HRCT Score Research Articles

Clinical Experience with Pirfenidone in Connective Tissue Diseases Related Interstitial Lung Diseases

Objective: Pirfenidone is a new, anti-fibrotic drug used for the treatmentof idiopathic pulmonary fibrosis (IPF). This study was designed to verify the effect of pirfenidone administrated in connective tissue diseases related interstitial lung disease (CTD-ILD). Methods: Twenty two patients diagnosed with CTD-ILD were administrated with a 6-months treatment with or without pirfenidone. At baseline and at months 3rd and 6th, pulmonary function tests, six minute walk distance (6MWD) and HRCT scores were performed. Results: PFD group presented a significant improvement in TLC in the 3rd and 6th month points and DLco was greatly increased in 6th month point, which were all adjusted by the baseline levels, compared with control group (p=0.043, 0.048 and 0.043, respectively). During the follow-up period, TLC was also improved compared to the initial time point and DLco increased obviously in the 6th month point in PFD group (p=0.005, 0.004 and p <0.001 respectively). A lower ground glass score and fibrotic score in the PFD group approached statistical significance after 6-months treatment with pirfenidone when compared with control group (p=0.037 and 0.018, respectively) and decreased greatly in the 6th month point when compared to the former time points in PFD group (p=0.006 and 0.013, respectively), whereas no such changes were observed in the control group. Conclusion: These results suggest that pirfenidone could improve the pulmonary function tests and HRCT scores of connective tissue diseases related interstitial lung disease. It is a potential therapeutic candidate for treatment.

Evaluating Qualitative and Quantitative Computerized Tomography Indicators of Chronic Obstructive Pulmonary Disease and Their Correlation with Pulmonary Function Tests.

SummaryBackgroundWith increasingly aging populations, chronic obstructive pulmonary disease (COPD) is the fourth leading cause of death today. Emphysematous changes, an important component of the disease, must be determined on HRCT, either qualitatively or quantitatively. The purpose of this study was to evaluate features that help determine emphysematous changes and correlate them with respiratory function tests (RFTs).Material/MethodsA total of thirty COPD patients and a control group of the same size, matched for age, were included in the study. The mean lung parenchyma density values on inspiration and expiration, visual HRCT scores, and pulmonary function tests were obtained. IBM SPSS statistical software (version 22) was used to perform correlation analysis (Pearson’s coefficient) and the Mann-Whitney U test.ResultsThe most valuable RFTs for determining emphysematous changes were DLCO, FEV1, and FEV1/FVC, in that order. Quantitative measures of the mean lung density had the highest correlation with coefficient on expiration.ConclusionsAs regards the comparison between objective and subjective density values, the HRCT-based visual density values are satisfactory. On the other hand, the best assessment can be performed with the use of mean density values on expiration. DLCO, FEV1, and FEV1/FVC were found to be valuable parameters in determining parenchymal changes.

A retrospective cohort study of outcome in systemic sclerosis-associated interstitial lung disease.

The relationship between the histological pattern and survival in systemic sclerosis-associated interstitial lung disease (SSc-ILD) is unclear. In patients with SSc-ILD, we investigated whether the clinical data obtained by non-invasive examinations could be used for prognostic evaluation, and attempted to clarify whether complicating acute exacerbation (AE) and the selection of pharmacological therapy were associated with survival. Thirty-five patients with SSc-ILD, who had not been diagnosed by surgical lung biopsy were analyzed, retrospectively. The HRCT findings were evaluated by 2 radiologists and classified into "CT-UIP" or "CT-inconsistent with UIP" patterns based on whole lung interpretations. HRCT scores were calculated based on the extent of abnormality evidenced by HRCT. The log-rank test was used to determine variables, including clinical parameters and histories. Twelve (34%) of the 35 patients died during a median follow-up period of approximately 7.9 years. The log-rank test showed that a higher mortality was associated with higher age, a CT-UIP pattern, a higher score for ground-glass attenuation with traction bronchiectasis on HRCT, and complicating AE, whereas a lower mortality was significantly associated with the use of immunosuppressants. A CT-UIP pattern was significantly associated with a higher incidence of later AE. Treatment with immunosuppressants was associated with a longer survival, and complicating AE is a predictor of shortened survival in SSc-ILD patients. Among the clinical parameters determined by non-invasive examinations, a CT-UIP pattern and the extent of fibrotic lesions on HRCT, but not a histological pattern of UIP, may be predictors of shortened survival.

Computer-Aided Tomographic Analysis of Interstitial Lung Disease (ILD) in Patients with Systemic Sclerosis (SSc). Correlation with Pulmonary Physiologic Tests and Patient-Centred Measures of Perceived Dyspnea and Functional Disability.

ObjectivesThis study was designed (a) to evaluate an improved quantitative lung fibrosis score based on a computer-aided diagnosis (CaM) system in patients with systemic sclerosis (SSc),—related interstitial lung disease (SSc-ILD), (b) to investigate the relationship between physiologic parameters (forced vital capacity [FVC] and single-breath diffusing capacity for carbon monoxide [DLCO]), patient-centred measures of dyspnea and functional disability and CaM and visual reader-based (CoVR) methods, and (c) to identify potential surrogate measures from quantitative and visual HRCT measurement.Methods126 patients with SSc underwent chest radiography, HRCT and PFTs. The following patient-centred measures were obtained: modified Borg Dyspnea Index (Borg score), VAS for breathing, and Health Assessment Questionnaire-Disability Index (HAQ-DI). HRCT abnormalities were scored according to the conventional visual reader-based score (CoVR) and by a CaM. The relationships among the HRCT scores, physiologic parameters (FVC and DLCO, % predicted) results and patient-centred measures, were calculated using linear regression analysis and Pearson’s correlation. Multivariate regression models were performed to identify the predictor variables on severity of pulmonary fibrosis.ResultsSubjects with limited cutaneous SSc had lower HAQ-DI scores than subjects with diffuse cutaneous SSc (p <0.001). CaM and CoVR scores were similar in the 2 groups. In univariate analysis, a strong correlation between CaM and CoVR was observed (p <0.0001). In multivariate analysis the CaM and CoVR scores were predicted by DLco, FVC, Borg score and HAQ-DI. Age, sex, disease duration, anti-topoisomerase antibodies and mRSS were not significantly associated with severity of pulmonary fibrosis on CaM- and CoVR methods.ConclusionsAlthough a close correlation between CaM score results and CoVR total score was found, CaM analysis showed a more significant correlation with DLco (more so than the FVC), patient-centred measures of perceived dyspnea and functional disability. Computer-aided tomographic analysis is computationally efficient, and in combination with physiologic and patient-centred measures, it could allow a means for accurately assessing and monitoring the disease progression or response to therapy.

Abstract P1-16-03: Quantitative versus semi-quantitative assessments of radiation-induced pulmonary fibrosis post adjuvant breast radiotherapy

Abstract Objective To evaluate the quantitative versus semi-quantitative assessments of radiation induced pulmonary fibrosis (RIPF) post adjuvant breast radiotherapy (RT). Methods High resolution computed tomography (HRCT) assessed lung physical density changes (CTD) and physician identified HRCT visual grading scores (CTS) were analysed at the minimum of 12 months post RT at one institution. The treated side in-portal lung regions for CTD and CTS assessments were: central-axis (CA) + regions 5cm superior &amp; inferior to CA and the corresponding mid anterolateral region respectively. Respiratory motion was accounted for by subtracting the untreated side lung density from the treated side. Mean lung densities correspond to each voxels were automatically calculated by Pinnacle software (Phillips, Eindhoven, The-Netherlands). Grading of CTS was according to the RTOG/EORTC (grade 0, 1, 2, and 3 defined as none, slight, patchy, and dense HRCT appearance respectively) and analysed by a radiologist (JL) and re-checked a radiation oncologist (PG). Results Total numbers of 403 patients were analysed. A substantial association was verified between CTD and CTS assessment. An increase of ∼0.01 g/ml (95% CI 0.003-0.02) in CTD with each CTS score increase of 1 was observed (Table-1a). The RIPF can be categorised quantitatively into three groups of CTS 0 vs. 1-2 vs. 3 based on the mean CTD (Table-1b). Table-1a. Correlation between CTD and CTS method. Table-1b. Grouping of CTS based on CTD methodTable-1a. Correlation between CTD and CTS methodCTSTreated side mean CTD - LeftTreated side mean CTD - RightMean Total00.055 (119)-0.011 (136)0.020 (255)10.065 (60)0.005 (56)0.036 (116)20.083 (15)0.012 (13)0.050 (28)30.108 (4)00.108 (4)Total0.061 (198)-0.005 (205)0.028 (403)Table-1b. Grouping of CTS based on CTD methodCTSTreated side mean CTD - LeftTreated side mean CTD - RightMean Total95% Confidence interval00.055 (119)-0.011 (136)0.020 (255)0.012-0.0271-20.069 (75)0.006 (69)0.039 (144)0.029-0.04830.108 (4)00.108 (4)0.079-0.137 A cut off CTD of 0.089 g/ml exemplified the best compromise between sensitivity (100%) and specificity (88.2%) for dense HRCT appearance. However, there was no good compromise of CTD cut off for slight and patchy HRCT score possibly due to intra observer variation and the scale of the CTD measure (small increase in CTD may not be detected visually by the observer). Multivariable analysis revealed increasing age, current smoker, V20 ≥ 10% (the volume of lung that was covered by the 20Gy isodose line), central lung distance ≥ 2cm (the distance between posterior RT tangents and the chest wall), combined endocrine &amp; chemotherapy, and treated side mean CTD to be significantly associated with development of grade ≥1 RIPF. Conclusions There was a good correlation between quantitative (CTD) and semi-quantitative (CTS) assessment of RIPF post adjuvant breast RT. The CTD method could be advantageous for both routine clinical practice and future clinical trials that require more detailed quantification of dense RIPF. Citation Format: Pramana A, Browne L, Cox H, Saba A, Pham K, Trakis S, Crawford K, Hall M, Batchelor N, Lim J, Graham P. Quantitative versus semi-quantitative assessments of radiation-induced pulmonary fibrosis post adjuvant breast radiotherapy. [abstract]. In: Proceedings of the Thirty-Eighth Annual CTRC-AACR San Antonio Breast Cancer Symposium: 2015 Dec 8-12; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2016;76(4 Suppl):Abstract nr P1-16-03.

The expression and clinical role of KL-6 in serum and BALF of patients with different diffuse interstitial lung diseases

To detect the levels of KL-6 in the serum and the bronchoalveolar lavage fluid (BALF) of patients with different type of diffuse interstitial lung diseases(DILD), and to analyze its correlation with pulmonary function, pulmonary HRCT scores and other parameters. Seventy-three patients with DILD were enrolled, including 34 patients with idiopathic pulmonary fibrosis (IPF), 10 patients with stage Ⅰ sarcoidosis(SAR Ⅰ), 15 patients with hypersensitivity pneumonia (HP), 14 patients with connective tissue diseases (CTD). Enzyme-linked immune sorbent assay (ELISA) was used to detect the levels of KL-6 in the serum and the BALF of these patients. In the IPF group, FVC predicted percentage and DLCO predicted percentage were significantly lower than those of the SAR Ⅰ group[(70±14)% vs (82±6)%, (49±13)% vs(81±6)%, P<0.05], but were no different compared to the CTD-ILD group(P>0.05). In the IPF group, the percentage of neutrophils in BALF was higher than that of the SAR Ⅰ group[(9±7)% vs (6±4)%, P<0.05]], and the percentage of lymphocytes and CD4/CD8 ratio in BALF were lower than those of the SAR Ⅰ group[(12±7)% vs (23±13)%, (1.5±0.8) vs(4.0±5.1), P<0.05]. In the IPF group, the level of KL-6 in serum was higher than that of the SAR Ⅰ and the HP group[(858±516)U/ml vs (339±168)U/ml, (553±287)U/ml, P<0.05], but was no different compared to the CTD-ILD group (P>0.05). In the CTD-ILD group, the level of KL-6 in serum was higher than that of the SAR Ⅰ [(687±350) U/ml vs (339±168)U/ml, P<0.05]]. In the IPF group, the level of KL-6 in BALF was (437±252)U/ml, and was higher than that of other 3 groups(P<0.05). In the IPF group, the level of serum KL-6 was negatively correlated with FVC and DLCO(r=-0.46, -0.58, P<0.01), the level of serum KL-6 was positively correlated with pulmonary HRCT reticular pattern and honeycombing scores(r=0.62, 0.41, P<0.05). In patients with IPF, the levels of KL-6 in serum and BALF were increased and the level of KL-6 in serum was correlated with FVC, DLCO and pulmonary HRCT reticular pattern and honeycombing scores.KL-6 may be a marker for the diagnosis of IPF.

A New Scale to Assess the Severity and Prognosis of Pulmonary Alveolar Proteinosis.

Background. Pulmonary Alveolar Proteinosis (PAP) is a syndrome characterized by pulmonary surfactant accumulation. Small proportion of PAP patients experienced spontaneous remission. Objective. The aim of this study was to assess the severity and prognosis of PAP using various indexes. Methods. Characteristics, PaO2, lung function parameters, and HRCT score of 101 patients with PAP were retrospectively analyzed. Many indexes were explored and integrated into a scale. Results. PaO2 was lower among smokers than among never-smokers. PaO2 differed between each pair of patient groups stratified according to HRCT score or DLCO, % predicted, which differed between any two groups stratified according to PaO2. The PAP patients who died presented with more symptoms, a higher HRCT score, and lower DLCO, % predicted, than survivors. Smoking status, symptoms, PaO2, HRCT score, and DLCO, % predicted, were integrated into a scale (severity and prognosis score of PAP (SPSP)). SPSP correlated positively with PaO2, FVC, % predicted, FEV1, % predicted, and DLCO, % predicted, and negatively with HRCT score. The patients who died displayed a higher SPSP than survivors. Conclusion. Smoking status, symptoms, PaO2, HRCT score, and DLCO, % predicted, were integrated into a scale (SPSP) that can be used to assess the severity and prognosis of PAP to some degree.

Immunoexpression of TGF-β/Smad and VEGF-A proteins in serum and BAL fluid of sarcoidosis patients.

BackgroundThe chronic course of pulmonary sarcoidosis can lead to lung dysfunction due to fibrosis, in which the signalling pathways TGF-β/Smad and VEGF-A may play a key role.MethodsWe evaluated immunoexpression of TGF-β1, Smad2, 3, and 7, and VEGF-A in serum and bronchoalveolar lavage (BAL) fluid of patients (n = 57) classified according to the presence of lung parenchymal involvement (radiological stage I vs. II-III), acute vs. insidious onset, lung function test (LFT) results, calcium metabolism parameters, percentage of BAL lymphocytes (BAL-L%), BAL CD4+/CD8+ ratio, age, and gender. Immunoexpression analysis of proteins was performed by ELISA.ResultsThe immunoexpression of all studied proteins were higher in serum than in BAL fluid of patients (p >0.05). The serum levels of TGF-β1 (p = 0.03), Smad2 (p = 0.01), and VEGF-A (p = 0.0002) were significantly higher in sarcoidosis patients compared to healthy controls. There were no differences within the sarcoidosis group between patients with vs. without parenchymal involvement, acute vs. insidious onset, or patients with normal vs. abnormal spirometry results. In patients with abnormal spirometry results a negative correlation was found between forced vital capacity (FVC) % predicted value and TGF-β1 immunoexpression in BAL fluid, and positive correlations were observed between the intensity of lung parenchymal changes estimated by high-resolution computed tomography (HRCT scores) and Smad 2 level in serum.ConclusionsTGF-β/Smad signalling pathway and VEGF-A participate in the pathogenesis of sarcoidosis. BAL TGF-β1, and Smad 2 in serum seem to be promising biomarkers with negative prognostic value, but further studies are required to confirmed our observations.

The prevention and therapeutics effect of sodium bicarbonate with gastric lavage, atomization inhalation and intravenous injection on the patients with paraquat poisoning and pulmonary fibrosis induced by paraquat poisoning

To observe the prevention effects of patients with lung exudation and pulmonary fibrosis induced by paraquat poisoning in sodium bicarbonate (SB) with gastric lavage, atomization inhalation and intravenous injection. To collect 38 patients with paraquat poisoning in hospital, after poison immediately with gastric lavage of 1.5% SB, and atomization inhalation of 5% SB 10~15 ml twice daily and intravenous injection of 5% SB twice a day, continuous application of 5~7 days. and the HRCT score and liver and kidney function was performed on patients with lung after treatment. And the extraction of 38 SB patients with previously untreated with SB for comparison. Lung HRCT average score in 72 h, 7 d, 30 d on patients with paraquat poisoning untreated with SB reached 2.87, 3.12, 2.13, HRCT display shows that the appearance of the wear glass shadow, grid shadow, honeycomb shadow, and other signs of fibrosis. Average HRCT reached 1.95, 2.20, 1.67 on patients treated with SB,signs of lung exudation and fibrosis was significantly reduced,compare two groups,there was statistically significance (P<0.01). And compared to the control group, activity of serum alanine aminotransferase (ALT) and aspartic acid transaminase (AST) decreased significantly in group of paraquat poisoning with triple application of SB, the level of serum urea nitrogen (BUN) and creatinine (Cr) significantly decreased, the difference is statistically significant (P<0.01). The triple application of SB can reduced the pulmonary fibrosis and effusion induced by paraquat poisoning,and protective effect on the function of liver and kidney is obvious, suggesting that the method for treatment of paraquat poisoning, prevention of paraquat lung and improve survival rate has the exact effect.

Prevalence of chronic hepatitis C virus (HCV) infection in patients with idiopathic pulmonary fibrosis

IntroductionPathogenic sequences leading to the development of idiopathic pulmonary fibrosis (IPF) are unclear; but one theory is that, in a genetically susceptible host, there is a “triggering agent or event inducing an inflammatory reaction in the lung that perpetuates itself and causes parenchymal fibrosis”. One potential source for a self-perpetuating triggering event could be a chronic viral infection. For this reason, evidence for an association between IPF and chronic viral infection has been sought by many investigators for several different viruses, including HCV. ObjectiveTo estimate the prevalence of chronic hepatitis C virus infection in patients with idiopathic pulmonary fibrosis via detection of HCV antibodies in selected patients in comparison to the control group. Patients and methodsIn this study we evaluated 30 patients diagnosed with idiopathic pulmonary fibrosis according to diagnostic criteria of American Thoracic Society (ATS) in comparison to 60 healthy control subjects. All enrolled subjects underwent a serologic test for HCV infection by detecting hepatitis C surface antigen (HCVsAg) by a third-generation enzyme-linked immunosorbent assay (ELISA) test. All patients had undergone dyspnea severity assessment by the mMRC score, routine laboratory testing including arterial blood gases (ABGs), pulmonary function testing (PFTs), chest-X-ray, high resolution CT scan (HRCT), liver ultrasonography and bronchoscopy (when needed). Results9 IPF patients were positive for HCV (30%), while 17 control subjects were positive for HCV (28.3%) (p=0.869). In HCV positive IPF patients there were more severe dyspnea as assessed by the mMRC score (p=0.042∗), lower FVC (p=0.011∗), SaO2% and PaO2 were significantly lower (p⩽0.001∗ for both parameters), and more severe HRCT scanning score (p=0.012∗), in comparison to HCV negative IPF patients. There was significant negative correlation between the HRCT score and FVC (p=0.011∗) in HCV positive IPF patients, there was significant negative correlation between liver cirrhosis and PaO2 (p=0.023∗), PaCO2 (p=0.002∗) in HCV positive IPF patients. ConclusionDespite the fact that we couldn’t confirm the hypothesis that HCV can be a causative agent in the development of IPF, however, we have shown that HCV can be a predisposing factor for the development of a more severe form of IPF. Therefore screening IPF patients for the presence of underlying HCV infection can have important therapeutic and prognostic implications in those patients.

Role of a diagnostic triad in bronchiectasis

ObjectiveTo differentiate cystic and cylindrical bronchiectasis utilizing a diagnostic triad of HRCT score, PFTs, and echocardiography. DesignA prospective observational cross sectional study. SettingAll bronchiectasis patients admitted to the chest department of Assiut and Al-Azhar Universities from May 2012 to June 2014 were enrolled in this study. Main outcome measuresHRCT score, pulmonary function tests parameters (FEV1, FEV1/FVC, FVC, PaO2, PaCO2 and DLCO) and complete echocardiographic assessment were done for all patients. ResultsObstructive pulmonary defect was observed in a majority of patients (56%). FVC%, FEV1 and FEV1/FVC were lower in patients with cylindrical as compared to cystic bronchiectasis. RV diameter was significantly greater in cystic bronchiectasis, and was positively correlated with pulmonary hypertension and negatively correlated with PaO2. PH was significantly greater in patients with cystic bronchiectasis and was positively correlated with PaCO2, and inversely correlated with PaO2, and FEV1. Global HRCT scores for cystic bronchiectasis were significantly correlated with the values of FEV1% and with SPAP. ConclusionsCystic bronchiectasis is associated with more severe lung function impairment and worse HRCT scores as compared with cylindrical bronchiectasis. In cystic bronchiectasis, HRCT scores correlated with FEV1% and SPAP and could be a predictor of future PH. HRCT depicts these pathological changes, moreover, high HRCT score not only reflects lung damage in those patients, but it can also be correlated with PH. PH; therefore can be a marker of lung damage in bronchiectatic patients.

Interleukin-6 in systemic sclerosis and potential correlation with pulmonary involvement

BackgroundA progressive pulmonary involvement is frequent in systemic sclerosis and it is the leading cause of morbidity and mortality. IL-6 has been implicated in the pathogenesis of systemic sclerosis via stimulation of fibroblasts to produce excess collagen and glycosaminoglycan. Specific correlation between IL-6 and lung involvement have not been found yet. AimTo study the possible correlation between lung involvement (assessed by spirometry and HRCT abnormalities) and the serum level of IL-6. Subjects and methods20 patients with scleroderma compared with 20 matched volunteers as control group. All participants underwent spirometry, HRCT scan and serum IL-6 measurements. HRCT signs were scored according to Warrick et al. score for systemic sclerosis. ResultsPatients showed a statistically significant reduction in FVC%, FEF 25–75% and significantly higher ESR and IL-6 compared to control. There was a highly significant positive correlation between the total HRCT score and serum IL-6. ConclusionSerum IL-6 could be a marker of the degree of pulmonary involvement in patients with systemic sclerosis.

S67 Lung Clearance Index (lci) Is A Sensitive Predictor Of High Resolution Computed Tomography (hrct) Scores In Children With Non-cf Bronchiectasis

Introduction and objectives LCI is a sensitive predictor of early cystic fibrosis (CF) lung disease, and correlates with HRCT better than spirometry (Thorax. 2008;63:129–134). The same is true in adults with non-CF bronchiectasis (Am J Respir Crit Care Med. 2014;189:586–592.), but by contrast, in PCD there were no relationships between LCI, HRCT or spirometry (Am J Respir Crit Care Med. 2013;188:545–549). It is unclear whether these differences reflect primary versus secondary ciliary dyskinesia, or CFTR versus non-CFTR disease. We hypothesised that in children with non-CF bronchiectasis, relationships between spirometry, LCI and HRCT will be similar to those in CF children and non-CF bronchiectasis adults, rather than PCD patients. Methods 12 children with non-CF bronchiectasis performed LCI and spirometry and underwent thoracic HRCT. HRCT scans were scored quantitatively (Thorax. 2013;68:532–539). Results were compared with those from large CF (n = 125) and PCD (n = 38) cohorts. Results In non-CF bronchiectasis there was a correlation between first second forced expired volume (FEV 1 ) and LCI (p = 0.009, r=-0.6), similar to that seen in CF (p 1 . Conclusions LCI is a good marker of structural lung disease in children with non-CF bronchiectasis and is more sensitive to HRCT abnormalities than spirometry, similar to adults, and CF at all ages. This suggests the different relationships seen in PCD result from the effects of primary versus secondary ciliary dyskinesia rather than CFTR versus non-CFTR lung disease. LCI may be useful in monitoring children with non-CF bronchiectasis, but this needs to be confirmed longitudinally. The results illustrate the importance of not extrapolating between different airway diseases.

SAT0305 Interstitial Lung Disease (ILD) in Patients with Anti-Jo-1 Syndrome (AJS): A Descriptive Analysis

ObjectivesTo evaluate the patterns, severity and prognostic parameters of ILD in AJS.MethodsWe identified 51 anti-Jo-1 patients with ILD between 2003 and 2012. Clinical and laboratory data were obtained along with...

Impact of Cystic versus Cylindrical Types of Bronchiectasis on Pulmonary and Cardiac Functions

Background: The combination of the clinical features, HRCT score and echocardiographic evidence of pulmonary hypertension help assess the prognosis in bronchiectasis. Aim: To test whether pulmonary and cardiac functions associated with bronchiectasis would differ according to its type (cystic versus cylindrical) utilizing HRCT score, PFTs and echocardiography. Patients and Methods: A cross-sectional study of patients with bronchiectasis was conducted at Chest and Internal Medicine Departments at Assiut University Hospital, Egypt. The diagnosis of bronchiectasis type was based on HRCT findings. PFTs, HRCT score and echocardiography were assessed in all cases. Results: We studied 56 patients with bronchiectasis; 31 were cystic (group A) and 25 were cylindrical (group B). Forced vital capacity (FVC%) and Dlco% were significantly lower in group A (P as compared with group B; whereas FEF 25%-75% and FEV1 (0.04) were lower in group B. Global HRCT score, RVD and SPAP were significantly higher in group A (P = 0.002) and correlated with FEV1% (r = ?0.51), and with SPAP (r = 0.16). Conclusions: Airway obstruction and small airway dysfunction were more significantly seen in cylindrical bronchiectasis. Patients with cystic bronchiectasis had significantly, higher global HRCT scores, RVD and SPAP. HRCT scores correlate with FEV 1% and SPAP and could be a predictor of future PH. Routine echocardiographic assessment of patients with bronchiectasis, particularly in those with cystic disease is highly recommended.

IL-4 polymorphisms, HRCT score and lung tissue markers in idiopathic pulmonary fibrosis

We studied the influence of IL-4 gene polymorphisms on the IPF phenotype, i.e., extent of radiological changes (HRCT interstitial (IS) and alveolar (AS) score) and histopathological markers from lung biopsies. 46 IPF patients underwent genotyping, 43 of them had HRCT and 14 patients had a surgical lung biopsy. The HRCT scans were evaluated for AS and IS. The histopathological evaluation comprised myofibroblast foci (MF), intensity of inflammation and fibrosis (Ashcroft score) and numbers of eosinophils and granulomas. For immunohistochemical evaluation primary antibodies against PAR-2, CD124, TGF beta, YY-1 and TSLP were used. The IL-4 and IL-4 R alpha gene polymorphisms were characterized. We found a correlation between eosinophils in lung biopsies and AS. The Ashcroft score was higher in IL-4 HA 2 GCC and MF were more frequent in IL-4 HA 2 TCC carriers. A relationship was found between IL-4 (-1098) A2 T and PAR-2 expression and IL-4 (-590) A1 T, IL-4 HA1TTT and CD124 expression. AS was lower in IL-4 (-590) A1 C, in IL-4 HA1 TCC and in IL-4RA (+1902) A1 A carriers. We suggest that the polymorphisms of IL-4 genes might influence the phenotype of IPF reflected by histopathological changes in lung biopsies and HRCT score.

AB0818 Nitric oxide diffusion and pulmonary capillary blood volume measurement in scleroderma patients

BackgroundCarbon monoxide diffusing capacity test (DLCO) is a non invasive diagnostic method for interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), frequent and severe complications of systemic sclerosis (SSc)....

FRI0396 Double-blind comparison of mycophenolate mofetil and oral cyclophosphamide for treatment of scleroderma-related interstitial lung disease (scleroderma lung study [SLS] II): rationale, design, methods, baseline characteristics and patient disposition

BackgroundCyclophosphamide (CYC), is the only therapy that has been shown thus far to be efficacious in scleroderma-related interstitial lung disease (SSc-ILD). However, the utility of CYC is constrained by its...

WS13.4 Chest HRCT (cCT) score predicts later Pseudomonas infection in young children with cystic fibrosis

WS13.4 Chest HRCT (cCT) score predicts later Pseudomonas infection in young children with cystic fibrosis

FRI0243 Extent of disease on high-resolution CT lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease

ObjectivesWe sought to determine whether the extent of disease on high-resolution CT lung (HRCT), measured using a simple grading system,[1] is predictive of decline and mortality in systemic sclerosis-related interstitial...