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Background and objective Interstitial lung disease (ILD) is a common pulmonary manifestation of systemic sclerosis (SSc). It is unknown whether radiographic fibrosis score predicts mortality in SSc-associated ILD (SSc-ILD). We retrospectively analysed patients with SSc-ILD to evaluate whether radiographic fibrosis score was a useful predictor of mortality. Methods We identified SSc-ILD patients evaluated at Kurashiki Central Hospital (Japan) from 2006 to 2016, and radiographic fibrosis scores based on the extent of reticulation and honeycombing on high-resolution computed tomography (HRCT) scanning were calculated by manually tracing around each fibrotic area. Independent predictors of overall survival were determined using the Cox proportional hazards model. Results The study included 48 patients, of whom 19 had usual interstitial pneumonia on HRCT. The median follow-up period was 56.6 months, and over the follow-up period 15 patients died. The 5-year survival was 72.4%. In the multivariate analysis, radiographic fibrosis score, age, being male and forced vital capacity were independently associated with an increased risk of death, while HRCT pattern was not. Conclusion A high radiographic fibrosis score was a poor prognostic factor in SSc-ILD. More widespread fibrosis was associated with an increased risk of death, independent of HRCT pattern.

Background and objective Lung cancer is prevalent among patients with interstitial pneumonia (IP). HRCT findings mucinous adenocarcinoma in patients with IP have not been described. Methods In 112 consecutive patients with 120 surgically resected IP-associated lung cancers, 42 patients had pathologically proven invasive adenocarcinoma (IA). A total of 14 out of 42 patients (10 men, 4 women, mean age, 68.4 years) had invasive mucinous adenocarcinoma. We reviewed the patients' medical records and HRCT scans. Results Invasive mucinous adenocarcinoma were most commonly associated with idiopathic IP (n = 13) affecting the lower lobe adjacent to a fibrocystic changes. In 11 patients with invasive mucinous adenocarcinoma or other types of IA, the tumour was adjacent to a fibrocystic lesion. In invasive mucinous adenocarcinoma, malignant signs included lobulation (n = 11), spiculation (n = 9), vascular convergence (n = 10) and pleural indentation (n = 2). Characteristic findings of mucinous adenocarcinoma (i.e. vague margins (n = 10), lobular-bounded margins (n = 11), air bronchogram (n = 11) and bubble-like low attenuation (n = 8)) were more common in invasive mucinous adenocarcinoma than in other IA types. All invasive mucinous adenocarcinoma tumours (n = 11) were closely associated with fibrosis. Conclusion Mixed ground-glass opacity and consolidation adjacent to a fibrocystic lesion with malignant signs and characteristic features of mucinous adenocarcinoma indicate malignancy.

Background and objective 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. Methods 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. Results 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. Conclusion 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.

Background and objective: Although systemic lupus erythematosus (SLE) is the most common connective tissue disease affecting the lung, few studies have assessed risk factors that predict pulmonary manifestations. The objectives of the present study were to determine the prevalence of lung manifestations in SLE patients from Western Saudi Arabia by analysing results from high-resolution computed tomography (HRCT) scans and to identify independent risk factors for lung involvement. Methods: This was a 10-year retrospective study involving 184 SLE patients. We examined all HRCT lung abnormalities and determined whether findings were associated with the presence of lupus nephritis (LN), SLE disease activity (as defined by SLE Disease Activity Index 2000 item scores ≥ 4 for any and all items) or levels of complement and anti-double-stranded DNA (anti-dsDNA). Results: We identified 61 patients (33%) with pulmonary involvement, and 52 (85%) of these subjects showed HRCT abnormalities. The most common HRCT findings were pleural effusion, consolidation and atelectasis (58%, 42% and 42%, respectively). There was a significant association between abnormal HRCT results and hypocomplementemia, high levels of anti-dsDNA and disease activity (P < 0.05), particularly with regard to pleuropericardial effusion and consolidation. Pulmonary abnormalities were significantly higher within the first five years after SLE diagnosis (P < 0.001). However, neither disease duration nor LN was associated with increased risk. Conclusions: Lung manifestations were frequent in SLE patients from Saudi Arabia, with pleural effusion, consolidation and atelectasis being the most common. Low complement levels, high anti-dsDNA levels and disease activity were significantly associated with abnormal HRCT findings (all P < 0.001).

Keywords: diagnosis; high-resolution computed tomography; idiopathic pulmonary fibrosis; surgical lung biopsy; usual interstitial pneumonia

Background and objective: Obstructive airflow limitation is the most common physiological impairment in sarcoidosis. This study determined the prevalence of airway hyperresponsiveness (AHR) in sarcoidosis, the correlation between responses to direct (using histamine) and indirect (using hypertonic saline) bronchial challenge, and the clinical, physiological and radiological predictors of AHR. Methods: Subjects with sarcoidosis and a baseline forced expiratory volume in 1 s (FEV1) >35% predicted underwent hypertonic and histamine challenge, lung function testing and high resolution computed tomography (HRCT) of the chest. AHR was defined as a 15% fall in FEV1 to hypertonic saline and a 20% fall in FEV1 to histamine. Results: The 52 subjects had well-preserved lung function (FEV1 = 2.8 ± 0.7 L, 87% predicted). AHR was detected in 5/47 (11%) to hypertonic saline and 19/43 (44%) to histamine challenge. On univariate analysis, response to histamine challenge was predicted by conglomerate fibrosis (P = 0.02) and reticular pattern (P = 0.03) on HRCT. The baseline % predicted forced expiratory volume in 1 s was significantly inversely associated with AHR on univariate (P = 0.004) and multivariate analysis (P = 0.01) when adjusted by HRCT patterns. Conclusions: The higher prevalence of AHR using histamine challenge than hypertonic saline challenge and the association with baseline % predicted FEV1 suggest that the AHR in sarcoidosis may reflect the consequences of airway remodelling following inflammation.

Background and objective: Chest MRI is increasingly used to assess pulmonary diseases, but its utility compared with high-resolution computed tomography (HRCT) has never been evaluated in children using specific performance outcomes. The aim of this study was to assess the accuracy and reliability of MRI compared with HRCT in children with non-cystic fibrosis (CF) chronic lung disease. Methods: Fifty subjects aged 5.9–20 years, with primary ciliary dyskinesia (n = 17), primary immunodeficiency (n = 17) or recurrent pneumonia (n = 16), underwent chest HRCT and MRI. The prevalence of lung abnormalities on HRCT was evaluated, and sensitivity, specificity, accuracy and positive and negative likelihood ratios for MRI versus HRCT were calculated. MRI and HRCT scans were also assessed using a modified Helbich score. Results: Bronchiectasis, mucous plugging, peribronchial wall thickening, consolidation, bullae, abscesses and emphysema were detected by HRCT in 72, 68, 66, 60, 10, 8 and 8% of subjects, respectively. Sensitivity, specificity, accuracy and positive and negative likelihood ratios for MRI were good or excellent for most of the changes that were assessed. Median total Helbich scores for HRCT and MRI were 10 (range 0–20) and 10 (range 0–18), respectively. There was good-to-excellent agreement between the two techniques for all scores (r ≥ 0.8). A Bland–Altman plot confirmed this agreement between total scores (bias value: 0.2 ± 1.18; 95% limits of agreement of mean difference: −2.12–2.52). Conclusions: Chest MRI was equivalent to HRCT to determine the extent of lung disease in children with non-CF lung disease. The findings support the use of chest MRI as an alternative to HRCT in diagnostic pathways for paediatric chronic lung disorders.

Background and objective: In this study the prevalence, lung function and prognosis of IPF combined with emphysema were evaluated. Methods: Consecutive patients with usual interstitial pneumonia (UIP) on high-resolution computed tomography (HRCT), with or without emphysema, were assessed retrospectively. The area of fibrosis in the base of the lungs was assessed by HRCT as minimal (

Idiopathic pulmonary fibrosis (IPF), a progressive and fatal diffuse parenchymal lung disease, is defined pathologically by the pattern of usual interstitial pneumonia (UIP). Unfortunately, a surgical lung biopsy cannot be performed in all patients due to comorbidities that may significantly increase the morbidity and mortality of the procedure. High-resolution computed tomography (HRCT) has been put forth as a surrogate to recognize pathological UIP. The quality of the HRCT impacts the ability to make a diagnosis of UIP and varies based on the centre performing the study and patient factors. The evaluation of the HRCT includes assessing the distribution and predominance of key radiographical findings, such as honeycomb, septal thickening, traction bronchiectasis and ground glass attenuation lesions. The combination of the pattern and distribution is what leads to a diagnosis and associated confidence level. HRCT features of definite UIP (subpleural, basal predominant honeycomb with septal thickening, traction bronchiectasis and ground glass attenuation lesions) have a high specificity for the UIP pathological pattern. In such cases, surgical lung biopsy can be avoided. There are caveats to using the HRCT to diagnose IPF in isolation as a variety of chronic pulmonary interstitial diseases may progress to a UIP pattern. Referral centres with experience in diffuse parenchymal lung disease that have multidisciplinary teams encompassing clinicians, radiologists and pathologists have the highest level of agreement in diagnosing IPF.

This study was performed to confirm the cross-sectional and longitudinal construct validity of the Saint George's Respiratory Questionnaire (SGRQ) for the measurement of health-related quality of life (HRQoL) in patients with IPF.Sixty-eight patients with IPF responded to the SGRQ and pulmonary function tests (PFT), dyspnoea testing, arterial blood gas analysis, 6-min walk tests (6MWT) and high-resolution computed tomography were performed in a baseline study. A follow-up study was performed on 45 of these patients.In the baseline study HRQoL as measured by the SGRQ was substantially impaired in IPF patients, especially in symptoms and activity domains. A significant decline in HRQoL was observed in the activity domain during follow up. TLC and changes in TLC showed the most significant inverse correlations with each SGRQ domain (r-0.3, P0.05). In a stepwise multiple regression analysis, TLC contributed most significantly to each SGRQ component baseline score. Similar results were also observed during follow up. There was a significant correlation between total CT scores and each component of the SGRQ (r0.3, Por = 0.001). Changes in ground-glass opacity on CT (CT-alv) were also correlated with changes in each SGRQ domain (r0.3, Por = 0.001). Stepwise multiple regression analysis showed that interstitial opacity on CT (CT-fib) contributed to variation in the baseline activity score, and that changes in CT-alv independently contributed to overall changes in the SGRQ domains during follow up. The dyspnoea score, and changes in the dyspnoea score, correlated significantly with the SGRQ sores, with the exception of the symptoms score, in both the baseline and follow-up studies.HRQoL as assessed by the SGRQ showed good cross-sectional and longitudinal construct validity in patients with IPF. However, additional studies are required to analyse the reliability and responsiveness so that the SGRQ can be used in patients with IPF.

Background and objective: Coccidioidomycosis is endemic in the south-western USA. This study described the clinicopathological features of pulmonary coccidioidomycosis found in otherwise healthy Japanese individuals. Methods: Four male patients with pulmonary coccidioidomycosis were assessed by clinical examination, serology, PCR assay, high-resolution computed tomography and histopathological findings. Results: All patients had returned to Japan from the USA and all presented with an abnormal CXR during routine assessment; two of the patients were symptomatic. High-resolution computed tomography showed a well-defined homogenous nodule or mass adjacent to the pleura. All patients underwent surgical wedge resection. Histopathological examination revealed encapsulated caseating epithelioid cell granulomas containing spherules and endospores. Serum IgG antibody was negative in all patients, but PCR assay using DNA from resected specimens demonstrated the presence of coccidioides-specific DNA. Conclusions: Pulmonary coccidioidomycosis is very rare in Japan, but it can be found incidentally during routine evaluation. The main high-resolution computed tomography findings are a well-defined homogenous nodule adjacent to the pleura, reflecting the histological feature of encapsulated granulomas. PCR assay is useful for confirming the presence of coccidioides.

Clinical analysis of chronic obstructive pulmonary disease phenotypes classified using high- resolution computed tomography FUJIMOTO K, KITAGUCHI Y, KUBO K, HONDA T. Respirology 2006; 11: 731-740 Objective and background: The present study was performed to clarify the clinical characteristics of patients with COPD classified into phenotypes according to the dominancy of emphysema and the presence of bronchial wall thickening (BWT) evaluated by chest high-resolution CT. Methods A total of 172 patients with stable COPD (FEV1 < 80%) were examined by chest high- resolution CT. Emphysematous changes and BWT were evaluated visually, and COPD patients were classified into three phenotypes: absence of emphysema, with little emphysema with or without BWT (A phenotype), emphysema without BWT (E phenotype) and emphysema with BWT phenotype (M phenotype). The clinical characteristics were compared among the three phenotypes. Results The A phenotype showed a higher prevalence of those who had never smoked and patients with wheezing both on exertion and at rest, higher values of BMI and diffusing capacity for carbon mononide (DLCO), milder lung hyperinflation, and greater reversibility of airflow limitation respon- sive to β2-agonist as compared with the E phenotype. The M phenotype showed a higher prevalence of patients complaining of a large amount of sputum, productive cough and wheezing, higher rate of exacerbation or hospitalization and greater reversibility of airflow limitation responsive to β2-agonist as compared with the E phenotype. Conclusions These findings suggest that the morphological phenotypes of COPD show several clinical characteristics and different responsiveness to bronchodilators.

Objective: Obstructive airway disease, bronchiectasis, non-specific parenchymal infiltration and bronchiolitis obliterans organizing pneumonia are seen occasionally in patients with inflammatory bowel disease. In the present study, we evaluated ulcerative colitis (UC) patients for latent pulmonary involvement. Methods: Fifteen patients (nine females, six males, mean age 44 years) were admitted into the study. All patients were free of respiratory symptoms. Ulcerative colitis was active in nine patients and all patients were using anti-inflammatory treatment. Pulmonary function tests (PFT), high resolution computed tomography of thorax (HRCT) and bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial biopsy (TBLB) were carried out in all subjects. Results: Mild airways obstruction was found in 1 of 13 patients. High resolution CT was abnormal in 4 of 15 patients. Ground glass appearance suggestive of an interstitial lung disease was present in three patients. Radiolucency and pneumocysts were present in the fourth patient suggesting obstructive small airway disease. High resolution CT findings correlated with disease activity (P < 0.05). Fiberoptic bronchoscopy was performed in 10 patients. A mixed type alveolitis was detected in BAL fluid in five patients. In eight patients, TBLB showed alveolar septal thickening by connective tissue, lymphocytic accumulation in the septal area and minimal septal fibrosis, suggesting subclinical pulmonary parenchymal involvement linked to UC. Conclusions: Our data suggest that latent interstitial pulmonary involvement may occur during the course of UC, despite an absence of symptoms of lung disease.

Objective: The aim of this study was to describe the lung function and the computed tomographic features in a selected group of non-smoking women with chronic obstructive pulmonary disease and biomass fuel exposure. Methodology: Thirty female patients currently exposed to biomass smoke were prospectively investigated for further evaluation using high resolution computed tomography (HRCT), pulmonary function tests and blood gas analysis. Results: The mean age was 59 ± 11 years. The patients were exposed to biomass smoke for 37.4 ± 10 years. Pulmonary function tests revealed severe obstruction. The most common HRCT findings were increased lung volume or diffuse emphysema, thickening of interlobular septae, focal emphysematous areas, increased cardiothoracic ratio, and increased bronchovascular arborization. Conclusion: Biomass fuel has deleterious effects on pulmonary function and structure leading to obstructive and restrictive pathologies.

Objective: The aim of the study was to determine the clinical profile, aetiology and radiological categories in children diagnosed with bronchiolitis obliterans (BO). Methodology: We undertook a review of the medical records and radiological studies of 14 children with BO. Results: Unresolving cough and wheeze after a short respiratory illness was the commonest presentation. A viral pneumonitis was identified as the initial respiratory event prior to the development of BO in six children and Mycoplasma pneumoniae was the cause in another three children. Chest X-ray findings could be divided into four distinct patterns that were hyperinflation (n = 5), mixed pattern of atelectasis, hyperlucency and bronchial thickening (n = 4), unilateral small hyperlucent lung (n = 3) and unilateral collapse of one lung (n = 2). High resolution computed tomogram (HRCT) chest showing areas of hyperaeration and mosaic ground glass patterns with bronchial thickening were commonly found in patients whose chest X-ray showed bilateral changes. Patients with bilateral lung changes were more likely to have failure to thrive and persistent respiratory symptoms on follow up. Conclusion: A diagnosis of BO can be made from typical clinical features combined with an understanding of the different chest X-ray categories and HRCT of the chest. A viral aetiology was the commonest cause for BO in our series.

We analysed 53 cases of laboratory-confirmed Mycoplasma pneumoniae infection with cough lasting ≥ 7 days and chest radiography showing no abnormal findings. Twenty-two (41%) of those patients showed abnormal findings on chest high-resolution computed tomography. In the daily clinical setting, for assessment of acute cough, physicians should be aware that it is difficult to confirm bronchiolitis or bronchopneumonia due to M. pneumoniae by chest radiography.