High-resolution Computed Tomography Scores Research Articles

Bronchiectasis: correlation of high-resolution CT findings with health-related quality of life

To evaluate the relationship between the severity of bronchiectatic diseases, as evident on high-resolution computed tomography (HRCT) and the patient's quality of life measured using the St George's Respiratory Questionnaire (SGRQ). Forty-six patients (25 women, 21 men, mean age: 63 years) with bronchiectatic disease as evident on recent HRCT examinations were recruited. Each patient completed the SGRQ and underwent respiratory function tests. HRCT findings were blindly and independently scored by two radiologists, using the modified Bhalla scoring system. The relationships between HRCT scores, SGRQ scores and pulmonary function tests were evaluated. The patients' total CT score did not correlate with the SGRQ scores. However, patients with more advanced disease on HRCT, significantly differed in their SGRQ scores from patients with milder bronchiectatic disease. A significant correlation was found between the CT scores for the middle and distal lung zones and the activity, impacts and total SGRQ scores. No correlation was found between CT scores and respiratory function test indices. However, a significant correlation was found between the SGRQ scores and most of the respiratory function test indices. A correlation between the severity of bronchiectatic disease as expressed in HRCT and the health-related quality of life exists in patients with a more severe bronchiectatic disease but not in patients with mild disease. Such correlation depends on the location of the bronchiectasis in the pulmonary tree.

Pulmonary involvement in patients with early rheumatoid arthritis

Objectives: To assess the pleuropulmonary changes in patients with early rheumatoid arthritis (RA), using high‐resolution computed tomography (HRCT).Methods: Forty‐three non‐smoking patients with early RA were included. The disease duration was<1 year, without previous treatment. Disease activity was assessed using the 28‐joint indices score (DAS28). Hand and wrist X‐rays were evaluated using Larsen's criteria. Pulmonary functional tests (PFTs) were performed in 32 patients. The patients and 18 non‐smokers healthy individuals were assessed by plain chest X‐ray (CXR) and HRCT of the lungs.Results: HRCT revealed air trapping in 69% (25/36), bronchiectasis in 58% (25/43), bronchial wall thickening in 52% (22/43) and ground glass opacities (GGOs) in 35% (15/43) of the patients. Pleural thickening and effusion were observed in 11% (5/43). CXR was abnormal in one patient revealing a single pulmonary nodule. GGOs were the only HRCT sign observed exclusively in RA patients. All the other abnormalities were depicted in the control group at the same frequency as in the patients. However, the extent (as expressed by the HRCT score) of air trapping, bronchiectasis and bronchial wall thickening was significantly greater in the patients than in the control group (p<0.05). The PFTs were within normal values. DAS28, PFTs, and the Larsen score did not show any significant correlation with either each HRCT sign score separately or the total score.Conclusions: Lung abnormalities are frequently observed in patients with early RA on HRCT, even when CXR and PFTs are normal. Limited areas of GGOs were the abnormalities depicted exclusively in patients.

Extrinsic allergic alveolitis: comparative study of the bronchoalveolar lavage profiles and radiological presentation

Introduction: Extrinsic allergic alveolitis (EAA) is an immunologically mediated interstitial lung disease. The abnormalities in the bronchoalveolar lavage (BAL) fluid cell counts are almost always seen in patients with EAA...

Validity of the Saint George's Respiratory Questionnaire in the evaluation of the health-related quality of life in patients with interstitial lung disease secondary to systemic sclerosis

Interstitial lung disease (ILD) profoundly affects the health-related quality of life (HRQoL) in patients with systemic sclerosis (SSc). We tested the validity of the Saint George's Respiratory Questionnaire (SGRQ), a lung-specific HRQoL-evaluation tool, in a population of SSc patients with ILD. Twenty-eight consecutive SSc patients with a restrictive pulmonary involvement, defined as a forced vital capacity <80% of the predicted, with no pulmonary hypertension were considered. All the patients filled in the Medical Research Council (MRC) scale for perceived breathlessness, the SGRQ and the Disability Index of the Health Assessment Questionnaire (HAQ DI), and underwent evaluation with complete pulmonary function testing (PFT), 6-minute walk distance (6MWD) and high-resolution computed tomography (HRCT). The SGRQ 'activity' scores inversely correlated with the 6MWD (r = -0.86, P < 0.001) and forced vital capacity percentage of predicted values (r = -0.47) and directly correlated with HRCT (r = 0.41, P < 0.05), MRC (rho = 0.64, P < 0.001) or HAQ DI scores (r = 0.62, P < 0.001), independently of disease duration or subset. On the contrary, HAQ DI scores were influenced by those variables and corrected correlations with 6MWD (r = 0.56, P < 0.001) or HRCT scores (r = 0.36, P = NS) were less strong than those observed with the SGRQ. The SGRQ, although not specifically designed for scleroderma, is a valid respiratory-specific questionnaire for the evaluation of HRQoL in patients with SSc-related ILD. The SGRQ performs better in relation to exercise capacity and lung imaging than other non-respiratory-specific questionnaires widely used in scleroderma studies. Further studies are needed to address its ability to assess changes over time or in response to therapy.

Hyperpolarized 3helium magnetic resonance ventilation imaging of the lung in cystic fibrosis: comparison with high resolution CT and spirometry

The purpose of this study was to compare hyperpolarized 3helium magnetic resonance imaging (3He MRI) of the lungs in adults with cystic fibrosis (CF) with high-resolution computed tomography (HRCT) and spirometry. Eight patients with stable CF prospectively underwent 3He MRI, HRCT, and spirometry within 1 week. Three-dimensional (3D) gradient-echo sequence was used during an 18-s breath-hold following inhalation of hyperpolarized 3He. Each lung was divided into six zones; 3He MRI was scored as percentage ventilation per lung zone. HRCT was scored using a modified Bhalla scoring system. Univariate (Spearman rank) and multivariate correlations were performed between 3He MRI, HRCT, and spirometry. Results are expressed as mean+/-SD (range). Spirometry is expressed as percent predicted. There were four men and four women, mean age = 31.9+/-9 (20-46). Mean forced expiratory volume in 1 s (FEV)1 = 52%+/-29 (27-93). Mean 3He MRI score = 74%+/-25 (55-100). Mean HRCT score = 48.8+/-24 (13.5-83). The correlation between 3He MRI and HRCT was strong (R = +/-0.89, p < 0.001). Bronchiectasis was the only independent predictor of 3He MRI; 3He MRI correlated better with FEV1 and forced vital capacity (FVC) (R = 0.86 and 0.93, p < 0.01, respectively) than HRCT (R = +/-0.72 and +/-0.81, p < 0.05, respectively). This study showed that 3He MRI correlates strongly with structural HRCT abnormalities and is a stronger correlate of spirometry than HRCT in CF.

Nontuberculous mycobacteria in bronchiectasis: prevalence and patient characteristics

The aim of the current study was to investigate the prevalence and clinical associations of nontuberculous mycobacteria (NTM) in a well-characterised cohort of patients with adult-onset bronchiectasis. The sputum of all patients attending a tertiary referral bronchiectasis clinic between April 2002 and August 2003 was examined for mycobacteria as part of an extensive diagnostic work-up. NTM-positive patients subsequently had further sputa examined. A modified bronchiectasis scoring system was applied to all high-resolution computed tomography (HRCT) scans from NTM-positive patients, and a matched cohort without NTM. Out of 98 patients attending the clinic, 10 had NTM in their sputum on first culture; of those, eight provided multiple positive cultures. Three patients were treated for NTM infection. A higher proportion of NTM-positive than -negative patients were subsequently diagnosed with cystic fibrosis (two out of nine versus two out of 75). On HRCT scoring, more patients in the NTM-positive group had peripheral mucus plugging than in the NTM-negative group. In the current prospective study of a large cohort of patients with bronchiectasis, 10% cultured positive for nontuberculous mycobacteria in a random clinic sputum sample. Few clinical parameters were helpful in discriminating between groups, except for a higher prevalence of previously undiagnosed cystic fibrosis and of peripheral mucus plugging on high-resolution computed tomography in the nontuberculous mycobacteria group.

Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/dermatomyositis

To study the efficacy and safety of monthly intravenous pulse cyclophosphamide (IVCYC) therapy for progressive interstitial pneumonia in polymyositis/dermatomyositis (PM/DM). Seventeen patients with PM/DM/amyopathic DM (mean age 51.4 +/- 10.4, mean follow-up 32 months) who received IVCYC for progressive interstitial pneumonia between August 1993 and October 2002 were studied. Nine patients had failed to respond to previous treatment with high-dose steroid and/or immunosuppressant. Cyclophosphamide (300-800 mg/m2) was given at least six times every 4 weeks. Oral prednisolone (0.5-1 mg/kg/day) was administered for the first 2 weeks and was gradually tapered. Response to treatment was evaluated based on the degree of exertional dyspnea, pulmonary function test and high-resolution computed tomography (HRCT). Eleven of 17 patients showed improvement in their dyspnea; six out of seven patients who had required oxygen treatment before IVCYC no longer did so after IVCYC. Eight of 17 patients had >or=10% improvement of vital capacity (VC)% and 9/17 had >or=10 point reduction in their HRCT score. Twelve patients had exhibited at least one result. Two patients with anti-Jo-1 antibodies showed a flare-up of interstitial pneumonia or myositis. After the IVCYC therapy, mean VC% improved by 15% (from 68 to 83%, P = 0.0034). The extent of abnormal lesions in HRCT was reduced from 24 to 13% (P = 0.0055). There was neither death nor severe toxicities observed. In this open-label study, IVCYC improved symptoms, pulmonary function tests and HRCT findings in patients with PM/DM. Longitudinal controlled studies are required to further confirm the efficacy of IVCYC.

Atopy in Patients with Bronchiectasis: More than Coincidence

Bronchiectasis is common in developing countries, but its precise underlying mechanism can be detected in only about 40% of the cases. The studies reporting the frequency of atopy and its relation to radiological findings and lung function in bronchiectasis are limited in number, and the results are controversial. The present study was designed to investigate the relationship between atopy and bronchiectasis by means of high resolution computed tomography (HRCT) and pulmonary function tests. Skin prick test, HRCT and pulmonary function tests, including spirometric values of forced expiratory volume in one second (FEV1), FEV1/FVC (forced vital capacity) ratio were performed in 121 bronchiectatic patients of unknown etiology and in 68 healthy controls. Atopy and HRCT scores for the severity of atopy and extent of bronchiectasis respectively were determined for each patient. The rate of atopy (48.8% vs 11.8%) and mean atopy score (14.3 +/- 10.1 mm vs 5.5 +/- 2.1 mm) were significantly higher in patients with bronchiectasis than those in controls. Atopic patients had significantly worse spirometric values and more extended bronchiectasis than non-atopics. There is a significant correlation between atopy and HRCT scores (r = 0.54, p < 0.001), indicating that the more severe atopy is the more extended bronchiectasis. In conclusion, we suggest that the rate of atopy is higher in bronchiectatic patients than that in healthy controls. Bronchiectatic patients with atopy have lower spirometric values and higher HRCT scores. Atopy might be considered as a deteriorating and/or a causative or contributing factor for development of bronchiectasis.

Prevalence of interstitial lung involvement in patients with connective tissue diseases assessed with high‐resolution computed tomography

Objectives: To assess the prevalence of interstitial lung disease (ILD) in patients with different forms of connective tissue disease (CTD) using non‐invasive procedures including high‐resolution computed tomography (HRCT) and to evaluate the relationship between the imaging and functional status of the patients.Methods: Eighty‐one subjects with CTD (47 inpatients and 34 outpatients) were evaluated with pulmonary function tests (PFT) and radiological investigations. The extent and severity of lung disease was quantified with an HRCT scoring system previously used in patients with systemic sclerosis (SSc). Interstitial lung involvement was defined as predominantly fibrotic or inflammatory based on HRCT abnormalities.Results: HRCT abnormalities suggestive of ILD were observed in 69 patients (85.1%), whereas PFT and plain radiograph alterations occurred less frequently (40.7%). The most frequent HRCT abnormalities were septal/subpleural lines and ground‐glass appearance whereas lesions consistent with advanced fibrosis were observed in a minority of patients. The HRCT score was higher in patients with abnormal PFT (p<0.001). Thirty‐five patients had predominant fibrosis and 34 patients predominantly inflammatory abnormalities. A score of 10 points represented the best compromise between sensitivity and specificity in predicting functional impairment.Conclusions: A high prevalence of ILD was found based on HRCT abnormalities. However, HRCT scans characterized by minor abnormalities have poor specificity for clinically significant disease and functional findings should also be considered. The large number of patients with predominantly inflammatory HRCT abnormalities suggests that many cases of ILD may be diagnosed in a relatively early stage of the disease.

Persistence of lung inflammation and lung cytokines with high-resolution CT abnormalities during recovery from SARS

BackgroundDuring the acute phase of severe acute respiratory syndrome (SARS), mononuclear cells infiltration, alveolar cell desquamation and hyaline membrane formation have been described, together with dysregulation of plasma cytokine levels. Persistent high-resolution computed tomography (HRCT) abnormalities occur in SARS patients up to 40 days after recovery.MethodsTo determine further the time course of recovery of lung inflammation, we investigated the HRCT and inflammatory profiles, and coronavirus persistence in bronchoalveolar lavage fluid (BALF) of 12 patients at recovery at 60 and 90 days.ResultsAt 60 days, compared to normal controls, SARS patients had increased cellularity of BALF with increased alveolar macrophages (AM) and CD8 cells. HRCT scores were increased and correlated with T-cell numbers and their subpopulations, and inversely with CD4/CD8 ratio. TNF-α, IL-6, IL-8, RANTES and MCP-1 levels were increased. Viral particles in AM were detected by electron microscopy in 7 of 12 SARS patients with high HRCT score. On day 90, HRCT scores improved significantly in 10 of 12 patients, with normalization of BALF cell counts in 6 of 12 patients with repeat bronchoscopy. Pulse steroid therapy and prolonged fever were two independent factors associated with delayed resolution of pneumonitis, in this non-randomized, retrospective analysis.ConclusionResolution of pneumonitis is delayed in some patients during SARS recovery and may be associated with delayed clearance of coronavirus, Complete resolution may occur by 90 days or later.

Circulating levels of interleukin-18 in asbestos-exposed workers

Occupational exposure to asbestos has been associated with the development of pulmonary diseases through a persistent inflammatory response initiated by reactive oxygen species and the subsequent release of proinflammatory mediators such as interleukin (IL)-1, IL-6 and tumour necrosis factor (TNF)-a. Recently, IL-18, a pleiotropic proinflammatory cytokine and potent inducer of gene expression and synthesis of INF-g, TNF-a and IL-1 in immunocompetent cells, has been described. IL-18 serum concentrations were measured in 20 asbestos-exposed workers and in a group of 20 healthy donors. The workers underwent physical examination, pulmonary function tests and high-resolution computed tomography (HRCT). IL-18 levels were assayed in peripheral blood using the immunoenzymatic method with a commercial kit. The pulmonary function tests revealed a restrictive ventilatory deficit in nine (45%) cases and an obstructive ventilatory deficit in five (25%). The remaining six (30%) workers exhibited normal spirometric values. HRCT findings were: bilateral pleural plaques in 8 (40%) workers and bilateral parenchymal fibrosis in 12 (60%), with mean scores of 2.559 / 1.89. The exposed workers displayed significantly higher IL-18 concentrations than controls and those with parenchymal fibrosis displayed significantly higher values than workers with pleural plaques (P=0.001). In subjects with parenchymal fibrosis, HRCT scores significantly correlated with serum levels of IL-18 (P=0.001). Although the mechanisms leading to IL-18 release in asbestosis are unknown, the present preliminary results underscore the potential role of this cytokine in the pathogenesis of pulmonary toxicity.

High resolution computed tomographic features of pulmonary alveolar microlithiasis

Background: Pulmonary alveolar microlithiasis (PAM) is a rare, chronic lung disease with unknown etiology and with a nonuniform clinical course. Nonuniformity of clinical course might be related to the degree of pulmonary parenchymal alterations, which can be revealed with high resolution computed tomography (HRCT). However, HRCT findings of PAM were not fully described in the current literature. Aim: The aim of this study was to interpret and to contribute to describe HRCT findings of PAM and to investigate a correlation between profusion of micro nodules (MN) and pulmonary parenchymal alterations in patients with PAM. Material and methods: Ten male patients with PAM (mean age: 22 ± 3.2) were included into the study. HRCT images were assessed for patterns, distribution, and profusion of pulmonary abnormalities. Dividing the lungs into three zones, profusion of abnormalities was assessed. A profusion score (1–4) was given and the scores of each zone were then summed to obtain a global profusion score for HRCT ranging from 0 to 12. Also a parenchymal alteration score (PAS) was defined with respect to profusion of abnormalities. Chest X-rays were also scored. Results: All of ten patients with PAM had findings of interstitial lung disease in varying degrees on their HRCTs. HRCT findings of patients with PAM were as following: MN, parenchymal bands (PB), ground glass opacity (GGO) and, sub pleural interstitial thickening (SPIT) in 10 patients; interlobular septal thickening (ILST), in 9 patients; paraseptal emphysema (PSA) in 8 patients; centrilobular emphysema (CLA) in 7 patients; bronchiectasis (BE), confluent micro nodules (CMN) in 6 patients; peri bronchovascular interstitial thickening (PBIT) in 5 patients; panacinar emphysema (PANAA) in 3 patients; pleural calcification (PC) in 2 patients. A significant correlation between MN scores and PAS ( r = 0.68, p = 0.031, MN scores and GGO scores ( r = 0.69, p = 0.027) and, MN scores and CLA scores ( r = 0.67, p = 0.034) was detected. We also found significant correlations between HRCT scores and results of pulmonary function tests (PFTs), HRCT scores and chest X-ray score (CXRS) and, CXRS and results of PFTs. Conclusion: We conclude that patients with PAM may have all findings of interstitial lung disease in varying degrees as well as MNs on their HRCTs. More importantly, this study suggests a proportional relationship between profusion of MNs and parenchymal alterations in patients with PAM. This study also suggests that the degree of parenchymal alterations closely related with the degree of pulmonary function loss in patients with PAM.

Anti-neutrophil cytoplasmatic antibodies and lung disease in cystic fibrosis

Background: Bactericidal-permeability-increasing protein (BPI) is a potent anti-microbial protein produced by neutrophil granulocytes. Anti-neutrophil cytoplasmatic antibodies (ANCA) directed against BPI have been detected in up to 91% in patients with cystic fibrosis (CF). We aimed to evaluate the prevalence of BPI-ANCA in our CF patients and to determine whether presence of BPI-ANCA is correlated with organ damage. Methods: Twenty-four patients performed respiratory function testing and pulmonary high-resolution computed tomography (HRCT). HRCT was scored by using a modified Bhalla method. Serum samples were analysed by direct binding enzyme-linked immunosorbent assay for BPI-ANCA. Results: The prevalence of anti-BPI-IgG was 71% and anti-BPI-IgA 33%. Twenty-nine percent of our patients were positive for both BPI-ANCA isotypes. Mean HRCT score was 8.0 ranging from 0 to 22, bronchiectasis presented the most common finding (79%). There was a significant correlation between BPI-ANCA and both HRCT score and FEV 1 ( p<0.01). High levels of BPI-ANCA were correlated to chronic Pseudomonas aeruginosa lung infection ( p<0.01). Conclusions: BPI-ANCA was common in our study group. Highly significant correlations between BPI-ANCA and parameters to evaluate lung disease in CF may be a consequence of the inflammation process, or it may indicate a pathogenic role of BPI-ANCA levels in the development of lung disease. More research is needed and the clinical significance of our findings needs further evaluation.

High-resolution computed tomography in young patients with cystic fibrosis: Distribution of abnormalities and correlation with pulmonary function tests

ObjectiveTo assess pulmonary abnormalities detected by high-resolution computed tomography (HRCT) in young children with cystic fibrosis (CF) and mild to moderate lung disease. Study designHigh-resolution computed tomography was performed in 60 children, 6 to 10 years old, with mild to moderate lung disease (forced expiratory volume in 1 minute [FEV1], 52–137; mean, 102; SD, 15% predicted). HRCTs were scored by using a system that evaluates each lobe for severity and extent of CF lung disease. Findings of CF lung disease were tabulated in all subjects and in a subgroup with normal pulmonary function tests. HRCT scores were correlated with FEV1, forced vital capacity (FVC), and forced expiratory flow between 25% and 75% of expired vital capacity (FEF25-75) in 57 patients. ResultsBronchiectasis was present in 35% of subjects, mucous plugging in 15%, and air trapping in 63%. No abnormality was detected in 25%. In 37 subjects with FEV1, FVC, and FEF25-75 >85% predicted, bronchiectasis was present in 30%. In 17% of these subjects, bronchiectasis was seen in ≥4 lobes. Correlations between HRCT scores and FEV1 were significant and showed fair to moderate correlation (r=0.36–0.46). ConclusionsHigh-resolution computed tomography demonstrated a broad range of pulmonary abnormalities in young patients with CF with mild to moderate lung disease. In this study, abnormalities, including bronchiectasis, were common in young children with CF and normal pulmonary function tests.

Immunoglobulin therapy to control lung damage in patients with common variable immunodeficiency

Background: Lung damage progression is the most frequent condition in patients with common variable immunodeficiency (CVID). Appropriate immunoglobulin dose adjustments and follow-up guidelines to evaluate this have not been well established. Objective: To assess the evolution of lung damage once stable residual serum levels of IgG over 600 mg/dl had been achieved. Methods: A prospective study was conducted in 24 adult patients consecutively diagnosed with CVID, with no previous intravenous immunoglobulin (IVIG) treatment. IVIG dose, total serum IgG level, bacterial infection rate, pulmonary function tests (PFTs) and high resolution computed tomography (HRCT) of the thorax were monitored over 2 years. Moreover, outcome data were determined by measurement of chronic pulmonary disease (CPD). Results: IVIG dose variability (205–372 mg/kg/21 days) to obtain the required serum IgG levels was determined. Patients with CPD needed higher doses than those without CPD ( p=0.045). A significant reduction in severe and mild infections/patient-year was observed during treatment. Overall, there were no changes in PFTs and HRCT scores in patients without CPD, but both improved in patients with CPD. An increase of over 15% in overall HRCT score was detected in two patients without evidence of impairment in either clinical status or PFT values. Conclusions: Residual levels of total IgG over 600 mg/dl may help prevent progression of lung damage in patients with CVID. Levels of IgG, clinical manifestations and PFTs seem sufficient for routine follow-up. HRCT examination of the thorax, at least biennially, may help to identify patients in whom lung injury is progressing even though they may remain symptom-free and with stable PFTs.

Does Technetium-99m Diethylenetriaminepentaacetate Clearance Predict the Clinical Course of Idiopathic Pulmonary Fibrosis?

Clearance of inhaled technetium-99m diethylenetriaminepentaacetate (99mTc-DTPA) is a potential indicator of disease activity and progression in idiopathic pulmonary fibrosis (IPF). The objective of the present study was to evaluate the prognostic value of 99mTc-DTPA scans in IPF. A total of 22 patients (18 males), aged 33 to 80 years with IPF were followed for six to 20 months (mean 13 months). At diagnosis, high resolution computed tomography (HRCT) scans showed a honeycomb pattern with bibasilar reticular opacities in all cases. At T0 (diagnosis) and T1 (follow-up), each patient had pulmonary function tests (forced vital capacity, diffusing capacity of the lung for carbon monoxide and partial arterial O2 pressure), extension of fibrosis evaluated by HRCT visual score and 99mTc-DTPA lung clearance. Results at T0 and T1 were compared, taking into account the whole population and patients with relatively fast and slow 99mTc-DTPA wash-out. 99mTc-DTPA clearance did not show any significant correlation with functional tests or HRCT score. These findings indicate that clearance of inhaled 99mTc-DTPA is not of value in following the progress of IPF.

Retrospective review of children presenting with non cystic fibrosis bronchiectasis: HRCT features and clinical relationships

Non cystic fibrosis (CF) bronchiectasis in children presents with a spectrum of disease severity. Our aims were to document the extent and severity of disease in children with non-CF bronchiectasis, to review the inter- and intraobserver agreement for the high-resolution computed tomography (HRCT) features examined, and to assess correlations between HRCT features and clinical measures of severity. We performed a retrospective review of 56 children from the Starship Children's Hospital. HRCT scans were scored by a modified Bhalla system, and the chest X-rays using the Brasfield score. Scores were correlated with demographics, number of hospitalizations, disease duration, pulmonary function, clinical examination, and chronic sputum infection. The bronchiectasis seen was widespread and severe, particularly in Maori and Pacific Island children. The kappa coefficient for intraobserver agreement was better than that for interobserver agreement. Comparisons between HRCT scan and lung function parameters showed that the strongest relationships were between forced expiratory volume in 1 sec (FEV(1)) and forced expiratory flow between 25-75% of forced vital capacity (FEF(25-75)) with the extent of bronchiectasis, bronchial wall thickening, and air trapping. Children with digital clubbing and chest deformity showed significantly higher scores for extent of bronchiectasis, bronchial wall dilatation and thickness, and overall computed tomography (CT) score. No relationship was demonstrated between chronic sputum infection and CT score. The HRCT score demonstrated a stronger correlation between the extent and severity of bronchiectasis, and spirometry values, than the chest X-ray score. In conclusion, pediatric non-CF bronchiectasis in Auckland is extensive and severe. The good intraobserver ratings mean that consistency of scoring is possible on repeated scans. This study cannot comment on the relationships of CT and less severe disease.

Sarcoidosis: assessment of disease severity using HRCT.

The value of high-resolution computed tomography (HRCT) in diagnosing and assessing inflammatory activity in sarcoidosis is well established. The aim of the present study was to address the intra- and inter-observer agreements of the HRCT score by Oberstein et al. [8], and to evaluate the relationship between HRCT findings and disease severity expressed in respiratory functional impairment in sarcoidosis. The clinical records of 80 known sarcoidosis patients visiting the outpatient clinic between January 2000 and August 2001, who underwent a HRCT as well as lung function tests (including exercise testing), were reviewed. Two readers scored the first 60 HRCT images twice. Weighted kappa and intra-class correlation coefficient were used to assess the reliability of the HRCT scoring system. Spearman's rank correlation coefficients and multiple regression analyses were performed to evaluate the relationship between HRCT findings (first reading, reader A) and respiratory functional impairment. Intra- and inter-reader reliability demonstrated good agreement. All HRCT subscores, except enlargement of lymph nodes, were correlated to the FEV(1), FVC, DLco, Pao(2)max (all p<0.05) and A-aPo(2 )max ( p<0.001). Furthermore, HRCT abnormalities, but not the chest radiographic stage, were strongly associated with functional parameters. Abnormal changes of lung parenchyma, established by HRCT features, were associated with respiratory functional impairment in sarcoidosis. Moreover, compared with the radiographic stages, HRCT findings appeared to be much more sensitive in depicting respiratory disability, especially abnormal gas exchange.

Non-CF bronchiectasis: clinical and HRCT evaluation.

Childhood bronchiectasis not related to underlying disease is still common in some populations in affluent countries. The aims of the study were to: 1) describe demographics, 2) evaluate the effectiveness of routine investigations, and 3) determine the relationship between spirometry and radiology scoring systems, in children with chronic suppurative lung disease (CSLD) living in Central Australia. Data of children living in Central Australia aged </=15 years with noncystic fibrosis CSLD were identified over 2 years. Chest high-resolution computed tomography (HRCT) scans were interpreted by a pediatric radiologist and scored according to three systems. Sixty-five children were identified, resulting in a prevalence of at least 14.7/1,000 aboriginal children aged </=15 years. Comorbidities (most common: suppurative otitis media in >70%) and early hospitalisation for pneumonia were common (median age, 0.5 years). Previous admissions for pneumonia were almost universally present and significantly more common than bronchiolitis (95% CI for proportional difference, 0.4-0.51). Although the majority did not have a treatable underlying cause, investigations had significant impact on management in 12.3% of children. None of the chest HRCT scores related to any spirometry data. In conclusion, CSLD is unacceptably common in indigenous children of this region, commences early in life, and is associated with significant comorbidities. Spirometry data do not reflect the severity of lung disease in HRCT scans. While improvement in the living standards is of utmost importance, the medical management that includes thorough investigations of these children should not be neglected.

Tidal expiratory flow limitation, dyspnoea and exercise capacity in patients with bilateral bronchiectasis.

In this study the authors investigated whether expiratory flow limitation (FL) is present during tidal breathing in patients with bilateral bronchiectasis (BB) and whether it is related to the severity of chronic dyspnoea (Medical Research Council (MRC) dyspnoea scale), exercise capacity (maximal mechanical power output (WRmax)) and severity of the disease, as assessed by high-resolution computed tomography (HRCT) scoring. Lung function, MRC dyspnoea, HRCT score, WRmax and FL were assessed in 23 stable caucasian patients (six males) aged 56 +/- 17 yrs. FL was assessed at rest both in seated and supine positions. To detect FL, the negative expiratory pressure (NEP) technique was used. The degree of FL was rated using a five-point FL score. WRmax was measured using a cyclo-ergometer. According to the NEP technique, five patients were FL during resting breathing when supine but not seated, four were FL both seated and supine, and 14 were NFL both seated and supine. Furthermore, it was shown that: 1) in stable BB patients FL during resting breathing is common, especially in the supine position; 2) the degree of MRC dyspnoea is closely related to the five-point FL score; 3) WRmax (% pred) is more closely correlated with the MRC dyspnoea score than with the five-point FL score; and 4) HRCT score is closely related to forced expiratory volume in one second % pred but not five-point FL score. In conclusion, flow limitation is common at rest in sitting and supine positions in patients with bilateral bronchiectasis. Flow limitation and reduced exercise capacity are both associated with more severe dyspnoea. Finally, high-resolution computed tomography scoring correlates best with forced expiratory volume in one second.