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Introduction: Autoimmune pulmonary alveolar proteinosis (APAP) is caused by macrophage dysfunction due to anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibody. APAP complicated with sarcoidosis is rarely reported. Subject and Methods: We have diagnosed two cases of APAP complicated with sarcoidosis. We have measured serum levels of anti-GM-CSF autoantibody in our institute, and investigated the changes of its serum levels during the clinical course. Results: Case 1 is 42-years old female and Case 2 is 51-years old male at the diagnosis of sarcoidosis. Serum anti-GM-CSF antibody was positive in both cases. Case 1 was a typical APAP diagnosed at the age of 29 with milky bronchoalveolar lavage and crazy paving pattern on high-resolution CT. APAP was resolved after GM-CSF inhalation therapy at the age of 30 and sarcoidosis with lung, skin and eye lesions, occurred after the 10-year stable period of APAP. Case 2 was diagnosed as APAP and sarcoidosis, simultaneously. Surgical lung biopsy specimens showed granulomatous lesion consistent with sarcoidosis and intra-alveolar Periodic Acid Schiff stain-positive proteinaceous material compatible with APAP. Sarcoidosis lesions were also observed in skin and eye. In both cases, serum levels of angiotensin converting enzyme was decreased and dyspnea with hypoxemia was improved after the corticosteroid therapy. In both cases, APAP lesions were limited and serum levels of anti-GM-CSF antibody was low at the diagnosis of sarcoidosis. Conclusions: Anti-GM-CSF autoantibody was associated with clinical couse. Financial support: This study was partially granted from AMED to Y. Inoue and T. Arai.

The purpose was to identify distinguishing CT features of pathologically diagnosed asbestosis, and correlate diagnostic confidence with asbestos body burden. Thirty-three workers (mean age at CT: 73 years) with clinical diagnoses of asbestosis, who were autopsied (n = 30) or underwent lobectomy (n = 3), were collected. Two radiologists independently scored high-resolution CT images for various CT findings and the likelihood of asbestosis was scored. Two pathologists reviewed the pathology specimens and scored the confidence of their diagnoses. Asbestos body count was correlated with CT and pathology scores. Pathologically, 15 cases were diagnosed as asbestosis and 18 cases with various lung fibroses other than asbestosis. On CT, only the score of the subpleural curvilinear lines was significantly higher in asbestosis (p = 0.03). Accuracy of CT diagnosis of asbestosis with a high confidence ranged from 0.73 to 0.79. Asbestos body count positively correlated with CT likelihood of asbestosis (r = 0.503, p = 0.003), and with the confidence level of pathological diagnosis (r = 0.637, p

Idiopathic pleuroparenchymal fibroelastosis is an acceptable entity among IIPs and not as rare as previously reported http://ow.ly/I5NEz

The International Classification of High-Resolution Computed Tomography (HRCT) for Occupational and Environmental Respiratory Diseases (ICOERD) is used to screen and diagnose respiratory illnesses. Using univariate and multivariate analysis, we investigated the relationship between subject characteristics and parenchymal abnormalities according to ICOERD, and the results of ventilatory function tests (VFT). Thirty-five patients with and 27 controls without mineral-dust exposure underwent VFT and HRCT. We recorded all subjects' occupational history for mineral dust exposure and smoking history. Experts independently assessed HRCT using the ICOERD parenchymal abnormalities (Items) grades for well-defined rounded opacities (RO), linear and/or irregular opacities (IR), and emphysema (EM). High-resolution computed tomography showed that 11 patients had RO; 15 patients, IR; and 19 patients, EM. According to the multiple regression model, age and height had significant associations with many indices ventilatory functions such as vital capacity, forced vital capacity, and forced expiratory volume in 1 s (FEV1). The EM summed grades on the upper, middle, and lower zones of the right and left lungs also had significant associations with FEV1 and the maximum mid-expiratory flow rate. The results suggest the ICOERD notation is adequate based on the good and significant multiple regression modeling of ventilatory function with the EM summed grades.

Introduction: Autoimmune pulmonary alveolar proteinosis (APAP), caused by macrophage dysfunction due to the anti-granulocyte-macrophage colony-stimulating factor autoantibody, is known to be complicated with opportunistic infection. We have already reported frequency of infection was 5.3% (Inoue Y, AJRCCM 2008). Subjects and Methods: Questionnaire about APAP cases complicated with infections, was mailed to 195 hospitals in Japan and was returned from 27 hospitals. Subjects of this study were APAP cases complicated with infections managed in the 27 hospitals. Pathogens, site of infection, and clinical course were examined. Results: Frequency of infection was 27 cases (7.2%) out of 375 APAP cases. Lung comorbidities were COPD (n=3), pneumoconiosis (n=1) and interstitial pneumonia (n=2), and three cases were treated with corticosteroids and/or immunosuppressant. Pathogen was bacteria (n=15), fungus (n=5), mycobacterium (n=6), Nocardia (n=2) and virus (n=1). Tuberculous lymphadenitis (n=2), meningeal cryptococcosis (n=1), aspergillus sinusitis (n=1), herpes simplex virus encephalitis (n=1) occurred out of the lung. Diagnosis of APAP preceded incidence of infection in 20 cases (74%). Disease severity of 6 cases (22.2%) improved after the occurrence of infection. Whole lung lavage could not be performed in 2 cases with infection, one case of which died with respiratory failure. Conclusions: Frequency of infection in APAP cases were similar to the previous report. Infection might improve or deteriorate the clinical course of APAP. Financial support: This study was partially granted from AMED; DLD/14526278 to Y. Inoue and T. Arai and PAP/14526182 to Y. Inoue.

Background Recently, we became to use two anti-fibrotic agents for treatment of idiopathic pulmonary fibrosis (IPF). Nintedanib and pirfenidone were each approved in Japan in Aug 2015 ans Dec 2008, respectively. Aim The aim of this study is to evaluate efficacy and safety of the two anti-fibrotic agents in real world cases treated in our institute. Methods: We reviewed medical records of IPF patients with Nintedanib group (N-group, n=43) and Pirfenidone group(P-group, n=133). We picked up findings of blood test, pulmonary function test(FVC, %DLco), adverse events(AEs), and outcome including survival and ocuurence of acute exacerbation(AEx). Data after treatment was collected within 52wks from baseline. We compared the decline of FVC before and after in N-group(n=27) and in P-group(n=67). We calculated the discontinuation time from AEs between two groups in GAP stageI-II using Kaplan-Meier analysis. Results Baseline characteristics (gender, age, FVC, and %DLco) and outcome(AEx and survival) were not significant between two groups. Patients number of GAP stage I-II in P-group(stageI-II/III 88/34) was significantly fewer than that of N-group(stageI-II/III 35/5)(p=0.0485). Duration of treatment was not significantly difference between two groups(N:31wks, P:35wks, p=0.3438). Decline of FVC after treatment was not significantly diffenrence between 2 groups(N:89ml/year, P:106ml/year, p=0.5011). Main AEs in N-group were diarrhea(47%), and liver disorder(37%). Avarage continuation time of two group was not significant (N:35wks, P:34wks, Log-rank test:p=0.4404). Conclusion Tolerability and safety of two anti-fibrotic agents in GAP stageI-II was not signifcantly difference in 52 wks.

Cryptogenic organizing pneumonia (COP) generally responds well to corticosteroids with a favorable outcome. Rare cases of organizing pneumonia are rapidly progressive. Yousem et al. studied pathologic predictors of idiopathic bronchiolitis obliterans organizing pneumonia/COP with an unfavorable prognosis. Beardsley and Rassl proposed the name fibrosing organizing pneumonia (FOP). A 74-year-old female non-smoker presented with a 2-week history of dry cough followed by dyspnea and a fever. The clinical course was fulminant, but we successfully performed bronchoscopy. After the diagnosis of FOP, we treated the patient with mechanical ventilation and high-doses of steroids/immunosuppressants, which improved the disease.

Objectives Evidence on the safety and long-term efficacy of super selective bronchial artery embolisation (ssBAE) using platinum coils in patients with haemoptysis is insufficient. The objective of the present study was to evaluate the safety and the 3-year postprocedure haemoptysis-free survival rate of de novo elective ssBAE using platinum coils rather than particles for the treatment of haemoptysis. Design A single-centre retrospective observational study. Setting Hemoptysis and Pulmonary Circulation Center in Japan. Participants A total of 489 consecutive patients with massive and non-massive haemoptysis who underwent de novo elective ssBAE without malignancy or haemodialysis. Interventions ssBAE using platinum coils. All patients underwent CT angiography before the procedure for identifying haemoptysis-related arteries (HRAs) and for procedural planning. Primary and secondary outcome measures The composite of the 3-year recurrence of haemoptysis and mortality from the day of the last ssBAE session. Each component of the primary end point and procedural success defined as successful embolisation of all target HRAs were also evaluated. Results The median patient age was 69 years, and 46.4% were men. The total number of target vessels was 4 (quartile 2–7), and the procedural success rate was 93.4%. There were 8 (1.6%) major complications: 1 aortic dissection, 2 symptomatic cerebellar infarctions and 5 mediastinal haematoma cases. The haemoptysis-free survival rates were estimated by the Kaplan-Meier analysis at 86.9% (95% CI 83.7% to 90.2%) at 1 year, 79.4% (74.8% to 84.3%) at 2 years and 57.6% (45.1% to 73.4%) at 3 years. Although not statistically significant by the adjusted analysis of variance with multiple imputation of missing variables, cryptogenic haemoptysis tended to show the most favourable outcome and non-tuberculous mycobacterium showed the worst outcome (adjusted p=0.250). Conclusions We demonstrated the safety and long-term efficacy of elective ssBAE using platinum coils and established that it can be a valuable therapeutic option for treating patients with haemoptysis.

Background Pulmonary nocardiosis is a rare but severe infection caused by Nocardia species. This study aimed at describing the clinical characteristics and prognosis of pulmonary nocardiosis. Methods An observational, retrospective study was undertaken of patients diagnosed with pulmonary nocardiosis over a 13-year period at the Kinki-Chuo Chest Medical Center, Osaka, Japan. Results Seven patients with airway nocardial colonization and 59 patients with pulmonary nocardiosis were identified, one of whom had disseminated nocardiosis. Patients with pulmonary nocardiosis were predominantly male patients (73%), with a mean age of 66 (range, 15–88) years. New-onset cough and dyspnea were the most common manifestations (76%). Although 52 (88%) patients had at least one underlying pulmonary disease, most patients did not appear to be systemically immunocompromised. The predominant abnormality on chest computed tomography in pulmonary nocardiosis was airspace consolidation (52%), sometimes associated with cavitation. Multivariate Cox proportional-hazards analysis revealed the following significant and independent risk factors for overall mortality: age >68 years (hazard ratio [HR], 4.7; 95% confidence interval [CI], 1.6–14; p =0.05), pulmonary aspergillosis (HR, 8.8; 95% CI, 2.4–33; p =0.01), and trimethoprim/sulfamethoxazole (TMP-SMZ) resistance (HR, 4.3; 95% CI, 1.6–11; p =0.04). Conclusions Clinicians should be aware that pulmonary nocardiosis can occur even in immunocompetent patients, especially those with an underlying pulmonary disease. In pulmonary nocardiosis, older age, pulmonary aspergillosis, and TMP-SMZ resistance are associated with increased risk of mortality.

Treatment of autoimmune pulmonary alveolar proteinosis (aPAP) by subcutaneous injection or inhaled therapy of granulocyte-macrophage colony-stimulating factor (GM-CSF) has been demonstrated to be safe and efficacious in several reports. However, some reports of subcutaneous injection described transient benefit in most instances. The durability of response to inhaled GM-CSF therapy is not well characterized.To elucidate the risk factors for recurrence of aPAP after GM-CSF inhalation, 35 patients were followed up, monitoring for the use of any additional PAP therapies and disease severity score every 6 months. Physiologic, serologic, and radiologic features of the patients were analyzed for the findings of 30-month observation after the end of inhalation therapy.During the observation, 23 patients remained free from additional treatments, and twelve patients required additional treatments. There were no significant differences in age, sex, symptoms, oxygenation indexes, or anti-GM-CSF antibody levels at the beginning of treatment between the two groups. Baseline vital capacity (% predicted, %VC) were higher among those who required additional treatment (P.01). Those patients not requiring additional treatment maintained the improved disease severity score initially achieved. A significant difference in the time to additional treatment between the high %VC group (%VC≥80.5) and the low %VC group was seen by a Kaplan-Meier analysis and a log-rank test (P.0005).These results demonstrate that inhaled GM-CSF therapy sustained remission of aPAP in more than one-half of cases, and baseline %VC might be a prognostic factor for disease recurrence.ISRCTN Register and JMACCT Clinical Trial Registry; No.: ISRCTN18931678 and JMAIIA00013; URL: http://www.isrctn.org and http://www.jmacct.med.or.jp.

Background Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease with a poor prognosis. Recently, pirfenidone was reported to slow the rate of decline in vital capacity and improve progression-free survival in IPF. The purpose of this study was to clarify the factors that predicted a good response to pirfenidone, as well as its adverse effects. Methods Forty-one IPF cases, treated with pirfenidone from January 2009 to January 2011, were enrolled in this investigation. Disease severity was classified into grades I–IV, as defined by the Japanese Respiratory Society (JRS). Short-term responsiveness to pirfenidone was evaluated by the modified criteria of the JRS. Predictors of nausea, anorexia, or both that represented important adverse effects were examined by multivariate Cox proportional hazard analyses. Predictors of short-time responsiveness were examined by multivariate logistic regression analyses. Results Diagnosed by a surgical lung biopsy (SLB), the mild cases of grade I/II were predictors of good, short-term responsiveness. Patients taking acid-secretion inhibitors, including proton pump inhibitors and histamine H2-receptor antagonists, showed less anorexia, nausea, or both. Only 1 case was administered drugs to activate gastrointestinal motility. Conclusions We concluded that IPF patients with a mild disease, diagnosis by SLB, or both showed indications of a good response to pirfenidone. In addition, acid-secretion inhibitors may reduce the frequency of anorexia, nausea, or both from pirfenidone.

BackgroundIn lymphangioleiomyomatosis (LAM), tuberous sclerosis gene mutations activate the mechanistic target of the rapamycin pathway, resulting in vascular endothelial growth factor-D (VEGF-D) overproduction. While the utility of serum VEGF-D testing for the diagnosis of LAM is outlined in ATS/JRS LAM Guidelines, the assay has not been fully validated for Asian populations. Our aims were to validate serum VEGF-D testing in Japan, by directly comparing measurements in Japan and the U.S., determining the diagnostic cut-off for serum VEGF-D levels among the Japanese women with typical thin walled cystic change on CT, and determining the performance of VEGF-D as a prognostic biomarker.Subjects and methodsWe determined serum VEGF-D levels from 108 LAM patients, 14 disease controls, and 51 healthy volunteers from the Japanese population. Measurements of 61 LAM patients were compared to those from the principal VEGF-D laboratory in the U.S at Cincinnati Children's Hospital Medical Center. We correlated baseline serum VEGF-D levels with baseline and longitudinal clinical data to determine how pregnancy, sirolimus or gonadotrophin-releasing hormone (GnRH) agonists influence serum VEGF-D levels.ResultsSerum VEGF-D measurements in Japan and the U.S. were very similar. Baseline serum VEGF-D levels effectively distinguished LAM from other diseases and healthy volunteers at a cut-off level of 645 pg/ml and were diagnostically specific at 800 pg/ml, consistent with the recommendations of the ATS/JRS LAM Guidelines. Baseline serum VEGF-D correlated negatively with the DLco baseline % predicted and with the annual decrease in DLco % predicted. There was no significant association between baseline serum VEGF-D level and the outcomes of death or transplant. Serum VEGF-D levels markedly decreased during treatment with sirolimus, but not with GnRH analogues. Serum VEGF-D levels of most LAM patients did not increase over time, and neither pregnancy nor menopause significantly modulated serum VEGF-D levels.ConclusionsSerum VEGF-D is a useful diagnostic and therapeutic biomarker for LAM. Satisfactory precision and international inter-laboratory agreement of the clinical assay support VEGF-D recommendations in the ATS/JRS LAM Guidelines for the Japanese population.

Background and Aims: Corticosteroids was not recommended for idiopathic pulmonary fibrosis (IPF) by IPF guideline (AJRCCM 2011). However, impact of corticosteroids on idiopathic interstitial pneumonias (IIPs) with possible usual interstitial pneumonia (UIP) HRCT pattern (IIPs/HRCT possible UIP) remains to be solved and this is the aims of this study. Subjects and Methods: Enrolled subjects of this trial were 98 cases with IIPs/HRCT possible UIP in our institute between 2005 and 2009: gender (M/F: 69/29), IPF/UIP (n=24) and Non-IPF (n=74), %FVC (median 80.6%) and %DLco (median 55.7%). Corticosteroids were introduced in 6 cases of IPF/UIP and 16 cases of Non-IPF. Impact of corticosteroids on IIPs/HRCT possible UIP was evaluated by Cox proportional hazard regression analysis. Results: Survival of IPF/UIP and Non-IPF was similar (p=0.886, Log-rank test). Survival of corticosteroids treated cases was significantly worse than that of non-treated cases both in IPF/UIP (p=0.045) and in Non-IPF (p=0.019) (Log-rank test); however, impact of corticosteroids on survival of IIPs/HRCT possible UIP was not significant after adjustment with significant prognostic factors in all cases; %FVC, %DLco and Male. Corticosteroids were not a significant predictor of AE in IPF/UIP (p=0.215), but significant in Non-IPF (p=0.007) by Log-rank test. However, impact of corticosteroids on incidence of AE was not significant after adjustment with predictors of AE in all cases; %DLco and %FVC. Conclusions: Corticosteroids did not alter the clinical course of IIPs/HRCT possible UIP. Acknowledgement: This study is partially supported by grant of National Hospital Organization and Japanese Ministry of Health Labour and Welfare.

In 2007, we reported a large cohort of autoimmune pulmonary alveolar proteinosis (n=248, PAP) in Japan, and showed the incidence and prevalence were 0.24 - 0.49/million/year and 2.04 - 6.2/million (AJRCCM 2007), and 1.4%, 1.4%, and 5.7% of patients had interstitial lung diseases (ILD), autoimmune diseases, and infections. The aims of this study are; (1) To establish a nationwide consecutive registration system for PAP, updating the incidence and prevalence, and (2) To show the usefulness of the system via a surveillance of intractable cases. Methods: (1) All patients, who received the serum anti-GM-CSF autoantibodies tests and were definitely diagnosed, were annually consecutively registered to our database (2011-2014). Three institutes (Niigata, Kinki-Chuo, Aichi) measured the antibodies. (2) Surveillance for the intractable cases (dead, complications such as ILD, infection, etc.) was conducted via the system. Results: (1) Total 903 cases were registered. 88 cases/year have been newly diagnosed in average. The incidence and the prevalence were 0.69/million/year and 6.11/million. 92.9, 6.1, 0.4, and 0.6% were autoimmune, secondary, congenital, and unclassifiable PAP. (2)50% of the institutes responded, providing the data of 579 cases (64% of total patients). 14, 11.2, 10.2, 3.8, 6.7, and 7.9% had dead, respiratory failure, ILD, autoimmune diseases, infections, extra-pulmonary complications. Conclusions: We established a simple consecutive nation-wide registration system for PAP in Japan, providing updated annual epidemiological data and effective surveillance data. This study was granted from AMED (#15652554 , YI).

Intramedullary spinal cord metastasis of non-small cell lung cancer is rare, and it has a short prognosis. We report a 53-year-old man diagnosed with cT4N0M0, stage IIIA squamous cell lung cancer. Ten months after left pneumonectomy (pT4N0M0), an intramedullary spinal cord tumor developed at the axis level. The intramedullary spinal cord tumor was resected, and he was diagnosed with metastatic squamous cell lung cancer. Radiotherapies and another tumor resection were conducted, as he had a good performance status and the discrete lesion was associated with the risk of brain stem compression. Multimodal local treatments for intramedullary spinal cord metastasis caused the tumor to shrink, and he lived for 25 months after the spinal metastasis occurred.

Background: The prognosis of combined cases of pulmonary fibrosis and emphysema is unresolved partially because radiological differentiation between usual interstitial pneumonia and nonspecific interstitial pneumonia is difficult in coexisting emphysema cases. Objective: The purpose of this study was to clarify the clinical impact of emphysema on the survival of patients with idiopathic pulmonary fibrosis (IPF). Methods: One hundred and seven patients with interstitial lung diseases were diagnosed by surgical lung biopsies between 2006 and 2012, and 47 patients were diagnosed with IPF through multidisciplinary discussion. Emphysema on high-resolution computed tomography scans was evaluated semiquantitatively by visual scoring. Results: Eight out of the 47 IPF patients showed a higher emphysema score (>3) and were diagnosed to have IPF-emphysema. The median survival time of patients with IPF-emphysema (1,734 days) from the initial diagnosis was significantly shorter than that of patients with IPF alone (2,229 days) by Kaplan-Meier analysis (p = 0.007, log-rank test). Univariate Cox proportional hazard regression analyses revealed that a higher total emphysema score (>3.0) was a significantly poor prognostic factor in addition to Krebs von den Lungen-6, surfactant protein-D, arterial oxygen tension, percent forced vital capacity, and percent diffusing capacity of carbon monoxide (%DLCO). Multivariate Cox proportional hazard regression analyses with the stepwise method showed that higher total emphysema score (>3) and %DLCO were significantly poor prognostic factors. Conclusions: The prognosis of IPF-emphysema was significantly worse than that of IPF alone.

To determine whether the HRCT findings are useful to differentiate asbestosis from idiopathic pulmonary fibrosis (IPF).We assessed HRCT scans from patients with asbestosis (n = 96) and IPF (n = 65). The frequencies and extent of parenchymal abnormalities and the frequencies of pleural changes were evaluated by consensus of two chest radiologists.There was a significant difference between IPF and asbestosis in pleural changes. In addition, there were significant differences between IPF and asbestosis in several parenchymal abnormalities on CT, especially in the less advanced stage of both diseases. On multivariate analysis, HRCT features that distinguished asbestosis from IPF were subpleural lines at a distance of less than 5 mm from the inner chest wall, subpleural dots and parenchymal bands.There are significant differences between IPF and asbestosis in the parenchymal and pleural abnormalities on CT.

The 60-film set was developed by experts (Expert Group) for examining 8 indices: sensitivity (X(1)) and specificity (X(2)) for pneumoconiosis, sensitivity(X(3)) and specificity for (X(4)) large opacities, sensitivity (X(5)) and specificity (X(6)) for pleural plaque, profusion increment consistency for small opacities (X(7)), and shape differentiation for small opacities (X(8)) of physicians' reading skills on pneumoconiosis X-ray according to ILO 2000 Classification. The aim of this study was to assess the appropriateness of the exam film set for evaluating physicians' reading skills. 29 physicians (A1-Group) and 24 physicians (A2-Group) attended the 1st and 2nd "Asian Intensive Reader of Pneumoconioses" (AIR Pneumo) training course, respectively, and 22 physicians (B-Group) attended Brazilian training course. After training, they took examination of reading 60-film exam set. The examinees' reading results in terms of 8 indices were compared between the examinee groups and the Expert Group by parametric unpaired t-test. The Examinee Group consisting of A1-Group, A2-Group and B-Group was inferior to the Expert Group in all indices. There was no significant difference for X(7) of A1-Group, X(7) and X(8) of A2-Group (p0.05) compared with the Expert Group. There was a significant difference in X(8) at p0.05 between A1-Group and A2-Group, in X(3) at p0.05 between A1-Group and B-Group, in both X(1) and in X(3) at p0.05 between A2-Group and B-Group. Accordingly, the 60-film set providing 8 indices designed might be a good method for evaluation of the physicians' reading proficiency at different training settings.

Rationale: Inhaled granulocyte/macrophage–colony stimulating factor (GM-CSF) is a promising therapy for pulmonary alveolar proteinosis (PAP) but has not been adequately studied. Objectives: To evaluate safety and efficacy of inhaled GM-CSF in patients with unremitting or progressive PAP. Methods: We conducted a national, multicenter, self-controlled, phase II trial at nine pulmonary centers throughout Japan. Patients who had lung biopsy or cytology findings diagnostic of PAP, an elevated serum GM-CSF antibody level, and a PaO2 of less than 75 mm Hg entered a 12-week observation period. Those who improved (i.e., alveolar–arterial oxygen difference [A–aDO2] decreased by 10 mm Hg) during observation were excluded. The rest entered sequential periods of high-dose therapy (250 μg Days 1–8, none Days 9–14; × six cycles; 12 wk); low-dose therapy (125 μg Days 1–4, none Days 5–14; × six cycles; 12 wk), and follow-up (52 wk). Measurements and Main Results: Fifty patients with PAP were enrolled in the study. During observation, nine improved and two withdrew; all of these were excluded. Of 35 patients completing the high- and low-dose therapy, 24 improved, resulting in an overall response rate of 62% (24/39; intention-to-treat analysis) and reduction in A–aDO2 of 12.3 mm Hg (95% confidence interval, 8.4–16.2; n = 35, P < 0.001). No serious adverse events occurred, and serum GM-CSF autoantibody levels were unchanged. A treatment-emergent correlation occurred between A–aDO2 and diffusing capacity of the lung, and high-resolution CT revealed improvement of ground-glass opacity. Twenty-nine of 35 patients remained stable without further therapy for 1 year. Conclusions: Inhaled GM-CSF therapy is safe, effective, and provides a sustained therapeutic effect in autoimmune PAP. Clinical trial registered with www.controlled-trials.com/isrctn (ISRCTN18931678), www.jmacct.med.or.jp/english (JMA-IIA00013).

Acquired pulmonary alveolar proteinosis (PAP) has been reclassified into autoimmune or secondary PAP according to the occurrence of serum granulocyte macrophage colony-stimulating factor autoantibody. Most patients undergo high-resolution CT (HRCT) scanning in order for physicians to make a differential diagnosis of diffuse lung diseases, but no information is available to distinguish the HRCT scan features of secondary PAP from those of autoimmune PAP. The objective of this study was to characterize the HRCT scan features of autoimmune and secondary PAP.HRCT scans of 42 patients (21 patients each in the autoimmune PAP and secondary PAP groups) were centrally collected and evaluated in a blinded manner.Ground-glass opacities (GGO) were a major finding in both the autoimmune PAP and secondary PAP groups. In the secondary PAP group, GGOs typically showed a diffuse pattern (62%), whereas GGOs showed a patchy geographic pattern in the autoimmune PAP group (71%; p0.005). The so-called "crazy-paving" appearance and subpleural sparing were frequently seen in the autoimmune PAP group (both 71%), whereas they were less frequently seen in the secondary PAP group (14% and 33%, respectively). The involved area of GGO was even in craniocaudal distribution for the secondary PAP group, whereas it was predominant in the lower lung field compared with the upper lung field in the autoimmune PAP group (p0.05).Typical HRCT scan findings for autoimmune PAP patients were GGO with a patchy geographic pattern, subpleural sparing, crazy-paving appearance, and predominance in the lower lung field. These findings were rather infrequent for secondary PAP patients.

Purpose: We have developed a classification of high-resolution computed tomography (HRCT) images for screening, surveillance and epidemiological studies of respiratory diseases caused by occupational and environmental factors. The proposed classification consists of three parts: a guideline explaining the elements of the classification scheme, a reading sheet, and reference films to aid in assessing thin-section CT films. We assessed the reliability of the proposed classification system by blinded, independent trial reading. Materials and Methods: Seven independent radiologists and pulmonologists performed a trial reading to measure the reliability of the classification system using HRCT films from 27 pneumoconiosis patients and 7 normal controls. Results: The agreement was moderate to good for rounded opacities (weighted κ=0.68 and 0.64), irregular opacities (0.59, 0.48), honeycombing (0.65, 0.47), emphysema (0.76, 0.62) and large opacities (0.48, 0.52). Ground glass opacities (0.16, 0.20) showed poor to fair agreement. Intra-reader agreement of each of the seven readers was moderate to good (mean: weighted κ=0.52-0.80) for parenchymal findings, but the agreement was relatively low (mean weighted κ=0.52) for ground glass opacities. Conclusion: The proposed classification is able to describe early dust-related fibrotic changes and provide a semi-quantitative description of the HRCT features of major fibrotic changes in the parenchyma and pleura. Reliability, as measured by inter-reader agreement, was satisfactory.

The purpose of this study was to determine whether measurements of lung attenuation at inspiration and expiration obtained from 3D lung reconstructions reflect the severity of chronic obstructive pulmonary disease.Seventy-six patients with chronic obstructive pulmonary disease underwent MDCT with 3D postprocessing at full inspiration and full expiration. Inspiratory and expiratory mean lung density, percentage of lung volume with attenuation values less than -910 HU and -950 HU at inspiration and expiration, expiratory to inspiratory mean lung density ratio, and fifth and 15th percentiles of the lung attenuation distribution curve at inspiration and expiration were measured.When forced expiratory volume in the first second of expiration (FEV(1)) was 50% or greater than predicted value, mean lung density and lower attenuation volume measured from inspiratory MDCT scans correlated better with FEV(1) and ratio of FEV(1) to forced vital capacity (FVC) than did those from expiratory scans. When FEV(1) was less than 50% of predicted value, mean lung density and lower attenuation volume measured from expiratory MDCT scans correlated better with FEV(1) and ratio of residual volume to total lung capacity than did those values from inspiratory scans. Fifth percentile and 15th percentile of the lung attenuation distribution curve at both full inspiration and full expiration correlated well with FEV(1)/FVC and diffusing capacity of the lung for carbon monoxide as a percentage of predicted value but not well with FEV(1) as a percentage of predicted value regardless of FEV(1).Measurements of lung attenuation obtained at inspiration and visual score better reflect abnormal results of pulmonary function tests in patients with less severe chronic obstructive pulmonary disease than do measurements obtained at expiration. Measurements of lung attenuation obtained at expiration better reflect pulmonary function abnormalities in patients with severe chronic obstructive pulmonary disease.

OBJECTIVE. The purpose of this study was to evaluate the radiographic and CT findings of inhalational talc pneumoconiosis.CONCLUSION. Large opacities of talc pneumoconiosis progress more often than do small opacities. The CT findings of talc pneumoconiosis overlap those of silicosis and asbestosis.

Introduction: Rapid improvement of clinical findings is observed after the treatment with corticosteroid, but relapse commonly occur in cryptogenic organizing pneumonia (COP). Objective: We attempt to reveal the clinical factors to predict recurrence of COP. Subjects and Methods: The pathology files of our institute from 2007 to 2013 were retrospectively reviewed and 301 patients with biopsy-proven organizing pneumonia (OP) were selected. Secondary OP was excluded and 92 patients were diagnosed with COP. We collected laboratory data including C-reactive protein (CRP), Krebs von den Lungen (KL)-6, surfactant protein (SP)-D, arterial blood gas analysis and pulmonary function tests. Clinical factors were compared between relapsed cases and non-relapsed cases by Fisher9s exact test or Wilcoxon rank sum test, and its predictive significance of recurrence was evaluated by Log-rank test. Results: The median age was 68.2 years and 65 cases were male. Median observation period was 434 days. Corticosteroids and/or immunosuppressant were administered to 34 patients. Relapse of COP was observed in 14 cases. Median duration until the first recurrence was 289 days. Serum CRP was significantly elevated in relapsed cases than non-relapsed cases. There was no significant difference in serum KL-6 and SP-D between two groups. Relapsed cases needed treatment significantly more frequently than non-relapsed cases (p=0.006). Risk of relapse was significantly higher in treated cases (p=0.017) and cases with higher serum CRP (p=0.0006). Conclusion: Necessity of treatment and high serum CRP are significant predictive factors of recurrence in COP patients. (This study is partially supported by Grant from National Hospital Organization).

Introduction: Lymphangioleiomyomatosis (LAM) is a rare multisystem disease, caused by the proliferation of LAM cells leading to destruction and remodeling of the tissues. Renin–angiotensin system and mast cells have been reported to function on cell proliferation and tissue remodeling in LAM (AJRCMB 2006, AJRCCM 2002, Respir Res 2012). Aim of this study is to clarify the role of measurement of serum angiotensin-converting enzyme (ACE) activity as a biomarker for LAM. Subjects and methods: 102 patients of LAM including 15 tuberlous sclerosis complex (TSC)-LAM, 37 healthy volunteers (HV) and 16 other lung disease controls (OLD) were enrolled. Serum ACE activities were measured by Kasahara9s method. Serum VEGF-D levels and clinical measures were compared with serum ACE. Results: Serum ACE significantly elevated in LAM {14.6 (11.7-19.1) median (IQR) IU/L } compared in HV {10.5 (7.6-12.7) IU/L } or OLD {9.9 (7.4-11.6) IU/L } (p Discussion and conclusion: Serum ACE significantly increased in LAM, which was associated with lymphangioleiomyomas and serum VEGF-D, suggesting lymphatic abnormalities. Serum ACE may have accessory role in LAM which differ from VEGF-D. This study was partially supported by the grant from JMHLW (YI).

Acute exacerbations (AEs) of idiopathic pulmonary fibrosis (IPF) and other idiopathic interstitial pneumonia (IIP) have a poor prognosis. This study aims to clarify the incidence and prognosis of AE in IPF and the other IIP.A total of 229 patients were enrolled, of whom 92 had IPF and 137 had 'IIP other than IPF' based on the American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association (ATS/ERS/JRS/ALAT) 2011 IPF Guidelines. IIP other than IPF included 11 patients with a surgical lung biopsy (SLB) and the remainder without such a biopsy. IIP other than IPF was further classified into IIP with a 'possible usual interstitial pneumonia (UIP)' pattern on HRCT (n = 75) and IIP with 'inconsistent with UIP' pattern (n = 62) based on published guidelines. Predictors of AE and the prognosis after AE were examined in these groups.The 1-year incidence of AE in IPF, IIP with possible UIP HRCT patterns and IIP with inconsistent with UIP HRCT patterns was 16.5%, 8.9% and 4.0%, respectively. AE occurred significantly more frequently in IPF than in IIP with possible UIP and inconsistent with UIP HRCT patterns after adjustment for BMI, modified Medical Research Council score and %forced vital capacity. Prognosis of AE-IIP with possible UIP HRCT pattern was significantly worse than that of AE-IPF.Although AE occurred significantly less frequently in IIP with possible UIP and inconsistent with UIP HRCT patterns than in IPF, the prognosis of AE-IIP with possible UIP HRCT patterns might be worse than that of AE-IPF.

Objective To reevaluate idiopathic pulmonary fibrosis (IPF) cases which had surgical lung biopsy (SLB) for diagnosis of usual interstitial pneumonia (UIP), and examine the influence of computed tomography (CT) findings and clinical information based on diagnostic certainty. Methods Ninety-five cases with multidisciplinary diagnoses of IPF were identified from eight institutions. All cases had SLB. Two expert chest radiologists and five expert pulmonologists used a 5-point scale to grade their level of certainty in the diagnosis of a radiological pattern of UIP or a clinical diagnosis of IPF (level 1 "definitely no" to level 5 "definitely yes"). Radiologists independently evaluated thin-section CT images and pulmonologists independently assessed clinical information. The two groups then discussed their diagnosis to obtain a final consensus, and listed alternative diagnoses. Changes in the level of certainty during the diagnostic process were investigated. Results The level of certainty for IPF was judged as low (level 1 or 2) in 32 cases (34%) by radiologists and in three cases (3%) by pulmonologists; in the final consensus 39 cases (41%) were judged as low. Chronic hypersensitivity pneumonitis (CHP), interstitial pneumonia associated with collagen tissue diseases (CTD-IP), and idiopathic nonspecific interstitial pneumonia (idiopathic NSIP) were listed as alternative diagnoses. Conclusions In this retrospective series, some cases that had UIP confirmed on SLB for IPF diagnosis were classified into a low-level certainty group by expert chest radiologists and pulmonologists. When a diagnosis of IPF is made, the possibility of CHP, CTD-IP, and idiopathic NSIP must be also considered.

The International Classification of High-resolution Computed Tomography (HRCT) for Occupational and Environmental Respiratory Diseases (ICOERD) has been developed for the screening, diagnosis, and epidemiological reporting of respiratory diseases caused by occupational hazards. This study aimed to establish a correlation between readings of HRCT (according to the ICOERD) and those of chest radiography (CXR) pneumoconiotic parenchymal opacities (according to the International Labor Organization Classification/International Classification of Radiographs of Pneumoconioses [ILO/ICRP]). Forty-six patients with and 28 controls without mineral dust exposure underwent posterior-anterior CXR and HRCT. We recorded all subjects' exposure and smoking history. Experts independently read CXRs (using ILO/ICRP). Experts independently assessed HRCT using the ICOERD parenchymal abnormalities grades for well-defined rounded opacities (RO), linear and/or irregular opacities (IR), and emphysema (EM). The correlation between the ICOERD summed grades and ILO/ICRP profusions was evaluated using Spearman's rank-order correlation. Twenty-three patients had small opacities on CXR. HRCT showed that 21 patients had RO; 20 patients, IR opacities; and 23 patients, EM. The correlation between ILO/ICRP profusions and the ICOERD grades was 0.844 for rounded opacities (p

Summer-type hypersensitivity pneumonitis includes a spectrum of granulomatous lung diseases that result from the inhalation of Trichosporon species in the summer. Hot tub lung is a granulomatous lung disease caused by the inhalation of water aerosols containing non-tuberculous mycobacteria. We herein describe a case of hot tub lung that deteriorated during the winter season. Every winter, the patient's symptoms, laboratory findings and chest images worsened. Genetically identical Mycobacterium avium strains were detected in his sputum and bathtub. The circulation of bathtub water during the winter months only exacerbated his symptoms in the winter.

Correct diagnosis of diffuse cystic lung diseases is established in most cases by critical review of HRCT features http://ow.ly/NvrCc

OBJECTIVE. The purpose of this study was to evaluate the high-resolution CT findings of drug-induced eosinophilic pneumonias.CONCLUSION. Drug-induced eosinophilic pneumonias usually manifest as areas of consolidation and ground-glass opacity most commonly involving the outer third of the lungs.

A 52-year-old Japanese man with congenital amblyopia and oculocutaneous albinism was admitted to our hospital. Chest CT showed reticular opacities and traction bronchiectasis without honeycombing. Specimens obtained by a video-assisted thoracoscopic surgery showed patchy chronic fibrotic lesions. We diagnosed him with Hermansky-Pudlak syndrome (HPS). A mutation in the HPS1 gene was detected, and the diagnosis was confirmed. The patient was treated with prednisolone, pirfenidone, and azathioprine, but he nevertheless died within four months. Autopsy lung specimens showed diffuse alveolar damage suggesting comparatively rapid deterioration, although this presentation was not typical of an acute exacerbation. These pathological changes may be a possible progression pattern in HPS patients.

We report a case of serious complications following computed tomography (CT)-guided radiofrequency ablation (RFA) performed for the treatment of lung cancer with interstitial pneumonia. The patient developed delayed-onset pyopneumothorax, which required 6 months of antibiotic treatment, drainage, and video-assisted thoracoscopic debridement. Although CT-guided RFA is a promising, effective procedure for difficult-to-treat lung cancer, the present case suggests a risk of complications for patients complicated with interstitial pneumonia and warrants caution.

This study compared high-resolution computed tomography (CT) findings between 10 survivors and 21 nonsurvivors of acute interstitial pneumonia and evaluated whether the CT findings were predictive of patients' response to treatment. The survivor and nonsurvivor groups with pathologically or clinically diagnosed acute interstitial pneumonia were similar in age, sex, disease duration, and lung injury score. Retrospective, subjective evaluations of the CT scans were conducted by two independent observers without knowledge of patient outcomes. CT findings were graded on a one to six scale corresponding to consecutive pathologic phases as follows: areas of (1) normal attenuation, (2) ground-glass attenuation, (3) consolidation, (4) ground-glass attenuation associated with traction bronchiolectasis or bronchiectasis, (5) consolidation associated with traction bronchiolectasis or bronchiectasis, and (6) honeycombing. An overall score was obtained by quantifying the extent of each abnormality in three lung zones in each lung. The extent of ground-glass attenuation or consolidation associated with traction bronchiolectasis or bronchiectasis was less in survivors than nonsurvivors (p = 0.004 and p = 0.009, respectively). Architectural distortion was less frequent, and ground-glass attenuation or consolidation without traction bronchiolectasis or bronchiectasis was more extensive in survivors than in nonsurvivors (p = 0.007, p = 0.002, and p = 0.029, respectively). Overall CT scores of survivors were significantly lower than those of nonsurvivors (p = 0.0003). A CT score of less than 245 had an 80% positive and a 90% negative predictive value for survival. There was good interobserver agreement in the assessment of the CT findings (Kappa 0.75). The results indicate that CT assessment is potentially helpful in predicting patient prognosis in acute interstitial pneumonia regardless of the degree of physiologic abnormality.

The most common of the pneumoconioses are silicosis, CWP, and asbestosis. The former two are characterized by the presence of small nodular opacities predominantly distributed in the upper zones of the lung. The small nodular opacities are classified into two patterns on HRCT: (1) ill-defined fine branching lines and (2) well-defined discrete nodules. Asbestosis demonstrates thickened interlobular and intralobular lines, subpleural dot-like or curvilinear opacities, and honeycombing on HRCT, predominantly distributed in the bases of the lungs. Although HRCT findings of other pneumoconioses are variable and nonspecific, there are predominant and characteristic findings for each type of pneumoconiosis. HRCT is useful in achieving more accurate categorization of the parenchymal changes in each type of pneumoconiosis.

Lung injury caused by chemicals includes bronchitis, bronchiolitis, chemical pneumonitis, pulmonary edema, acute respiratory distress syndrome, organizing pneumonia, hypersensitivity pneumonitis, acute eosinophilic pneumonia, and sarcoid-like granulomatous lung disease. Each chemical induces variable pathophysiology and the situation resembles to the drug induced lung disease. The HRCT features are variable and nonspecific, however HRCT may be useful in the evaluation of the lung injuries and so we should know about HRCT features of lung parenchymal abnormalities caused by chemicals.

AIM: The purpose of this study was to describe the clinical and high-resolution computed tomographic (HRCT) findings in patients with pulmonary tuberculosis who developed respiratory failure after starting chemotherapy. MATERIALS AND METHODS: The clinical records, chest radiographs, and HRCT findings in five patients with non-miliary pulmonary tuberculosis who developed respiratory failure after starting chemotherapy were reviewed. RESULTS: Chest radiographs taken early in the course of acute respiratory failure showed progression of the original lesions with ( n =4) or without ( n =1) new areas of opacity away from the site of the original lesions. HRCT demonstrated widespread ground-glass attenuation with a reticular pattern as well as segmental or lobar consolidation with cavitation and nodules, consistent with active tuberculous foci in all five cases. Prominent interlobular septal thickening was seen in two cases. Four of the five patients had received corticosteroids. Of these five, two died and three recovered with continued corticosteroid therapy. Transbronchial biopsy in three cases showed evidence of acute alveolar damage. CONCLUSION: In selected patients with tuberculosis who develop respiratory failure following the initiation of antituberculous therapy, HRCT may be a helpful adjunct to clinical evaluation in differentiating hypersensitivity reactions (presumed to be due to the release of mycobacterial antigens) from other pulmonary complications. Akira, M. and Sakatani, M . (2001) Clinical Radiology 56 , 550–555.

The Japanese Classification of Computed Tomography for Pneumoconioses with Standard Films: Comparison with the ILO International Classification of Radiographs for Pneumoconioses: Narufumi SUGANUMA, et al. Department of Environmental Health, School of Medicine, Fukui Medical University—Objective: Computed tomography (CT) has recently come to be used for personal diagnosis of pneumoconioses and preliminarily for epidemiological purposes. This study aimed to compare the diagnosis of pneumoconioses by the Japanese Classification of CT for Pneumoconioses (Hosoda-Shida Classification) with that by the ILO International Classification of Radiographs of Pneumoconioses (ILO 1980 standard). The Hosoda-Shida Classification is also described in this article. Subjects and Methods: CT and chest posterior-anterior X-ray (CXR) were performed in 21 subjects with an occupational history of mining, and 6 subject without exposure to any risk of pneumoconiosis. Three radiologists independently described the findings of CT and CXR according to both the Hosoda-Shida Classification and the ILO 1980 standard, respectively. Results: At least two of the three readers agreed in determining both the profusion and the type of small rounded opacities in 96% (26/27) of the CT films. The inter-reader agreement of profusion was satisfactory with the Cohen's weighted kappa value of 0.57 to 0.71. The weighted kappa for CXR and CT in describing the profusion and the type of small rounded opacities were 0.70 and 0.77, respectively. Conclusion: The Hosoda-Shida Classification for pneumoconioses is shown to be reliable and compatible with the ILO 1980 standard in describing the profusion and the type of small opacities. (J Occup Health 2001; 43: 24-31)

Background Collagen vascular disease-associated interstitial lung disease (CVD-ILD) must be differentiated from idiopathic pulmonary fibrosis (IPF) since prognosis and treatment strategies differ between these two conditions. However, differentiating between CVD-ILD and IPF is often difficult. Purpose To examine the utility of expiratory high-resolution computed tomography (HRCT) for differentiating between CVD-ILD and IPF. Material and Methods Seventy patients were examined with expiratory and inspiratory HRCT with CVD-ILD ( n = 36) or IPF ( n = 34). Associated diagnoses in patients with CVD-ILD were rheumatoid arthritis ( n = 22), Sjögren syndrome ( n = 3), scleroderma ( n = 2), polymyositis/dermatomyositis ( n = 1), and unspecified connective tissue disease ( n = 8). Parenchymal abnormalities on inspiratory HRCT and visual extent of air trapping on expiratory HRCT were evaluated, statistical differences in HRCT findings between the two conditions were determined, and air trapping CT scores were correlated with the results of pulmonary function testing. Results Air trapping was found in 27 (75%) of 36 cases of CVD-ILD and four (12%) of 34 cases of IPF. Seventeen of the 27 cases for which air trapping was exhibited with CVD-ILD were diagnosed with rheumatoid arthritis. A significant difference in frequency of air trapping was seen between CVD-ILD and IPF ( P Conclusion The presence of air trapping on expiratory HRCT suggests CVD-ILD rather than IPF.

To determine whether various eosinophilic lung diseases can be differentiated by means of thin-section computed tomography (CT).Thin-section CT scans in 111 patients with eosinophilic lung diseases-40 with chronic eosinophilic pneumonia, 16 with Churg-Strauss syndrome, 16 with allergic bronchopulmonary aspergillosis (ABPA), 13 with acute eosinophilic pneumonia, 12 with simple pulmonary eosinophilia, 11 with drug-induced eosinophilic pneumonia, and three with hypereosinophilic syndrome-were assessed independently by two observers. The observers recorded the abnormalities, diagnosis, and degree of confidence in the diagnosis.The two observers made a correct first-choice diagnosis on average in 61% of readings. The correct diagnosis was made in 78% of cases of chronic eosinophilic pneumonia; 81%, acute eosinophilic pneumonia; 44%, Churg-Strauss syndrome; 84%, ABPA; 17%, simple pulmonary eosinophilia; 27%, drug-induced eosinophilic pneumonia; and 33%, hypereosinophilic syndrome. The two observers made a correct diagnosis with a high degree of confidence in 36% of readings. There was moderate agreement between the observers for the correct diagnosis (kappa, 0.47) and for the correct diagnosis with a high degree of confidence (kappa, 0.59).Although eosinophilic lung diseases often can be differentiated by means of thin-section CT, correlation between CT findings and careful clinical evaluation are required for a definitive diagnosis.

The purpose of this study was to determine whether acute parenchymal lung diseases can be differentiated on the basis of the pattern and distribution of abnormalities revealed on high-resolution CT.High-resolution CT scans of 90 patients with acute parenchymal lung diseases (19 with bacterial pneumonia, 13 with mycoplasmal pneumonia, 21 with acute interstitial pneumonia, 18 with hypersensitivity pneumonitis, 10 with acute eosinophilic pneumonia, and nine with pulmonary hemorrhage) were independently assessed by two observers who had no knowledge of clinical or pathologic data. The observers recorded abnormalities, their first-choice diagnosis, and their degree of confidence in their first-choice diagnosis.The two observers made a correct first-choice diagnosis in an average of 55 (61%) of 90 cases. Correct first-choice diagnosis was made in 50% of cases of bacterial pneumonia, 62% of mycoplasmal pneumonia, 90% of acute interstitial pneumonia, 72% of hypersensitivity pneumonitis, 30% of acute eosinophilic pneumonia, and 28% of pulmonary hemorrhage. CT findings allowed distinction between infectious and noninfectious causes in 81 (90%) of 90 cases.High-resolution CT is helpful in the differential diagnosis of infectious from noninfectious acute parenchymal lung disease. However, high-resolution CT is of limited value in making a specific diagnosis.

Purpose The purpose of this study was to describe the long-term follow-up CT evaluation in rheumatoid arthritis (RA)-associated lung disease. Method Thin-section CT scans from 29 patients with RA and suspected associated lung disease were reviewed. Twenty-two patients underwent sequential CT evaluation during 3 to 108 months of follow-up (mean 28 months). Histologic comfirmation of pulmonary involvement was available in 19 patients. Results Three major patterns were identified: reticulation with or without honey-combing (n = 19), centrilobular branching lines with or without bronchial dilatation (n = 5), and consolidation (n = 5). Reticulation and centrilobular branching lines corresponded to usual interstitial pneumonia (n = 14) and bronchiolitis obliterans (n = 1), respectively. Consolidation corresponded to bronchiolitis obliterans organizing pneumonia (BOOP; n = 3) and coexistent chronic eosinophilic pneumonia (CEP) and BOOP (n = 1). Patients with reticulation had rapid deterioration when there was new appearance of multifocal areas of ground-glass attenuation. Centrilobular branching lines progressed to bronchiectasis in one case. There was mild progression of existing bronchiectasis associated with centrilobular branching lines in one case. Area of consolidation in two patients with BOOP and one with coexistent CEP and BOOP evolved into honeycombing at serial CT. Conclusion Thin-section CT is a noninvasive technique for monitoring disease morphology in RA-associated lung disease. Initial CT findings and their evolution on sequential examinations may be useful in evaluating prognosis.

OBJECTIVE: The purpose of this study was to correlate thin-section CT findings with histologic findings for the three histopathologic subtypes of localized bronchioloalveolar carcinoma. CONCLUSION: Thin-section CT can be used to predict the histologic subtype of bronchioloalveolar carcinoma.

To determine whether idiopathic interstitial pneumonias can be differentiated on the basis of the pattern and distribution of abnormalities at thin-section computed tomography (CT).Thin-section CT scans in 129 patients with histologically proved idiopathic interstitial pneumonia (35 with usual interstitial pneumonia [UIP], 24 with bronchiolitis obliterans organizing pneumonia [BOOP], 23 with desquamative interstitial pneumonia [DIP], 20 with acute interstitial pneumonia [AIP], and 27 with nonspecific interstitial pneumonia and fibrosis [NIPF]) were independently assessed by two observers without knowledge of clinical or histologic data. The observers recorded the abnormalities, diagnosis, and degree of confidence in their diagnosis. Differential diagnosis was limited to the five types of idiopathic interstitial pneumonia.The two observers made a correct diagnosis, on average, in 74 (57%) cases. On average, the correct diagnosis was made in 25 (71%) cases of UIP, 19 (79%) of BOOP, 14.5 (63%) of DIP, 13 (65%) of AIP, and 2.5 (9%) of NIPF. The two observers made a correct diagnosis with a high degree of confidence in 50 (39%) readings. There was moderate agreement between the observers for the correct diagnosis (k = 0.55) and for the correct diagnosis with a high degree of confidence (k = 0.65).Except for NIPF, the various subtypes of idiopathic interstitial pneumonias often have a characteristic appearance that allows differentiation at thin-section CT.

This study describes the computed tomography features of various fulminant forms of idiopathic interstitial pneumonia and to clarify the usefulness of computed tomography in such patients. Computed tomography scans in 19 patients with fulminant forms of idiopathic interstitial pneumonia were reviewed. This study included patients with acute interstitial pneumonia (n = 7), an accelerated form of idiopathic pulmonary fibrosis (n = 2), and an acute exacerbation of idiopathic pulmonary fibrosis (n = 10). Pathologic confirmation of the diagnosis was obtained in all patients. Follow-up computed tomography scans were available for eight patients, and postmortem computed tomography scans were available for three patients. All patients had progressive ground-glass attenuation, consolidation, or both. In patients with an acute exacerbation of idiopathic pulmonary fibrosis, subpleural honeycombing was also seen. Follow-up computed tomography showed a change from ground-glass attenuation to consolidation with distortion. Architectural distortion, traction bronchiectasis, and ground-glass opacity were the prominent features in the initial computed tomography scans obtained more than 7 days after the onset of symptoms, and cystic lesions were seen in follow-up computed tomography obtained more than 1 month after the onset. High-resolution computed tomography was more sensitive than the plain radiographs in the early detection of these entities. Computed tomography examination at the onset of the acute symptoms is useful in classifying these fulminant forms of idiopathic interstitial pneumonia.