Mixed infiltrative and obstructive disease on high-resolution CT: differential diagnosis and functional correlates in a consecutive series

Fourteen of 400 consecutive patients having high-resolution computed tomography (HRCT) with expiratory images showed findings of infiltrative lung disease on inspiratory HRCT and air trapping on expiratory CT. Diagnoses included hypersensitivity pneumonitis, sarcoidosis, atypical infection, and pulmonary edema. The extent of infiltrative abnormalities and air trapping were correlated with pulmonary function tests (PFT) in 11 patients. PFT indicated a mixed pattern in five, an obstructive pattern in three, and a restrictive pattern in three. Forced expiratory volume (FEV) in 1 second/forced vital capacity (FVC) correlated significantly with the extent of air-trapping (r = 0.60; p = 0.05). The extent of infiltrative abnormalities correlated significantly and negatively with forced vital capacity (r = -0.82, p = 0.002), FEV1 (r = -0.59, p = 0.05), total lung capacity (TLC) (r = -0.67, p = 0.05), and DLCO (r = -0.75, p = 0.02). Findings of lung infiltration on inspiratory HRCT scans and air trapping on expiratory CT correlated respectively with PFT measures of restrictive and obstructive lung disease.     

Pulmonary involvement in inflammatory bowel disease

AIM:To determine the relationship of pulmonary abnormalities and bowel disease activity in inflammatory bowel disease(IBD).METHODS:Thirty ulcerative colitis(UC)and nine Crohn’s disease patients,and 20 control subjects were enrolled in this prospective study.Detailed clinical information was obtained.Extent and activity of the bowel disease were established endoscopically.Each patient underwent pulmonary function tests and high-resolution computed tomography(HRCT).Blood samples for measurement of C-reactive protein(CRP),erythrocyte sedimentation rate(ESR),angiotensin converting enzyme and total IgE were delivered by the patients.RESULTS:Ten(25.6%)patients had respiratory symptoms.A pulmonary function abnormality was present in 22 of 39 patients.Among all patients,the most prevalent abnormalities in lung functions were a decrease in forced expiratory volume in 1 s(FEV1),FEV1/forced vital capacity(FVC),forced expiratory flow(FEF)25%-75%,transfer coefficient for carbon monoxide(DLCO),DLCO/alveolar volume.Increased respiratory symptoms score was associated with high endoscopic activity index in UC patients.Endoscopic and clinical activities in UC patients were correlated with FEV1,FEV1/FVC,and FEF 25%-75%.Smoking status,duration of disease and medication were not correlated with pulmonary physiological test results,HRCT abnormalities,clinical/endoscopic disease activity,CRP,ESR or total IgE level or body mass index.CONCLUSION:It is important that respiratory manifestations are recognized and treated early in IBD.Otherwise,they can lead to destructive and irreversible changes in the airway wall.     

Quantitative assessment of pulmonary function in lymphangioleiomyomatosis patients using high-resolution computed tomography and pulmonary function tests

BackgroundTo explore the feasibility of using quantitative high-resolution computed tomography (HRCT) to evaluate pulmonary function in patients with pulmonary lymphangioleiomyomatosis (PLAM).MethodsPulmonary function tests (PFTs) were performed in 30 patients with pathologically confirmed PLAM with the use of HRCT. These results were correlated with quantitative HRCT in 21 patients.ResultsThere were significant correlations between the HRCT parameters for lung function and PFT parameters. Among these parameters, emphysema volume (EV), pulmonary volume with a pixel index less than the trigger threshold (−950 HU) to account for a proportion of total lung volume [PI-950 (%)] and forced expiratory volume in 1 second/forced vital capacity [FEV1/FVC (%)] had the strongest correlations, reaching values between −0.71 and −0.68. HRCT lung function might therefore also be helpful for predicting changes in lung function before and after treatment.ConclusionsHRCT is helpful for the assessment of pulmonary function in PLAM patients and can assist in the clinical evaluation of lung function and treatment response in patients with this disease.     

Quantitative computed tomography detects peripheral airway disease in asthmatic children

The aim of this study was to compare air-trapping as quantified by high-resolution computed tomography (HRCT) of the chest with measures of lung function and airway inflammation in children with mild to moderate asthma. Plethysmography indices, respiratory resistance, and reactance before and after bronchodilator with impulse oscillation (IOS), exhaled nitric oxide (eNO), total eosinophil count (TEC), and serum eosinophil cationic protein (ECP) levels were measured in 21 subjects. A single-cut HRCT image at end-expiration was obtained. Air-trapping was quantified and expressed in terms of the pixel index (PI) by determining the percentage of pixels in lung fields below -856 and -910 Hounsfeld units (HU). Pairwise linear correlations between PI and other parameters were evaluated. Subjects had only mild airflow limitation based on prebronchodilator forced expiratory volume in 1 sec (FEV(1)), but were hyperinflated and had air-trapping based on elevated total lung capacity (TLC) and residual volume (RV)/TLC ratio, respectively. The PI at -856 HU was positively correlated with % predicted TLC, total gas volume (TGV), and ECP level, and was inversely correlated with FEV(1)/forced vital capacity (FVC) and % predicted forced expiratory flow between 25-75% FVC (FEF(25-75)). The PI at -910 HU correlated similarly with these variables, and also correlated positively with IOS bronchodilator reversibility. This data suggest that quantitative HRCT may be a useful tool in the evaluation of peripheral airflow obstruction in children with asthma.     

A comparison of the end-tidal CO2 measured by portable capnometer and the arterial PCO2 in spontaneously breathing patients

An end-tidal CO2 (ETCO2) monitor (capnometer) is used most often as a noninvasive substitute for PaCO2 in anesthesia, anesthetic recovery and intensive care. However, the utility and accuracy of the portable capnometer in spontaneously breathing patients with or without chronic pulmonary diseases has received little recognition. To determine the utility of the portable capnometer in general wards and in in-home care, we examined the correlation between ETCO2 measured by a portable capnometer and simultaneous PaCO2 measured in 41 spontaneously breathing patients. TV-ETCO2 (ETCO2 measured by tidal volume maneuver) was lower than PaCO2 by an average of 9.0 mmHg and VC-ETCO2 (ETCO2 measured by vital capacity maneuver) was lower than PaCO2 by an average of 0.5 mmHg. The mean difference between PaCO2 and VC-ETCO2 was not statistically significant. Regression analysis showed a close correlation between VC-ETCO2 and PaCO2 (r = 0.91, P < 0.0001). Thus,VC-ETCO2 was highly correlated with PaCO2. Furthermore, a close correlation between VC-ETCO2 and PaCO2 was also observed in patients with compromised pulmonary function (r = 0.88, P < 0.0001 in patients with below 70% of FEV(1.0)%; r = 0.89, P < 0.0001 in patients with below 80% of %VC). Our studies show that VC-ETCO2 measured by the portable capnometer gives a reliable pointestimate of PaCO2, and can be useful to evaluate the respiratory condition of spontaneously breathing patients in general wards and in in-home care.     

Functional significance of the decreased attenuation sign on expiratory CT in pulmonary sarcoidosis : report of four cases

We describe four patients with proven sarcoidosis and minor pulmonary involvement according to high-resolution CT (HRCT) findings in whom the recently described sign of decreased attenuation on expiratory HRCT scan appeared associated with the reduction of the single-breath diffusing capacity of the lung for carbon monoxide (DLCO) and the DLCO adjusted for alveolar volume. These alterations were, in part, reversible under steroid treatment. Major indexes of airway obstruction (FEV(1)/vital capacity ratio and FEV(1)) were normal, while the maximum expiratory flow at 25% above the residual volume of FVC was reduced. These observations suggest that an expiratory HRCT mosaic pattern and diffusion impairment may be early findings in pulmonary sarcoidosis and may be useful for its detection and follow-up.     

The effects of azithromycin on patients with diffuse panbronchiolitis: a retrospective study of 29 cases

Background

Diffuse panbronchiolitis (DPB), a chronic inflammatory disease of the airway, is treated with macrolide antibiotics. The ability of azithromycin to improve DPB prognosis, as detected by high-resolution computed tomography (HRCT) scans and lung function tests, has not been studied in a large retrospective of patients. Our study aims to investigate the effects of azithromycin on patients with DPB using lung function tests and radiologic images.

Methods

Twenty-nine patients with DPB were studied; their medical records were collected and analyzed retrospectively. Patients studied were hospitalized in the respiratory department of the Yixing Hospital, affiliated with Jiangsu University. Azithromycin was administered for 6-17 months. Changes in lung function and HRCT scans after treatment with azithromycin for six months were compared with pre-treatment values and images respectively.

Results

Azithromycin therapy for six months resulted in rapid improvements in lung function, demonstrated by forced expiratory volume in one second (FEV1.0%), forced expiratory volume in one second over the forced vital capacity (FEV1.0/FVC), and forced expiratory volume with 75% vital capacity (FEF75%) values. In addition, improvements were seen in small nodular shadows, dilated peripheral bronchi, bronchial wall thickening, and tree-in-bud pattern, as detected by chest HRCT scans.

Conclusions

Long-term therapy with azithromycin is effective for patients with DPB.KEYWORDS : Diffuse panbronchiolitis (DPB), azithromycin, lung function, high-resolution computed tomography (HRCT)     

Pulmonary function and high-resolution CT findings five years after disease onset in patients with Wegener's granulomatosis

OBJECTIVE: Although pulmonary involvement is common in Wegener's granulomatosis (WG), little is known about the pulmonary outcome. We evaluated the relationship between clinical disease characteristics and pulmonary function and high-resolution computed tomography (HRCT) findings after disease duration of 5 years. METHODS: A pulmonary function test (PFT) and pulmonary HRCT were performed in 41 patients from a population-based register of WG. Clinical predictors for abnormal PFT and HRCT were tested by logistic regression. RESULTS: Previous WG-related lung involvement (PLI) had occurred in 80% of patients, but only 24% of patients still reported pulmonary symptoms at the research visit. One-third of patients had abnormal PFT findings, with reduced alveolar diffusion by KCO (transfer coefficient) being most common (24%). The number of PLI episodes was associated with reduced KCO and reduced FEV1% (forced expiratory volume in 1 s as a percentage of forced vital capacity) (overall presence 10%). Reduced KCO was also associated with disease duration. Reduced total lung capacity (TLC) (overall presence 8%) was only related to prior WG-related lung nodules. Pulmonary HRCT was abnormal in 80%, but with more severe abnormalities in only 30%. Pleural thickening and parenchymal bands were associated with PLI. None of the treatment variables was associated with the PFT or HRCT findings. CONCLUSION: Five years after disease onset a quarter of the WG patients reported pulmonary symptoms, had severe abnormalities on HRCT, and abnormal PFT. The correlation between these abnormalities was poor, but the number of pulmonary involvements was a risk factor for reduced gas diffusion, obstructive lung disease, parenchymal bands, and pleural thickening. Treatment variables had no discernible negative pulmonary effects.     

Patterns of lung disease in a "normal" smoking population: are emphysema and airflow obstruction found together?

STUDY OBJECTIVES: We determined whether emphysema demonstrated on high-resolution CT (HRCT) scanning in apparently well smokers is associated with airflow obstruction. INTERVENTIONS: Lung function testing and limited HRCT scanning. DESIGN: Lung function measurements and scans were analyzed independently of each other. We used analysis of covariance to compare FEV(1) and maximum expiratory flow at 50% of vital capacity (MEF(50)) values after suitable corrections, between subjects with and without parenchymal damage (emphysema and/or reduced carbon monoxide transfer coefficient [KCO]), and to compare indexes of parenchymal damage between subjects with and without airflow obstruction. SETTING: Radiology and lung function departments of a district general hospital. PARTICIPANTS: Eighty current cigarette smokers and 20 lifetime nonsmoking control subjects (aged 35 to 65 years) who volunteered following publicity in local media. In all subjects, FEV(1) was > 1.5 L; no subjects were known to have lung disease. Measurements and results: FEV(1) and MEF(50) were measured spirometrically; static lung volumes were measured by helium dilution and body plethysmography; KCO was measured by a single-breath technique. HRCT scans were analyzed for emphysema by two radiologists. Of smokers, 25% had HRCT emphysema, generally mild; 16.3% and 25% had reduced FEV(1) and MEF(50), respectively; 12.5% had reduced KCO. Smokers with airflow obstruction were not more likely to have parenchymal damage. Smokers with parenchymal damage did not have reduced airway function. Nonsmokers generally had normal airways and parenchyma. CONCLUSIONS: "Normal" smokers with lung damage had either airflow obstruction or parenchymal damage, but not generally both.     

Zinc chloride (smoke bomb) inhalation lung injury: clinical presentations, high-resolution CT findings, and pulmonary function test results

STUDY OBJECTIVES: Zinc chloride smoke inhalation injury (ZCSII) is uncommon and has been rarely described in previous studies. We hypothesized that structural changes of the lung might correlate with pulmonary function. To answer this question, we correlated findings from high-resolution CT (HRCT) scan and the results of pulmonary function tests (PFTs) in patients with ZCSII. DESIGN: Retrospective cohort study. SETTING: University hospital. PATIENTS: Twenty patients who had been hospitalized with ZCSII-related conditions. MEASUREMENTS: The study included HRCT scan scores (0 to 100), static and dynamic lung volumes, and diffusing capacity of the lung for carbon monoxide (D(LCO)). RESULTS: HRCT scans and PFTs were performed initially after injury (range, 3 to 21 days) in all patients and during the follow-up period (range, 27 to 66 days) in 10 patients. The predominant CT scan findings were patchy or diffuse ground-glass opacities with or without consolidation. The majority of patients showed a significant reduction of FVC, FEV1, total lung capacity, and D(LCO), but normal FEV1/FVC ratio values. Changes of functional parameters correlated well with HRCT scan scores. Substantial improvements in CT scan abnormalities and pulmonary function were observed at follow-up. CONCLUSIONS: The majority of our patients with ZCSII presented with a predominant parenchymal injury of the lung that was consistent with a restrictive type of functional impairment and a reduction in Dlco rather than with obstructive disease. Our results suggest that HRCT scanning and pulmonary function testing may reliably predict the severity of ZCSII.     

肺泡蛋白沉着症患者的肺功能与胸部分辨率CT的相关性

目的 探讨PAP患者的肺功能与高分辨率CT定量测量指标之间的相关性.方法 对2004至2007年在广州医学院第一附属医院呼吸疾病研究所住院的17例PAP患者(男10例,女7例,年龄15～51岁)进行肺通气及弥散功能检查和高分辨率CT检查,分析肺功能指标与高分辨率CT定量指标之间的相关性.比较其中6例行全肺肺泡灌洗治疗前、后的肺功能和高分辨率CT定量指标的变化.采用SPSS 13.0软件进行数据处理,结果 以x±s表示.结果 PAP患者的肺功能检查结果 以限制性通气功能障碍和弥散功能下降为主,弥散功能下降更为显著,DLCO占预计值%为(46±22)%,异常率为94.1%.肺功能检查结果 与高分辨率CT定量检查结果 之间存在一定的相关性,其中平均肺密度与FVC、肺重量与FEV1、肺含气容积比与呼气峰流量、DL.CO、DLCO与肺泡通气量比值(DLCO/VA)等指标的改变差异有统计学意义,(r值分别为-0.469、-0.482、0.511、0.659、0.692,均P<0.05),在所有指标中以肺含气容积比与DLCO/VA关系最为密切.6例肺泡灌洗术治疗的患者术后肺功能和高分辨率CT定量测量指标有所改善.结论 PAP的肺功能和胸部高分辨率CT改变之间存在相关性.2项检查同时进行可作为PAP患者随访的重要参考指标.     

Assessment of emphysema in COPD: a functional and radiologic study

OBJECTIVES: A combination of functional measurements reflecting a decrease in maximum flow, a degree of lung hyperinflation, the relationship between maximum inspiratory and expiratory flows, bronchodilator response, and diffusing capacity of the lung for carbon monoxide (DLCO) was used to quantify the extent of emphysema, as assessed by high-resolution CT (HRCT) scanning. DESIGN: Forced inspiratory and expiratory spirometry, lung volumes, reversibility test, and single-breath diffusing capacity were assessed before and after inhaling albuterol, 200 microg. Relationships between lung function variables and emphysema extent, as determined by HRCT scanning, were tested by univariate and multivariate analyses. SUBJECTS: Thirty-nine COPD outpatients with moderate-to-severe obstruction. MEASUREMENTS AND RESULTS: Emphysema extent, as assessed by HRCT scanning, ranged from 18 to 70%. All of the lung function parameters that were studied, except for the change in FEV1 percent predicted after salbutamol inhalation, correlated significantly with the extent of emphysema (r2 range, 0.19 to 0.44). Functional residual capacity, forced expiratory flow at 50% of FVC/forced inspiratory flow at 50% of FVC, DLCO/alveolar volume ratio, and bronchodilator-induced change in FEV1/FVC ratio were the only variables retained by stepwise multiple regression analysis. The multiple regression model explained 71% of the variability of emphysema extent measured by HRCT scanning. CONCLUSIONS: The combination of lung function measurements reflecting lung hyperinflation, bronchial collapsibility, lung diffusing capacity, and bronchodilator response provides a good estimate of the extent of emphysema, as evaluated by HRCT scanning. These data suggest that pulmonary function tests are useful in assessing and monitoring parenchymal damage in COPD patients.     

Growth of lung function in children with sickle cell anemia

Lung disease is a common cause of morbidity among children with sickle cell disease (SCD). Although cross-sectional studies of children with SCD describe abnormal pulmonary function, the pattern of lung function growth in these children compared to children in the general population is not known. To provide preliminary evidence that growth of lung function is attenuated in children with SCD, we conducted a retrospective cohort study of children with hemoglobin SS (HbSS) ages 6-19 years who received at least two spirometry assessments for clinical care. The growth of lung function in these cases was compared to age, gender, and race-specific children without SCD or respiratory complaints from the Harvard Six Cities Study (H6CS). Seventy-nine children with HbSS contributed 363 spirometry measurements (mean per child = 4.6, median = 4.0, range = 2-17) and 255 controls contributed 1,543 spirometry measurements (mean per child = 6.1, median = 6.0, range = 2-13). Longitudinal forced expiratory volume in 1 sec (FEV(1)) was lower for boys and girls with HbSS compared to children in the general population, P = 0.031 and P = 0.002, respectively. When compared to the H6CS cohort, girls with HbSS showed lower longitudinal forced vital capacity (FVC) (P < 0.001) and FEV(1)/FVC (0.038); there was no difference in FVC or FEV(1)/FVC between boys in the HbSS and H6CS cohort. We conclude that growth of lung function is reduced in children with HbSS compared to children in the general population. Gender may influence the risk of developing abnormal lung function and airway obstruction in children with HbSS.     

Spirometrically gated high-resolution CT findings in COPD: lung attenuation vs lung function and dyspnea severity

STUDY OBJECTIVES: The aim of this study was to investigate the relationship between high-resolution CT (HRCT) lung attenuation measurements, acquired under spirometric control of inspiratory and expiratory lung volume, and pulmonary dysfunction as well as dyspnea severity in patients with COPD.Patients and design: In 51 patients with COPD, we compared by linear regression, univariate and multivariate logistic regression airflow limitation (FEV(1)/vital capacity [VC]), hyperinflation (percentage of predicted residual volume [RV%]), parenchymal loss (percentage of predicted diffusing capacity of the lung for carbon monoxide [Dlco%]), and Medical Research Council (MRC) dyspnea scale with relative area with attenuation values < - 950 HU at 90% of VC [RAI(950)] and < - 910 HU at 10% of VC, respectively, and with mean lung attenuation measured at the same levels of VC (mean CT lung density at 10% of VC, and mean CT lung density at 90% of VC [MeanCTEXP]). RESULTS: All HRCT attenuation measurements were significantly related with functional abnormalities and dyspnea severity. In multivariate logistic models, with 1 indicating worse changes in dichotomous outcome variables, MeanCTEXP independently predicted FEV(1)/VC (odds ratio [OR], 0.24; 95% confidence interval [CI], 0.11 to 0.56), RV% (OR, 0.57; 95% CI, 0.42 to 0.77), and MRC dyspnea scale (OR, 0.63; 95% CI, 0.48 to 0.82), while RAI(950) independently predicted Dlco% (OR, 1.90; 95% CI, 1.37 to 2.65). CONCLUSIONS: Spirometrically gated measurements of HRCT lung attenuation reflect differently functional changes and dyspnea perception in COPD. Inspiratory measurements assess the extent of emphysematous tissue loss, and expiratory measurements may reflect airflow limitation and lung hyperinflation with attendant dyspnea perception. Pulmonary dysfunction in COPD cannot be assessed by a single modality of lung attenuation measurement.     

Detecting early structural lung damage in cystic fibrosis

In cystic fibrosis (CF) patients, both severe lung inflammation and severe lung damage occur early and persist throughout life. High-resolution computed tomography (HRCT), a more sensitive method of detecting structural abnormalities than chest X-ray, shows that airways undergo substantial thickening in early CF lung disease. Lung function tests, which are an indirect measure of structural integrity, are insensitive to localized or early damage. Thickening of the peripheral airways causes a reduction in maximal expiratory flow at 25% of forced vital capacity (MEF(25)) or other measurements of peripheral air flow. Reduced peripheral flows, even in the presence of normal forced expired volume in 1 sec (FEV(1)) and forced vital capacity (FVC), should be considered an early sign of substantial lung damage and should stimulate aggressive treatment to prevent further deterioration.     

Correlation of high-resolution CT, symptoms, and pulmonary function in patients during recovery from severe acute respiratory syndrome

STUDY OBJECTIVES: Little is known of the nature of the recovery period after severe acute respiratory syndrome (SARS) infection. We hypothesized that structural changes of the lung might correlate with symptoms and pulmonary function. To answer this question, we correlate findings of high-resolution CT (HRCT) with dyspnea scores and results of pulmonary function tests in patients during recovery from SARS. DESIGN: Retrospective follow-up cohort study. SETTING: University hospital. PATIENTS: Nineteen patients who recovered from SARS-related hospitalization. MEASUREMENTS: The study included HRCT scores (0 to 100), dyspnea scores (1 to 4), static and dynamic lung volumes, and diffusing capacity of the lung for carbon monoxide (DLCO). RESULTS: The interval between hospital discharge and HRCT study or functional assessment was 31.2 +/- 4.8 days (range, 25 to 38 days) [mean +/- SD]. All patients had HRCT abnormalities and were assigned to two groups: ground-glass opacity (GGO) only (n = 7, 36.8%) and GGO with fibrosis (GGO+F) [n = 12, 63.2%]. Most patients (16 of 19, 84.2%) had no zonal predominance. HRCT scores correlated well with dyspnea scores (r = 0.78, p < 0.01) and with a variety of pulmonary functional variables, with DLCO being the most significant (r = - 0.923, p < 0.001). Compared with the GGO group, the GGO+F group showed significantly lower FEV(1), FVC, total lung capacity, residual volume, and DLCO. CONCLUSIONS: HRCT findings correlate well with functional studies and clinical symptoms during recovery from SARS. Longer-term follow-up studies in a larger cohort of patients should be performed to investigate the clinical outcome of recovered SARS patients.     

Longitudinal changes in lung function and somatic growth in children with sickle cell disease

BACKGROUND: We studied the changes in the patterns of lung function and somatic growth over time in children and adolescents (10.6 +/- 3.5 years at first test) with hemoglobin SS (Hb-SS) sickle cell disease (SCD). METHODS: Lung function and somatic growth were measured twice with an interval of 42.3 +/- 23.3 months in 45 children (25 females and 20 males) with Hb-SS SCD. RESULTS: The lung volumes slightly decreased but remained borderline normal in both tests. All spirometric indices were within the normal range but significantly decreased (P<0.001) at the time of the second test indicating development of lower airway obstruction (forced expiratory volume in the first second (FEV(1)): 87 +/- 21 vs. 80 +/- 15; FEV(1)/forced vital capacity (FVC): 89 +/- 7 vs. 85 +/- 6; FEF(25-75): 89 +/- 32 vs. 76 +/- 24). "Normal" pattern of lung function was initially found in 56% of the patients, but in only 29% in the second test. In contrast, those with "obstructive" pattern increased from 22 to 44%, and those with "restrictive" pattern from 22 to 27%. There was no association between history of asthma and pattern of lung function. "Normal" Body Mass Index (BMI) was found in 64% of the patients, whereas 13% had "High" BMI and 22% "Low" BMI. The two latter patterns were associated with abnormal lung function but only patients with normal BMI showed actual decline overtime. CONCLUSION: SCD is characterized by a predominantly obstructive pattern of lung function that increases in prevalence over time. There was no apparent causal relationship between the pattern of somatic growth and the pattern of lung function.     

Pulmonary abnormalities on high-resolution CT demonstrate more rapid decline than FEV1 in adults with cystic fibrosis

BACKGROUND: FEV1 may remain stable while high-resolution CT (HRCT) appearances deteriorate in children with cystic fibrosis (CF). However, spirometry results commonly decline in older age groups. OBJECTIVES: To compare the rate of decline in HRCT abnormalities and spirometry results over time in an adult cohort with CF. METHODS: The HRCT scans of 39 consecutive patients (19 males and 20 females; mean age, 22 years; range, 16 to 48 years) with two HRCT scans > 18 months apart were randomly and blindly scored using a modified Bhalla scoring system by two independent chest radiologists. Age, body mass index, spirometry, and sputum cultures were recorded at the time of both HRCTs. Rates of change in clinical parameters and HRCT abnormalities were calculated and compared using repeated-measures analysis of variance. RESULTS: Mean FEV1 declined at a rate of - 2.3% per year, while mean HRCT total score declined at a rate of -2.7% per year. Several individual HRCT abnormalities as well as HRCT total scores declined significantly faster than FEV1 (p < 0.001). Six patients showed stable spirometry results but worsening HRCT scores. Mucus plugging and extent of bronchiectasis deteriorated at a more rapid rate in the group with mildly impaired lung function. Air trapping, collapse/consolidation, peribronchial thickening, severity of bronchiectasis, and generations of bronchial divisions involved deteriorated at a more rapid rate in the group with moderate-to-severely impaired lung function. CONCLUSIONS: Adult CF patients have more rapid rates of decline in HRCT abnormalities than in spirometry results. Individual HRCT abnormalities decline at different rates depending on the degree of lung function impairment.     

Lung dysfunction of chronic smokers with no signs of COPD

Cigarette smoking causes airflow limitation with lung hyperinflation being the primary causes of COPD. Fifty chronic smokers (CSs) with no signs of GOLD-adjusted COPD with smoking habit at least ≥10 pack-years (p/yrs) were divided into CS-mild (n = 24) with smoking history from ≥10 to ≤20 p/yrs and CS-heavy groups (n = 26) with smoking history ≥21 p/yrs. Spirometry, plethysmography and diffusing capacity were measured and lung computed tomography (CT) was performed. Residual volume (RV) (L) and RV/TLC (total lung capacity) ratio were significantly increased in CS-heavy when compared to CS-mild (p = 0.001, p = 0.03). A significant reduction of forced expiratory volume in 1 second/forced vital capacity (FEV(1)/FVC) ratio and airway specific conductance was shown in CS-heavy (p = 0.02, p = 0.03). Lung emphysema signs at CTs were revealed in 17 CSs and ten of them had declined diffusing capacity below 70% of predicted. The percentage of emphysematous lesions inversely and significantly correlated with measured diffusing capacity (p = 0.0009, r = --0.72). Study groups' smoking intensity inversely correlated the declined airway specific conductance (p = 0.004, r = --0.39) and increase of the RV (L) (p = 0.0004, r = 0.46). Multiple regression analysis determined that smoking intensity regardless of the subjects' age was significant factor for decline of airway specific conductance and increase of RV (L). Here we conclude that lung function deviation and lung structural changes are present in CSs before the clinical signs of airway obstruction reveal. Body plethysmography and diffusing capacity measurement with routine spirometry can provide valuable information for detection of changes reflecting to the early onset of COPD in CSs.     

Pulmonary function tests, high-resolution computerized tomography, α1-antitrypsin measurement, and early detection of pulmonary involvement in patients with systemic sclerosis

OBJECTIVE: Pulmonary disease represents a major complication of systemic sclerosis (SSc). However, pulmonary involvement is commonly silent. In this study, we investigated the relationship between serum alpha1-antitrypsin and other means of assessing pulmonary involvement. METHODS: Twenty-two patients affected by SSc were studied (mean age 37.6+/-14.3 years, mean duration of disease 9.9+/-11.9 years). Fourteen had the diffuse form of disease (dSSc) and eight had the limited form (lSSc). All patients underwent pulmonary function tests, high-resolution computed tomography (HRCT) of the lungs, echocardiography, and serum assessment of alpha1-antitrypsin. RESULTS: Mean percentage of predicted values of forced vital capacity was lower in patients with dSSc than with lSSc (72.3+/-17.8 vs 74.5+/-8, P=NS). Mean percentage of predicted values of forced expiratory volume in 1-s forced vital capacity (FEV1/FVC) was lower in patients with lSSc (79.8+/-7.5 for lSSc vs 84.4+/-7.8 for dSSc, P= NS). The overall HRCT score was 5.6+/-5.9 with no significant difference between disease subgroups. Pulmonary hypertension was detected in two cases, both with dSSc. Alpha1-antitrypsin was significantly higher in patients than in controls (P < 0.01), with no significant difference between disease subgroups, and correlated significantly with ground glass opacities in H RCT (P < 0.05) and the detection of diffusion defects (r= -0.61, P<0.01). No significant correlation was observed between skin score or degree of dyspnea with HRCT score, lung volume, or carbon monoxide diffusing capacity. CONCLUSION: Restrictive lung disease was more pronounced in patients with dSSc. Alpha1-antitrypsin levels correlated significantly with ground glass opacities, an early finding of pulmonary involvement in SSc. Extent and severity of skin involvement and degree of dyspnea were not related to pulmonary involvement.