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Airway-centered fibroelastosis is a distinct entity characterized by prominent airway-centered elastosis of the upper lobe with little or no pleural involvement. Little is known regarding its etiology; however, it was reported to have an idiopathic or asthma-associated etiology. We document, for the first time, 2 women (19 and 60 years old) who developed pleuroparenchymal fibroelastosis with a predominantly airway-centered distribution as a late complication (6 and 9 years later, respectively) of chemotherapy. The disease rapidly progressed following the manifestation of symptoms, and they subsequently died (3 and 2 years later, respectively). Therefore, post-chemotherapy long-term monitoring for this disease is warranted.

Autoimmune pulmonary alveolar proteinosis (APAP) is caused by macrophage dysfunction due to anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibody. We experienced 2 cases of APAP complicated with sarcoidosis in a 42-year-old woman and a 51-year-old man (age at the sarcoidosis diagnosis). APAP preceded sarcoidosis in the woman, and both diseases were diagnosed simultaneously in the man. Sarcoidosis lesions were observed in the lung, skin, and eyes, and the pathological findings of APAP were not marked at the diagnosis of sarcoidosis in either case. Low-grade positive serum anti-GM-CSF autoantibody was suspected to be correlated with the occurrence of sarcoidosis and resolution of APAP.

Background The clinical characteristics and prognostic impact of complication with pneumomediastinum in patients with interstitial pneumonias (IPs) are not well studied due to the relatively limited nature of available reports. The purpose of this study was to clarify the characteristics and prognostic factors of IPs complicated with pneumomediastinum. Methods Consecutive patients with IPs complicated with pneumomediastinum detected by computed tomography (CT) between July 1, 2011, and April 30, 2014 were retrospectively reviewed. Clinical data including symptoms associated with pneumomediastinum, laboratory data, lung function tests, treatments, and mortality were collected from medical records. Results Forty-five patients (25 males, 20 females), including 32 with idiopathic IP (IIPs) and 13 connective tissue disease-associated interstitial lung diseases (CTD-ILDs) were identified. The median age of onset of pneumomediastinum was 72 years (interquartile range [IQR] 68–79 years). The most common symptom associated with occurrence of pneumomediastinum was appearance or worsening of dyspnoea. No specific treatment was performed for most (84%) of the cases. The median period between occurrence and improvement of pneumomediastinum was 29 days (IQR 5–69 days). Multivariate analysis revealed that IIPs and no improvement of pneumomediastinum were associated with poor prognosis. Conclusions Patients with IIPs complicated with pneumomediastinum and those without improvement of pneumomediastinum had poor prognosis.

Pulmonary veno-occlusive disease (PVOD) is a rare disease in the subgroup of conditions known as pulmonary arterial hypertension. Although a histological examination is needed for a definitive diagnosis, a non-invasive diagnosis is required for patients with pulmonary hypertension because a lung biopsy is deemed risky. We herein report a 32-year-old woman diagnosed with PVOD via a surgical lung biopsy and autopsy whose disease showed radiological findings mimicking those of hypersensitivity pneumonitis (pneumonia) at the time of the transbronchial lung biopsy, without obvious pulmonary hypertension on admission. When clinicians encounter patients with interstitial lung disease, they should not forget the possibility of PVOD and should be alert for emerging pulmonary hypertension.

Background and objective Acute exacerbation (AE) in idiopathic pulmonary fibrosis (IPF) or other idiopathic interstitial pneumonias (IIP) is a poor prognostic event despite conventional therapy with corticosteroids and/or immunosuppressants. We aimed to evaluate the efficacy and safety of recombinant human soluble thrombomodulin (rhTM) for AE-IIP. Methods For this prospective single-arm open-label multicentre cohort study, we retrospectively registered 61 cases of AE-IIP treated with conventional therapy between 2011 and 2013 (control arm), and prospectively enrolled 39 cases of AE-IIP treated with conventional therapy and rhTM (380 U/kg/day for 6 days) between 2014 and 2016 (rhTM arm). To reduce potential confounding in treatment comparisons, an adjusted mortality analysis for 90-day survival was conducted with weighted Cox proportional hazards regression models using inverse probability of treatment weighting. Weights were derived from propensity scores estimated using a multivariable logistic regression analysis including potential confounders. Results The 90-day survival rates of AE-IIP patients treated with/without rhTM were 66.7% (26/39) and 47.5% (29/61), respectively. After adjusting for imbalances, rhTM therapy was significantly associated with reduced mortality (adjusted hazard ratio (HR): 0.453; 95% CI: 0.237-0.864; P = 0.0163). The frequencies of adverse events with/without rhTM were 17.9% (7/39) and 19.7% (12/61), which were similar in both arms (P = 1.0). Two bleeding-related adverse events occurred in the rhTM arm. Conclusion Safety and efficacy were observed for rhTM treatment of AE-IIP. A future randomized controlled trial is required to draw final conclusions.

Background Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease characterized by the abnormal accumulation of surfactant-like material within the alveolar spaces and distal bronchioles. If high-resolution computed tomography (HRCT) indicates the presence of PAP, a definitive diagnosis of PAP is established when consistent pathological findings are obtained. Herein, we retrospectively studied the yield and safety of bronchofiberscopy in the diagnosis of PAP. Methods One hundred and fifty consecutive patients with PAP were prospectively registered in the PAP cohort database of the National Hospital Organization Kinki-Chuo Chest Medical Center between January 1991 and December 2018. We examined 86 patients who underwent bronchofiberscopy with bronchoalveolar lavage (BAL) and transbronchial lung forceps biopsy (TBLB). Results The patients included 56 men and 30 women, with a median age of 57 years. All patients had autoimmune PAP, and the median level of anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies was 42.8 μg/mL. The diagnostic yield was 90.7% (78/86) with BAL and 81.4% (70/86) with TBLB. The combination of BAL and TBLB increased the yield to 98.8%. Age, disease severity score, and frequency of traction bronchiectasis on HRCT were significantly different between the TBLB-positive and TBLB-negative groups. No patient developed serious complications due to bronchofiberscopy; TBLB-related complications included pneumothorax (3.5%) and minimal bleeding (7.0%). Conclusions Bronchofiberscopy, in combination with BAL and TBLB, is an effective and safe method for the diagnosis of PAP, with a yield of 98.8%.

Nivolumab has been associated with unique adverse events known as immune-related adverse events. Although interstitial lung disease (ILD) is a life-threatening immune-related adverse event, the risk of ILD during nivolumab treatment is unclear. In this report, we encountered three patients with stage IV non-small cell lung cancer with signs of lung obstruction caused by tumor-mediated compression on imaging who developed acute ILD within 10 days of commencing nivolumab treatment. The first case involved a 74-year-old Japanese female never-smoker, the second a 67-year-old Japanese female never-smoker, and the third a 75-year-old Japanese female current-smoker. The first patient was administered nivolumab as third-line chemotherapy, the second was administered nivolumab as fifth-line chemotherapy, and the third was administered nivolumab as second-line chemotherapy. Regardless of aggressive treatments for ILD, 2 of 3 patients died. The findings of these cases suggest that obstructive findings in the lungs, which easily cause infections, may be an important risk factor for nivolumab-induced ILD.

Dyspnea is highly prevalent in patients with idiopathic interstitial pneumonias (IIPs).The objective of this study is to examine the effectiveness and safety of continuous subcutaneous morphine for dyspnea in terminally ill IIP patients.We retrospectively reviewed cases of terminally ill IIP patients who received continuous subcutaneous morphine for dyspnea.We reviewed dyspnea severity measured using numerical rating scale (NRS) and respiratory rate (RR) before and two and four hours after morphine initiation. We conducted subgroup analyses of patients with and without noninvasive positive pressure ventilation (NPPV).Twenty-five patients were included in this study. Median morphine dose at morphine initiation and two and four hours after treatment was 0.25, 0.25, and 0.5 mg/hour, respectively. Dyspnea NRS decreased significantly four hours after (mean ± standard deviation: 5.32 ± 2.58, p = 0.04) but not two hours (5.52 ± 2.43, p = 0.11) after morphine initiation compared with baseline (7.08 ± 2.33). RR did not change significantly either two or four hours after treatment compared with baseline. The median survival after morphine initiation was 47 hours. Patients who were not using NPPV showed significantly improved dyspnea both two and four hours after treatment compared with baseline, although patients who used NPPV showed no significant improvement with morphine. RR did not significantly change in either subgroup.Morphine might improve dyspnea in terminally ill IIP patients without decrease in RR.

The CT findings of pulmonary fibrosis in patients with pulmonary alveolar proteinosis (PAP) are not yet well defined. The objective of this study was to evaluate the CT findings of PAP with a focus on pulmonary fibrosis secondary to PAP.High-resolution CT (HRCT) scans of 44 patients with PAP were evaluated retrospectively with a focus on pulmonary fibrosis: 33 patients had autoimmune PAP, and 11 patients had secondary PAP. The intervals between the initial and last CT examinations ranged from 1 to 284 months (median, 60 months). The HRCT images were assessed by two chest radiologists independently; when the two radiologists disagreed, a final decision was made by consensus.A crazy-paving pattern was a more common HRCT finding in patients with autoimmune PAP than in those with secondary PAP. Traction bronchiectasis was found in four patients (9%) on the initial scans and in 10 patients (23%) on the last scans. There was no honeycombing on the initial scans. Honeycombing developed in two patients (5%): It was detected on 2-year follow-up in one patient and on 6-year follow-up in the other patient. Among the patients with autoimmune PAP, those with fibrosis detected on HRCT during follow-up had a worse prognosis than those without fibrosis detected on HRCT (p = 0.041).Fibrosis develops in approximately 20% of patients with PAP. The CT findings of parenchymal fibrosis suggest a poor outcome.

BackgroundAcute exacerbation (AE) in idiopathic pulmonary fibrosis and other idiopathic interstitial pneumonias (IIPs) are poor prognostic events although they are usually treated with conventional therapy with corticosteroids and immunosuppressants. Previously, we demonstrated the safety and efficacy of recombinant human soluble thrombomodulin (rhTM) for AE-IIP in the SETUP trial. Here, we aimed to clarify the efficacy of rhTM for poor-prognosis cases of AE-IIP.MethodsIn this study, we included 85 patients, in whom fibrin degradation product (FDP)/d-dimer was evaluated at AE, from the 100 patients in the SETUP trial. The AE-IIP patients in the rhTM arm (n=39) were diagnosed using the Japanese criteria from 2014 to 2016 and treated with intravenous rhTM for 6 days in addition to the conventional therapy. The AE-IIP patients in the control arm (n=46) were treated with the conventional therapy without rhTM between 2011 and 2013. The subjects were classified into higher and lower FDP/d-dimer groups based on the Japanese Association for Acute Medicine Disseminated Intravascular Coagulation scoring system. A multivariate Cox proportional hazard regression analysis with stepwise selection was performed to reveal the prognostic factors of AE-IIP.ResultsWe developed a prognostic scoring system using two significant prognostic factors, higher FDP/d-dimer at AE and prednisolone therapy before AE, with 3 and 2 points assigned for each parameter, respectively. The prognostic scores ranged from 0 to 5. Survival of AE-IIP patients with a prognostic score=0 was significantly better than that of patients with score ≥2. Survival was improved with the rhTM therapy (pConclusionsTreatment with rhTM might improve survival in AE-IIP cases with poor prognoses.Trial registration numberUMIN000014969, date: 28 August 2014.

A 34-year-old woman experiencing shortness of breath was referred to our hospital. The patient was diagnosed with sporadic lymphangioleiomyomatosis based on the observation of bilateral diffuse multiple thin-walled cysts on computed tomography of the chest, chylous effusion, elevated serum vascular endothelial growth factor-D levels and transbronchial biopsy findings. This patient was a hepatitis B virus (HBV) carrier. Treatment with 1 mg daily of sirolimus was started after HBV DNA was brought below the cut-off level using entecavir. Sirolimus was effective, as the chylous effusion resolved completely and the dyspnea improved. The sirolimus dosage was increased to 2 mg daily without causing HBV reactivation.

Background Lymphangioleiomyomatosis (LAM) and connective tissue diseases (CTDs) occur more frequently among women than men. We investigated the frequency of comorbid CTD and positive serum autoantibody findings in patients with LAM. Methods A total of 152 patients with LAM were prospectively and consecutively registered in the National Hospital Organization Kinki-Chuo Chest Medical Centre cohort. The clinical data were retrospectively analysed, and patients were categorised into the following three groups: a CTD group, a non-CTD-autoantibody-positive group, and a non-CTD-autoantibody-negative group. Results All patients were women. We identified five patients with comorbid CTDs (3.3%): Sjögren’s syndrome (SjS) (n = 3), systemic lupus erythematosus (n = 1), and rheumatoid arthritis (n = 1). One patient with SjS was also diagnosed with antiphospholipid antibody syndrome. The positive rate for anti nuclear antibody was 31.5% and 6.9% at dilution of 1:40 or higher, and those of 1:160 or higher, respectively. It tended to be lower in patients with LAM than in healthy women. The positive rate for anti-SS-A and anti-SS-B antibody was 7.9% and 1.8%, respectively. No significant differences in age, type of LAM, smoking status, serum vascular endothelial growth factor D level, respiratory function, treatment, or prognosis were observed among the three groups. Conclusions Comorbid CTDs, especially SjS, in LAM patients should be considered.

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

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: 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.

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.

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.

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.

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.

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.

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.

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.

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.

Purpose: To quantify observer agreement and analyze causes of disagreement in identifying honeycombing at chest computed tomography (CT). Materials and Methods: The institutional review board approved this multi-institutional HIPAA-compliant retrospective study, and informed patient consent was not required. Five core study members scored 80 CT images with a five-point scale (5 = definitely yes to 1 = definitely no) to establish a reference standard for the identification of honeycombing. Forty-three observers from various subspecialties and geographic regions scored the CT images by using the same scoring system. Weighted kappa values of honeycombing scores compared with the reference standard were analyzed to investigate intergroup differences. Images were divided into four groups to allow analysis of imaging features of cases in which there was disagreement: agreement on the presence of honeycombing, agreement on the absence of honeycombing, disagreement on the presence of honeycombing, and other (none of the preceding three groups applied). Results: Agreement of scores of honeycombing presence by 43 observers with the reference standard was moderate (Cohen weighted kappa values: 0.40-0.58). There were no significant differences in kappa values among groups defined by either subspecialty or geographic region (Tukey-Kramer test, P = .38 to >.99). In 29% of cases, there was disagreement on identification of honeycombing. These cases included honeycombing mixed with traction bronchiectasis, large cysts, and superimposed pulmonary emphysema. Conclusion: Identification of honeycombing at CT is subjective, and disagreement is largely caused by conditions that mimic honeycombing. (C) RSNA, 2012

We treated 8 cases of autoimmune pulmonary alveolar proteinosis (APAP) with whole lung lavage (WLL) in our hospital and evaluated the disease severity of APAP before and after WLL, adverse events (AEs) and recurrence-free survival in those cases who improved. In all cases, unilateral WLL was performed in both lungs. The median of total lavage volume in unilateral WLL was 17.9 L, and the median procedure time of unilateral WLL was 105 min. Fever was the most frequently observed AE (87.5% of all procedures). Pulmonary function tests (percentage of predicted value of VC, FEV1 and diffusing capacity of carbon monoxide), serum markers (KL-6, surfactant apoprotein (SP)-D, SP-A and carcinoembryonic antigens), arterial blood gas analyses (PaO2, AaDO2) and disease severity score all significantly improved after WLL. The serum levels of anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) antibody temporarily decreased after unilateral WLL, but returned to previous levels (before WLL) in 7 cases. The radiological findings improved in 6 cases. In the 7 improved cases in whom AaDO2 decreased more than 10 Torr, the median recurrence-free survival of APAP after WLL was 17.5 months. We concluded that WLL is an effective and safe method for the treatment of APAP, and all parameters except for anti GM-CSF antibody are useful to evaluate the effect of WLL.

PURPOSE: The authors reviewed serial computed tomographic (CT) scans obtained in 19 patients with diffuse panbronchiolitis (DPB) to evaluate changes in disease pattern over time. MATERIALS AND METHODS: Nineteen patients with DPB were entered into the study. After initial CT examination, 12 patients were randomly assigned to receive long-term low-dose (200 mg three times daily) erythromycin therapy; seven patients received no treatment. RESULTS: Follow-up CT scans revealed that centrilobular areas of high attenuation observed initially had progressed to dilatation of the proximal airway in some patients in the untreated group. In the treated group, the centrilobular and branched linear areas of high attenuation were decreased in number and size, although the airway dilatation and decreased lung attenuation in the peripheral areas remained unchanged or were slightly increased. CONCLUSION: CT scans are valuable in the study of the disease process and response to therapy in DPB.

Background The aims of this study were to retrospectively assess the change in findings on follow-up CT scans of patients with non-specific interstitial pneumonia (NSIP; median, 72 months; range, 3–216 months) and to clarify the correlation between the baseline CT findings and mortality. Methods The study included 50 patients with a histologic diagnosis of NSIP. Two observers evaluated the high-resolution CT (HRCT) findings independently and classified each case into one of the following three categories: (1) compatible with NSIP, (2) compatible with UIP or (3) suggestive of alternative diagnosis. The correlation between the HRCT findings and mortality was evaluated using the Kaplan–Meier method and the log-rank test, as well as Cox proportional hazards regression models. Results Ground-glass opacity and consolidation decreased, whereas coarseness of fibrosis and traction bronchiectasis increased on the follow-up HRCT scans, however, in 78% of cases the overall extent of parenchymal abnormalities had no change or decreased. Patients with HRCT diagnosed compatible with NSIP had a longer survival than those with HRCT findings more compatible UIP or an alternative diagnosis. On multivariate analysis, the coarseness of fibrosis alone was associated with prognosis (HR: 1.480; 95% CIs 1.100 to 1.990). Conclusions The HRCT patterns seen in patients with a histopathologic diagnosis of NSIP progress in a variable manner. Overall disease extent may decrease over time in some, while fibrosis may progress in others. The initial HRCT diagnosis may impact survival in this group of patients.

Objective: Occupational lung diseases have specific radiographic manifestations not always well known by physicians. In Japan, asbestos-related diseases became a public health concern after the "Kubota Shock", when a number of workers and residents living nearby a manufacturer of asbestos-made ducts developed mesothelioma caused by asbestos exposure. This preliminary intervention trial evaluated the effect of two-hour training on inexperienced physicians' skill in interpreting pneumoconiotic chest radiographs. Methods: One hundred-two Japanese physicians participated in two reading-tests, using 12 radiographs, before and after the two-hour training with ILO/ICRP and Japan Pneumoconioses Study Group (JPSG) reading materials. Physicians had to check for the presence or absence of small opacity and pleural plaque consistent with pneumoconiosis. Sensitivity and specificity equal or greater than 70% were considered good, 50 to 69% acceptable and less than 50%, poor. Results: Post-training improvements in physicians' skills were seen. For small opacity, there was an increase in the proportion of physicians with good specificity, from 42% to 60%. For pleural plaque, the proportion of physicians with good specificity and good sensitivity increased, from 60% to 67% and from 18% to 25%, respectively. Also, significant improvements were observed in overall sensitivity for pleural plaque, from 46% to 60% (p

To determine the specific high resolution CT (HRCT) features for "pseudoalveolar" sarcoidosis, we reviewed HRCT of 10 patients with pathologically proven sarcoidosis. On HRCT the lesions of pseudoalveolar sarcoidosis were shown as homogeneous or inhomogeneous opacities 1-4 cm in diameter located either along the bronchovascular bundles or in the lung periphery adjacent to the pleural surface. The margin was irregular, and numerous nodules 1-2 mm in diameter were observed in the surrounding lung. Air bronchograms were observed in 6 patients. In addition to these lesions, other CT abnormalities such as nodules, groundglass opacities, thickened bronchovascular bundles, and interlobular septa were found in all 10 cases. Good response of pseudoalveolar sarcoid lesions to administration of corticosteroid was observed in all three cases in which follow-up CT was available.

Summer-type hypersensitivity pneumonitis is an immunologic disease that occurs only in Japan. It is a form of hypersensitivity pneumonitis in which the clinical symptoms appear in the summer and subside spontaneously in mid autumn. The purpose of our study was to determine the CT findings in this condition, to compare the CT findings with those on chest radiographs, and to assess the variations in the CT findings over time. Accordingly, high-resolution CT scans and chest radiographs of 15 patients with summer-type hypersensitivity pneumonitis were retrospectively studied. Seven patients had sequential CT examinations 18-37 days apart. The CT scans and chest radiographs were reviewed by two observers independently. CT findings included diffuse micronodules (n = 15), slightly elevated lung density (n = 13), and patchy air-space consolidation (n = 13). In one patient, the findings on a chest radiograph were normal, while CT showed parenchymal abnormalities. In two cases, follow-up CT showed micronodular abnormalities after findings on the chest radiograph had returned to normal. Our results show that high-resolution CT findings of summer-type hypersensitivity pneumonitis include pulmonary micronodules, increased lung density, and air-space consolidation. High-resolution CT appears to be more useful than plain chest radiographs in the evaluation of pulmonary parenchymal abnormalities in this condition.

The serial computed tomography findings and prognosis of the acute exacerbation of idiopathic pulmonary fibrosis (IPF) are not yet well defined in a larger number of cases.To evaluate the parenchymal abnormalities and prognosis using high-resolution computed tomography (HRCT) in acute exacerbation of IPF.The study consisted of clinical, laboratory, and HRCT data before and at the time of acute exacerbation in 64 episodes of 58 patients with IPF. A semiquantitative analysis of overall extent of parenchymal abnormalities, extent of alveolar opacity (ground-glass attenuation and consolidation), and extent of fibrotic opacity (reticulation and honeycombing) on CT was performed by two chest radiologists. The newly appeared parenchymal abnormalities were also classified into three patterns: peripheral, multifocal, and diffuse.In all patients, HRCT scans taken at the exacerbation showed typical signs of IPF and newly developing alveolar opacity. They included 34 patients of peripheral pattern, 8 of multifocal pattern, and 16 of diffuse pattern. Twenty-five patients died and 33 survived after the initial exacerbation. Worse survival was associated with patients with diffuse type compared with patients with multifocal and peripheral type. The CT patterns and overall CT extent were associated with an increased hazard of death after adjusting for age, sex, smoking, baseline diffusion capacity for carbon monoxide, baseline FVC, and disease extent on CT. On multivariate analysis, the strongest correlations were observed between CT patterns (combined diffuse and multifocal versus peripheral) and survival (odds ratio, 4.629; 95% confidence interval, 1.900-11.278; P = 0.001).HRCT extent and patterns are predictive of survival in acute exacerbation of IPF.

A 56-year-old female non-smoker complained of general fatigue and pyrexia. Laboratory examination revealed elevation of white blood cells (WBC) 10200/microl and C-reactive protein (CRP) 13.3mg/dl. Chest radiograph showed bilateral patchy and ring-shaped consolidations in both lungs. Chest computed tomography (CT) revealed multiple round ground glass opacities fringed with consolidation, namely the "reversed halo sign". Transbronchial lung biopsy specimens showed polypoid granulation tissue in terminal air spaces, consistent with an organizing pneumonia pattern. Clinical findings suggesting collagen vascular diseases and drug induced lung diseases were not recognized, thus cryptogenic organizing pneumonia (COP) was diagnosed. High dose methylprednisolone therapy improved her condition, and the ring-shaped opacities on chest CT resolved. She relapsed twice during approximately five years after the disease onset. Chest CT at the recurrences revealed subpleural patchy consolidations without formation of the "reversed halo sign". The sign was originally supposed to be specific for cryptogenic organizing pneumonia. However, other diseases like sarcoidosis and paracoccidioidomycosis have been reported to show this sign. The meaning of the "reversed halo sign" should be examined based on the accumulation of more cases.

Objectives The aim of the present study was to determine how the pattern and extent of sarcoidosis changes over time on serial high-resolution CT (HRCT) scans and to identify CT scan findings that might be helpful in predicting the prognosis of patients with the disease. Methods The initial and last HRCT scan findings of 40 patients with pulmonary sarcoidosis during a mean follow-up period of 7.4 years (range, 3 to 18 years) were evaluated retrospectively. HRCT scan findings then were correlated with the pulmonary function tests. Results Parenchymal abnormalities in most patients with a predominant nodular pattern (18 patients) and multiple large nodular pattern (8 patients) disappeared or decreased in size on long-term follow-up CT scans. A conglomeration pattern (five patients) shrank and evolved into bronchial distortion. The shrinkage of the conglomeration pattern correlated with a decline of FEV1/FVC ratio, despite an improvement in FVC. A ground-glass opacity pattern (five patients) and a consolidation pattern (three patients) evolved into honeycombing. The evolution of the ground-glass opacity and consolidation patterns into honeycombing occurred along with a decline in FVC, although the parenchymal abnormalities became smaller. Conclusion Patients with a predominantly ground-glass opacity pattern and consolidation pattern seen on the initial CT scan had a worse prognosis and were susceptible to developing severe respiratory insufficiency. The predominant patterns seen on the initial HRCT scan may be helpful in predicting the outcomes of patients with sarcoidosis.

The purpose of this study was to establish the technique of multiplanar reconstruction (MPR) with multidetector-row (MDR) computed tomography (CT) guided needle biopsy for the diagnosis to access very difficult lesions. The CT guided percutaneous biopsy are well-established methods to obtain cytological and histological material such as the peripheral tumors in lung cancer. Occasionally, the conventional CT cannot permit planning a trajectory to avoid passage through bones, avoidance of bullae, fissures or vessels. In addition, some lesions are situated in less favorable locations such as those in the costophrenic recess or close to the mediastinum. Rarely can we diagnose them. MPR with MDR-CT has recently become widely available with applications for thoracic lesions. MPR images have been used to evaluate the location of small peripheral lung nodules to the relation of bullaes, vessels, and costophrenic recess. To diagnose these lesions, the usefulness of MPR were evaluated for an planning of an oblique approach of CT guided needle biopsy. MPR images were reconstructed as a line from the needle entry point to the target lesion. The first oblique image applied as the direction of posterior-anterior and cranio-caudal axis, and the second oblique image applied as the direction of posterior-anterior and left-right. Eleven out of 151 patients were required MPR technique to allow possible access to target, because of avoidance of bone and fissures in the needle pass or located in the costophrenic recess, between April 2001 and December 2002. The 5/11 patients were at the upper site (segment 1, 2 and 6) behind the scapula and ribs, 3/11 patients were at the lower lobe (segment 10) in the costophrenic recess, and 3/11 were middle lobe or segment 3 covered by the ribs and fissures. All the lesions except one were histologically diagnosed. Five patients were adenocarcinoma, and the other five patients were benign tumors. Pneumothorax occurred in one patient before we obtained the specimens. MPR guided needle biopsy with oblique approach was thought to be useful for diagnosis of very difficult thoracic lesions and would obviate an unnecessary surgical thoracoscopy.