional staining. The cytoplasmic material turned to be positive with direct fast scarlet (DFS) staining and AA amyloid immunostaining. Similar macrophages with AA amyloid were also found in the bronchoalveolar fluid. We experienced a case with AA amyloidosis affecting the lung diagnosed by the presence of intracytoplasmic amyloid in alveolar macrophages. The microscopic changes were so subtle that they may be overlooked. Recognition of amyloid deposition in alveolar macrophages may be an important clue to diagnose pulmonary amyloidosis. Such finding is of particular significance in the small-sized specimens, such as biopsies and cytologic smears

Recognition of Connective Tissue Disease-Related Interstitial Pneumonia Based on Histological Score—A Validation Study of an Online Diagnostic Decision Support Tool

Objectives: to evaluate the number of cases of idiopathic pulmonary fibrosis (IPF) that included histological features of connective tissue disease (CTD) and to check whether they demonstrated the clinical features of CTD, using a previously reported CTD-interstitial pneumonia (IP) index that histologically differentiates CTD-associated and idiopathic IP. Methods: patients diagnosed with IPF following video-assisted thoracoscopic biopsy through multidisciplinary team diagnosis between 2014 and 2017 were selected. Pathological observation was made by four pathologists who scored eight observational items needed for the CTD-IP index. Cases determined as CTD, by the CTD-IP index, were extracted, and their clinical features were compared. Results: a total of 94 cases of IPF were identified, of which 20 were classified into the CTD group using the CTD-IP index with reasonable interobserver agreement (k = 0.76). Cases pathologically classified into the CTD group were significantly associated with female sex, non-smoking history, autoantibody positivity, and CTD symptoms (p = 0.01, 0.03, 0.01, and 0.04, respectively). Conclusions: patients with IPF with pathological findings of CTD showed clinical characteristics similar to those of patients with CTD

Histologic factors associated with nintedanib efficacy in patients with idiopathic pulmonary fibrosis

Background Histopathologic factors predictive of nintedanib efficacy in idiopathic pulmonary fibrosis have not been studied. We aimed to describe the characteristics, focusing on histopathology, of idiopathic pulmonary fibrosis patients who did and did not respond to nintedanib. Methods This study retrospectively examined the clinicoradiopathologic features of 40 consecutive patients with surgical lung biopsy-confirmed idiopathic pulmonary fibrosis treated with nintedanib. Additionally, we compared the histopathologic scoring of 21 microscopic features between patients with functional or radiological progression and those with non-progression during 12 months of treatment. Results The histopathologic evaluation showed edematous changes in the interlobular septum as the only histologic finding observed more frequently in patients with both functional and radiological progression than in those without (58% vs. 14%, P = 0.007 and 50% vs. 0%, P = 0.003, respectively). Regarding per-year change, patients with edematous changes in the interlobular septum showed greater progression in median changes in spared area (-12%, interquartile range: [-25%-5%], vs. -3% [-7%-0%], P = 0.004) and reticular shadow (7% [3%-13%], vs. 0% [0%-5%], P = 0.041) on computed tomography. Functional and radiological progression-free survival were shorter in patients with edematous changes in the interlobular septum than in those without (6.6 months, 95% confidence interval: [5.9-25.3], vs. event <50%, [12.1-Not available], P = 0.0009, and 6.1 months, [5.2-6.6] vs. 14.5 months [7.8-not available], P<0.0001). Conclusions Edematous changes in the interlobular septum may indicate poor nintedanib efficacy in idiopathic pulmonary fibrosis. Further studies are needed to validate these findings and address the mechanism behind ECIS

Determining Response to Treatment for Drug-Induced Bronchocentric Granulomatosis by the Forced Oscillation Technique

Anti-tumor necrosis factor alpha (TNFα) therapy is widely used to treat various inflammatory conditions. Paradoxically, there are several case reports describing the development of bronchocentric granulomatosis treated with TNFα inhibitors, and it is difficult to determine the effect of treatment using conventional spirometry because the lesions are located in small airways. However, it has been reported that the forced oscillation technique (FOT) is useful in the evaluation of small airway disease in bronchial asthma or chronic obstructive pulmonary disease. We performed the FOT to determine the effect of treatment on bronchocentric granulomatosis and found it to be useful. We report the case of a 55-year-old female with ulcerative colitis who was treated with golimumab and who developed bronchocentric granulomatosis as a sarcoid-like reaction to golimumab. She was successfully treated with prednisone, and the treatment efficacy was confirmed by the FOT. The FOT may be useful in the evaluation of small airway disease in bronchocentric granulomatosis. This case may help inform clinicians of the usefulness of the FOT to assess small airway disease in various diseases

Evaluation of stress distribution due to shearing in non-oriented electrical steel by using synchrotron radiation

The influence of the shearing process on the iron loss of non-oriented electrical steels with grain sizes of 10 μm-150 μm was investigated. The deterioration ratio of iron loss was clearly smaller in sample with small grain sizes. The droop height, reflecting the amount of plastic deformation, displayed a good relationship with the deterioration of iron loss under the effect of the material grain size. To clarify the strain distribution around the sheared edge, the elastic strain in a sheet sample with the thickness of 0.30 mm and grain size of 10 μm was evaluated by using synchrotron radiation. The width of the region of elastic strain due to shearing was two or three times of the material thickness. The results of the plastic strain distribution obtained by the measurements were then used to estimate the iron loss deterioration rate in 5 mm width sheared samples. The estimated loss deteriotation coincided with the actual measured iron loss

Combination of pleuroparenchymal fibroelastosis with non-specific interstitial pneumonia and bronchiolitis obliterans as a complication of hematopoietic stem cell transplantation ? Clues to a potential mechanism

Pleuroparenchymal fibroelastosis (PPFE) is a newly described entity of interstitial lung disease,which has been recently recognized as a rare complication of bone marrow transplantation. We report a case of 30-year-old man who developed a unique combination of pleuroparenchymal fibroelastosis with cellular and fibrotic non-specific interstitial pneumonia (NSIP) and bronchiolitis obliterans (BO) sixteen yearsafter hematopoietic stem cell transplantation. Histological examination revealed almost exclusive infiltration of CD3-positive T lymphocytes associated with lymphoepithelial lesions and multi-focal denudation of covering epithelial cells in all components. This case suggests PPFE, NSIP, and BO might be conditions of the same spectrum, pathogenetically related to chronic graft-versus-host disease. Immunostaining for CD3 and CD20 in transbronchial lung biopsies may be helpful for identifying graft-versus-host-driven interstitial lung disease

Concordance between sequential transbronchial lung cryobiopsy and surgical lung biopsy in patients with diffuse interstitial lung disease

BACKGROUND: Increasing evidence indicates the utility of transbronchial lung cryobiopsy (TBLC) for the diagnosis of interstitial lung disease (ILD). However, only one study has compared TBLC and surgical lung biopsy (SLB) performed on the same patients. METHODS: We identified seven patients with ILD with TBLC and SLB. We evaluated the clinical characteristics and made a pathological diagnosis based on the official ATS/ERS/JRS/ALAT clinical practice guideline of idiopathic pulmonary fibrosis with both TBLC and SLB. RESULTS: Six cases were diagnosed as Usual interstitial pneumonia (UIP) in both TBLC and SLB. One case was diagnosed as indeterminate for UIP with TBLC and probable UIP with SLB. Etiological diagnosis with TBLC and SLB were concordant in 2 cases of idiopathic pulmonary fibrosis (IPF) but discordant for other diagnoses. Major histological findings of UIP including dense fibrosis, peripheral distribution, and fibroblastic foci showed high concordance between TBLC and SLB, which implies that TBLC can reliably detect these features. In contrast, loose fibrosis, cellular infiltration, and airway disease showed poor concordance between the two methods. CONCLUSION: Our study showed that TBLC is useful for UIP diagnosis but not for other ILD. With a multidisciplinary approach, diagnosis of IPF may be determined by TBLC, whereas ILD other than IPF may require SLB