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Anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5-Ab) is associated with fatal rapidly progressive interstitial lung disease (RP-ILD) in patients with dermatomyositis (DM). We attempted to clarify whether anti-MDA5-Ab is associated with long-term outcomes in patients with DM-ILD.Thirty-six patients with DM-ILD were retrospectively analyzed for their serum anti-MDA5-Ab by using an enzyme-linked immunosorbent assay. We analyzed the association between clinical parameters, including the serum levels of anti-MDA5-Ab and ferritin.Fourteen patients (39%) were positive for anti-MDA5-Ab. The serum levels of anti-MDA5-Ab and ferritin in 7 patients with acute death were higher than those in the surviving patients. An "unclassifiable pattern" on chest computed tomography and the development of RP-ILD were also prognostic markers. The serum levels of anti-MDA5-Ab and ferritin (cut-off levels, 100 IU/mL and 899 ng/mL, respectively) were markers predictive of acute death, showing good sensitivity (86% and 83%) and specificity (97% and 100%). All 7 patients with acute death developed RP-ILD and were positive for anti-MDA5-Ab, including 6 patients with a high titer (≥100 IU/mL), whereas only 2 patients (29%) developed RP-ILD among the 7 survivors with a low titer of anti-MDA5-Ab (100 IU/mL). In contrast, a low positive titer of anti-MDA5-Ab was not associated with changes in pulmonary function for 2 years.Although a high serum titer of anti-MDA5-Ab (≥100 IU/mL) is associated with acute death via the development of RP-ILD, outcomes in the chronic phase for patients with a low titer of anti-MDA5-Ab (100 IU/mL) were similar to those of patients without anti-MDA5-Ab.

Background We attempted to clarify whether the presence of anti-aminoacyl-transfer RNA synthetase antibody (anti-ARS Ab) or anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 Ab) is associated with the therapeutic response of polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD). Methods We retrospectively investigated 22 patients with PM/DM-ILD (10 positive for anti-ARS Ab and nine positive for anti-MDA5 Ab) for whom antibody analysis of conserved serum was possible. We assessed mortality in the first three months as the therapeutic response in the acute phase and compared changes in clinical data for up to one year considered as the chronic phase. We classified the clinical changes over the year into three groups: Improvement (increased % vital capacity [%VC] or diffusing capacity of the lung for carbon monoxide [%D LCO ]≥10 or 15%), deterioration (decreased %VC or %D LCO ≥10 or 15%), and no change (remainder of the changes). The extent of abnormality demonstrated by high-resolution computed tomography (HRCT) was scored. Results Positivity for anti-MDA5 Ab, but not for anti-ARS Ab, was associated with mortality in the first 3 months. Evaluation of the therapeutic response in the first year showed that positivity for the anti-ARS Ab, but not for the anti-MDA5 Ab, was associated with an improvement in %D LCO and a decline in the serum KL-6 levels. Positivity for the anti-ARS Ab or negativity for anti-MDA5 Ab was associated with a greater decrease in bronchial dilatation as seen by HRCT. Conclusions Anti-ARS and anti-MDA5 Abs are associated with the therapeutic response of PM/DM-ILD.

Background The relationship between the histological pattern and survival in systemic sclerosis-associated interstitial lung disease (SSc-ILD) is unclear. In patients with SSc-ILD, we investigated whether the clinical data obtained by non-invasive examinations could be used for prognostic evaluation, and attempted to clarify whether complicating acute exacerbation (AE) and the selection of pharmacological therapy were associated with survival. Methods Thirty-five patients with SSc-ILD, who had not been diagnosed by surgical lung biopsy were analyzed, retrospectively. The HRCT findings were evaluated by 2 radiologists and classified into "CT-UIP" or "CT-inconsistent with UIP" patterns based on whole lung interpretations. HRCT scores were calculated based on the extent of abnormality evidenced by HRCT. The log-rank test was used to determine variables, including clinical parameters and histories. Results Twelve (34%) of the 35 patients died during a median follow-up period of approximately 7.9 years. The log-rank test showed that a higher mortality was associated with higher age, a CT-UIP pattern, a higher score for ground-glass attenuation with traction bronchiectasis on HRCT, and complicating AE, whereas a lower mortality was significantly associated with the use of immunosuppressants. A CT-UIP pattern was significantly associated with a higher incidence of later AE. Conclusion Treatment with immunosuppressants was associated with a longer survival, and complicating AE is a predictor of shortened survival in SSc-ILD patients. Among the clinical parameters determined by non-invasive examinations, a CT-UIP pattern and the extent of fibrotic lesions on HRCT, but not a histological pattern of UIP, may be predictors of shortened survival.

BACKGROUND Lung-dominant connective tissue disease (LD-CTD) is a disease concept for interstitial pneumonia; however, it has not been robustly validated. /p study was conducted to elucidate the clinical, radiologic, and histologic features of LD-CTD. METHODS We retrospectively reviewed 44 consecutive patients with serologically defined LD-CTD who underwent surgical lung biopsy. Six were identified as having LD-CTD if they had specific autoantibodies but did not meet the criteria for connective tissue disease. We conducted a multidisciplinary diagnosis and evaluated major histologic patterns according to the current idiopathic interstitial pneumonias (IIPs) classification of 2013. Characteristic histologic features for LD-CTD (eg, prominent plasmacytic infiltration, lymphoid aggregates with germinal centers), high-resolution CT (HRCT) scan patterns, and prognosis were also assessed. METHODS The major histologic patterns were usual interstitial pneumonia (UIP) in 25 patients and nonspecific interstitial pneumonia (NSIP) in 13 patients. Two or more characteristic histologic features for LD-CTD were observed in 15 patients with histologic UIP (h-UIP) and 11 patients with histologic NSIP (h-NSIP). Fifteen patients with h-UIP (60%) showed an inconsistent UIP pattern on HRCT scan multidisciplinary discussion (MDD), 18 patients with h-UIP were labeled as having unclassifiable IIP. The annual change in percent predicted FVC improved significantly in patients with h-NSIP (P = .002), who also had better survival than those with h-UIP (P = .031). In contrast, survival was not associated with HRCT scan pattern (P = .79). CONCLUSIONS The major histologic patterns in LD-CTD were UIP followed by NSIP. Two-thirds of patients had characteristic histologic features for LD-CTD. A majority of patients with h-UIP were considered to have unclassifiable IIP based on MDD. Patients with h-UIP had worse survival than those with h-NSIP. CHEST 2015;148(6):1438-1446

The aim was to characterize the computed tomographic (CT) findings from Japanese patients with lymphangioleiomyomatosis (LAM).CT scans of the chest, abdomen, and pelvis from 124 patients with sporadic LAM (S-LAM, mean age, 37.4 years) and 14 patients with tuberous sclerosis complex (TSC)-LAM (mean age, 35.6 years) were analyzed.Pulmonary nodules (18.8%) and hepatic angiomyolipoma (AML, 24.3%) were more common in our patients than those in previous reports. Compared with TSC-LAM, S-LAM group had a higher frequency of pulmonary nodules (28.6% vs 32.3%, P0.01) and lower frequencies of air-space consolidation (21.4% vs 2.4%, P0.01), pneumothorax (28.6% vs 8.1%, P=0.02), pulmonary hilar lymphadenopathy (14.3% vs 0.8%, P0.01), renal AML (85.7% vs 17.4%, P0.01), hepatic AML (71.4% vs 17.4%, P0.01), and retrocrural lymphadenopathy (14.3% vs 1.4%, P=0.04). Axial lymphatic abnormalities (i.e., thoracic duct dilatation, lymphadenopathy, and lymphangioleiomyoma) were most common in the pelvis and tended to decrease in incidence with increased distance from the pelvis.The incidence of some CT findings in Japanese patients differed from those in previous reports. Axial lymphatic abnormalities noted here suggest that the origin of LAM cells may be the pelvis.

An 83-year-old man was transferred to our hospital due to worsening effort angina pectoris. Electrocardiogram during chest pain at rest showed ST-segment elevation with hyperacute T waves and negative U waves in leads V2 through V4 ([Figure 1A][1]). Sublingual administration of nitroglycerin

Although usual interstitial pneumonia (UIP) is the most common chronic interstitial pneumonia, understanding of pathologic backgrounds of CT findings has still not been enough. Since honeycombing on either scanning microgram or CT is essential for diagnosis of UIP in 2010 ATS-ERS-JRS-ALAT guide line, the role of radiologists has become much more important. We will summarize common and uncommon CT findings with radiologic-pathological correlation.

The purpose of this study was to identify independent CT findings that correlated with pulmonary function tests (PFTs) in patients with obstructive airway diseases.Sixty-eight patients with obstructive airway disease and 29 normal subjects (mean age, 52 years; 36 men and 61 women) underwent inspiratory and expiratory thin-section CT and PFTs. Patient with obvious emphysema was excluded. Two radiologists independently reviewed the images and semi-quantitatively evaluated lung attenuation (mosaic perfusion, air trapping) and airway abnormalities (extent and severity of bronchial wall thickening and bronchiectasis, bronchiolectasis or centrilobular nodules, mucous plugging). Univariate, multivariate and receiver operating characteristic (ROC) analyses were performed with CT findings and PFTs.Forty-two patients showed obstructive PFTs, 26 symptomatic patients showed near-normal PFTs. On univariate analysis, air trapping and bronchial wall thickening showed highest correlation with obstructive PFTs such as FEV1.0/FVC, MMEF and FEF75 (r ranged from -0.712 to -0.782; p0.001), while mosaic perfusion and mucous plugging showed moderate correlation, and bronchiectasis, bronchiolectasis and nodules showed the least, but significant, correlation. Multiple logistic analyses revealed air trapping and bronchial wall thickening as the only significant independent determinants of obstructive PFTs. ROC analysis revealed the cut-off value of air trapping for obstructive PFTs to be one-third of whole lung (area under curve, 0.847).Our study confirmed air trapping and bronchial wall thickening are the most important observations when imaging obstructive PFTs. The cut-off value of air trapping for identifying obstructive PFTs was one-third of lung irrespective of inspiratory CT findings.

To evaluate the detectability and signal intensities of small (or=2 cm) dysplastic nodules (DNs) and well-differentiated hepatocellular carcinomas (w-HCCs) by T2*-weighted gradient echo (GRE) sequences using an intermediate echo-time (TE) with ferucarbotran in a 1.0-T magnetic resonance imaging (MRI) unit.Pathologically confirmed DNs (n=13) and w-HCCs (n=31) with a median largest dimension of 1.1cm were scanned using ferucarbotran-enhanced MRI. Conventional T2*-weighted GRE sequences (conventional-T2*-GRE: repetition time, 280 ms; echo time, 14 ms; flip angle, 60 degrees) and specific T2*-weighted GRE sequences using an intermediate-TE (specific-T2*-GRE: repetition time, 140 ms; echo time, 8 ms; flip angle, 30 degrees) were obtained before and after ferucarbotran administration. Two independent observers scored all nodules for visibility and assigned confidence level scores to their observations. To assess the effect of ferucarbotran, the tumor-liver signal contrast-to-noise ratio (tumor-liver-CNR) was also calculated for detected nodules by the same two observers with consensus.There was good interobserver agreement regarding the presence of nodules for both sequence types. Qualitative and quantitative analyses indicated that specific-T2*GRE sequences were superior to conventional-T2*-GRE sequences for detecting DNs and w-HCCs with hypointense signals. The tumor-liver-CNR of DNs was significantly different between specific-T2*-GRE sequences and conventional-T2*-GRE sequences (Mann-Whitney test, P0.001). Both qualitative and quantitative analyses indicated that conventional-T2*-GRE sequences were superior to specific-T2*-GRE sequences for detecting w-HCCs with heterogeneous and hyperintense signals.Specific-T2*-GRE sequences with ferucarbotran are useful for detecting DNs and w-HCCs that produce hypointense signals on a 1.0-T MRI unit.

To assess the CT and magnetic resonance (MR) imaging findings of thymic epithelial tumors classified according to the current World Health Organization (WHO) histologic classification and to determine useful findings in differentiating the main subtypes.Sixty patients with thymic epithelial tumor who underwent both CT and MR imaging were reviewed retrospectively. All cases were classified according to the 2004 WHO classification. The following findings were assessed in each case on both CT and MRI: size of tumor, contour, perimeter of capsule; homogeneity, presence of septum, hemorrhage, necrotic or cystic component within tumor; presence of mediastinal lymphadenopathy, pleural effusion, and great vessel invasion. These imaging characteristics of 30 low-risk thymomas (4 type A, 12 type AB, and 14 type B1), 18 high-risk thymomas (11 type B2 and seven type B3), and 12 thymic carcinomas on CT and MR imaging were compared using the chi-square test. Comparison between CT and MR findings was performed by using McNemar test.On both CT and MR imaging, thymic carcinomas were more likely to have irregular contours (P.001), necrotic or cystic component (P.05), heterogeneous contrast-enhancement (P.05), lymphadenopathy (P.0001), and great vessel invasion (P.001) than low-risk and high-risk thymomas. On MR imaging, the findings of almost complete capsule, septum, and homogenous enhancement were more commonly seen in low-risk thymomas than high-risk thymomas and thymic carcinomas (P.05). MR imaging was superior to CT in the depiction of capsule, septum, or hemorrhage within tumor (all comparison, P.05).The presence of irregular contour, necrotic or cystic component, heterogeneous enhancement, lymphadenopathy, and great vessel invasion on CT or MR imaging are strongly suggestive of thymic carcinomas. On MR imaging, the findings of contour, capsule, septum, and homogenous enhancement are helpful in distinguishing low-risk thymomas from high-risk thymomas and thymic carcinomas.

AIM To assess the relationship between initial CT pattern and serial changes in CT findings and pulmonary function tests (PFTs) in patients with non-specific interstitial pneumonia (NSIP). MATERIALS AND METHODS Serial high resolution (HR) CTs and PFTs were retrospectively analyzed in 38 cases of histologically proven NSIP, including 4 with cellular NSIP, 13 with mixed cellular and fibrotic NSIP, and 21 with fibrotic NSIP. The presence and extent of various CT findings were assessed. A fibrosis index (defined as the ratio of the extent of a reticular/honeycomb pattern to the overall extent of abnormal parenchyma) was derived. RESULTS The predominant CT pattern was reticular/honeycomb in 27 (84%) cases and ground-glass/consolidation in 6 (16%) cases. Between scans, mean disease extent reduced by 5.2%. Disease extent reduced by >10% in 13 (34%) and increased by >10% in 6 (16%) patients. Histopathological subtype of NSIP did not correlate with individual CT pattern, predominant pattern, fibrosis index or serial change in disease extent on CT or PFTs. Response on follow-up CT was associated with fibrosis index, predominant pattern and extent of consolidation on initial CT. CONCLUSION In NSIP disease, progression on CT correlates with the predominant CT pattern, fibrosis index, and extent of consolidation but not with histopathological subtype. An inflammatory (ground-glass/consolidation) predominant pattern is associated with better outcome in terms of disease extent on HRCT.

Study objectives The purpose of this study was to describe the clinical, chest radiographic, high-resolution CT, and histopathologic features of clay dye "sendo" dust pneumoconiosis in seven rush mat ("tatami") workers. Patients Seven patients with a history of occupational exposure to sendo dust and radiographic changes suggestive of pneumoconiosis were retrospectively reviewed. Results The duration of exposure ranged from 15 to 45 years (median, 30 years). Three patients had cough, and four patients had abnormal pulmonary function test results. Chest radiographs showed nodular opacities Conclusion Rush mat workers' sendo dust pneumoconiosis is caused by dust containing free silica. The radiographic and high-resolution CT findings consist of small nodular opacities

The clinical effectiveness of erythromycin for patients with diffuse panbronchiolitis (DPB) was previously recognized. However, it remains unknown what kind of airway lesions change with the clinical effectiveness induced by erythromycin. We performed the present study to clarify this unknown. We devised a method for scoring findings on high-resolution computed tomography (HRCT) to aid in the objective evaluation of the airway lesions in patients with DPB. The 18 patients with DPB were treated with oral erythromycin, 600 mg/d. All patients were evaluated by pulmonary function tests and HRCT before and after 3 months of therapy. Characteristic HRCT findings in patients with DPB pretherapy were small nodules, airway ectasia, periairway thickening, and mucus plugging. After erythromycin therapy, there was significant reduction in scores for the extent of small nodular opacities, the severity of periairway thickening, and the extent of mucus plugging with a corresponding significant improvement in results of the pulmonary function test parameters. The present study demonstrated reversible airway lesions in patients with DPB in response to erythromycin therapy.

RESUMO: Nos doentes com cancro do pulmão, as pneumonias têm uma mortalidade elevada; contudo, os agentes patogénicos envolvidos não estão esclarecidos devido à contaminação habitual da expectoração.Este estudo tem como objectivo fazer a identificação das bactérias isoladas nas secreções brônquicas obtidas por broncofibroscopia.A população era constituída por 431 doentes, incluindo 210 com adenocarcinoma, 119 com carcinoma espino-celular, 17 com carcinoma de grandes células, 73 com carcinoma de pequenas células e 18 com outros cancros.O lavado brônquico foi efectuado com 20 m1 de soro fisiológico seguido de biópsia transbrônquica.As culturas dos lavados brônquicos foram negativas em 112 doentes e revelaram a e/ou não Streptococcus hemolítico em 14 doentes, Staphylococcus pneumoniae em 14 doentes, Pseudomonas aeruginosa em 12 doentes e outros microrganismos em 42 doentes.Em 16 doentes foram identificadas as mesmas bactérias quer na expectoração quer no lavado brônquico.Quando o Grupo A (negativo, a e/ou não Streptococcus, Neissema e Candida) é comparado com o grupo B (positivo para outros agentes patogénicos), a febre é significativamente mais frequente no grupo B. Estas bactérias podem colonizar os pulmões dos doentes com cancro e podem estar relacionados com os episódios febris após a broncoscopia, causando problemas clínicos durante a quimioterapia. COMENTÁRIO: As infecções pulmonares, particularmente as pneumonias, complicam frequentemente o curso do cancro do pulmão, sendo muitas vezes a causa de morte última.Os factores locais e sistémicos do cancro do pulmão predispõem os doentes para infecções pulmonares, assim como para outras infecções. Contudo, as bactérias mais frequentemente envolvidas nestas situações não estão completamente esclarecidas, obrigando a maior parte das vezes a uma terapêutica empírica. Assim, analisando os resultados deste trabalho, achamos que a broncoscopia é um método diagnóstico não só do cancro mas também no diagnóstico etiológico das infecções respiratórias e/ou pneumonias destes doentes. Os autores constataram que as bactérias patogénicas podem colonizar os pulmões sem causarem sintomas, sendo por vezes as mesmas que estão a colonizar a orofaringe. Perante estes factos, as bactérias patogénicas isoladas nos SB e no lavado brônquico parece terem importância clínica quando não são as mesmas isoladas na expectoração e orofaringe, cujo tratamento com antibioticoterapia dirigida pode reduzir as complicações dos doentes a fazer quimioterapia.Apesar de poder haver colonização pulmonar sem sintomatologia, a maior parte dos resultados também permite afirmar que a febre pós-broncoscopia não obriga sempre a tratamento de urgência e que não influencia a sobrevida deste grupo de doentes. Contudo, o tratamento etiológico, orientado pelos exames bacteriológicos culturais do lavado brônquico, pode ser útil prevenindo os episódios de febre de determinados doentes que complicam a quimioterapia. Palavras-chave: Lavagem brônquica, cancro do pulmão, febre, bactéria