Bronchiectasis Severity Research Articles

COPD-related Bronchiectasis; Independent Impact on Disease Course and Outcomes

Background: COPD and radiographic bronchiectasis frequently coexist but the effect of this on the clinical course of COPD is not fully understood. We determined the impact of bronchiectasis on clinical outcomes in COPD patients, independent of coexisting emphysema and bronchial wall thickening (BWT). Methods: COPD patients admitted with first exacerbation 1998–2008 were identified retrospectively using ICD10 codes J44.0,1,8,9. Patients with suitable CT scans were graded for severity of bronchiectasis, emphysema and BWT on a 5 point scale (0-absent, 1-minor, 2-mild, 3-moderate, 4-severe). Results: 406 patients (71 ± 11 years, 56% male, FEV1 52 ± 23% predicted) were included; 278 (69%) patients had bronchiectasis: minor, 112 (40%); mild, 81 (29%); moderate, 62 (22%); severe 23 (8%). Bronchiectasis severity correlated with severity of BWT (p < 0.001) but not emphysema (p = 0.090). Bronchiectasis independently determined sputum isolation of Pseudomonas aeruginosa (Odds ratio (OR) 1.39 (95% CI 1.07 to 1.80), p = 0.013) and atypical mycobacteria (OR 2.44 (95% CI 1.04 to 5.69), p = 0.040), annual respiratory admissions (p = 0.044) and inpatient days (p < 0.001), but did not predict survival (p = 0.256). Conclusions: Radiographic bronchiectasis in COPD patients is associated with increased respiratory infection and hospitalisation, independent of coexisting emphysema and BWT. COPD-related bronchiectasis is therefore an important diagnosis with potential implications for treatment.

Mannose-binding lectin 2 gene polymorphism and lung damage in primary ciliary dyskinesia.

Mannose-binding lectin (MBL) plays an important role in innate immunity and has been reported to be associated with the age-related decline in lung function in cystic fibrosis. MBL polymorphisms are associated with lung function decline in Primary Ciliary Dyskinesia (PCD). We performed sputum microbiology, spirometry pre- and post-administration of salbutamol, ciliary motion analysis, ultrastructural assessment of cilia, ciliogenesis in culture, and chest high resolution computed tomography in children with a clinical history of respiratory tract infections and/or presence of bronchiectasis suggestive of PCD or secondary ciliary dyskinesia (SCD). All subjects were evaluated for single nucleotide polymorphisms in the gene encoding MBL-2. The diagnosis of PCD was established in 45 subjects, while in the remaining 53 the diagnosis was SCD. A significant bronchodilator response was observed only in PCD associated with the MBL2-3 genotype, which is known to be associated with low/undetectable MBL serum levels. Also, bronchiectasis severity was significantly greater in subjects with MBL2-3 in both PCD and SCD. No other association was found between MBL genotypes and clinical findings. MBL plays a relatively minor role as a disease modifier in PCD. A similar finding in SCD supports the likely significance of this result.

Sleep disordered breathing and airway disease in primary ciliary dyskinesia

Sleep-disordered breathing (SDB) may develop in primary ciliary dyskinesia (PCD), leading to these diseases worsening one another. Sixteen stable PCD patients (4.9-17.2 years) and 42 controls underwent overnight respiratory polysomnography (rPSG) and Sleep Disturbances Scale for Children (SDSC). In PCD we assessed nasal endoscopy, pulmonary function tests and chest high-resolution computed tomography (HRCT). Compared with controls, PCD had higher obstructive apnoea (4.7 vs 0.2, P < 0.001), central apnoea (0.8 vs 0.2, P < 0.001), hypopnoea (1.8 vs 0.2, P < 0.001), apnoea-hypopnoea (7.8 vs 0.6, P < 0.001), oxygen desaturation indexes (ODI; 0.7 vs 0.2, P = 0.002), and mean oxygen desaturation (4% vs 1%, P < 0.001), while mean and nadir oxygen saturation (97.1% vs 98.1, P < 0.001) (93% vs 97.2%, P < 0.001) were lower, respectively. In PCD, SDSC was unrelated to rPSG (P > 0.05), with total score and subscores of disorders in initiating and maintaining sleep, and sleep-wake transition lower than controls. PCD patients had chronic rhinosinusitis (100%) and adenoidal hypertrophy (50%). Total HRCT score was 7 (range 0-14). ODI correlated with functional residual capacity (r = 0.8, P = 0.02), total HRCT (r = 0.6, P = 0.03) and peribronchial thickening scores (r = 0.7, P = 0.02). Oxygen saturation was associated with bronchiectasis severity score (r = -0.6, P = 0.02). PCD's parents may underestimate SDB. As nocturnal desaturation is associated with lung function and structure abnormalities, SDB may significantly contribute to pulmonary morbidity.

Bronchiectasis Severity: Time to Score

Bronchiectasis Severity: Time to Score

The Bronchiectasis Severity Index. An International Derivation and Validation Study

There are no risk stratification tools for morbidity and mortality in bronchiectasis. Identifying patients at risk of exacerbations, hospital admissions, and mortality is vital for future research. This study describes the derivation and validation of the Bronchiectasis Severity Index (BSI). Derivation of the BSI used data from a prospective cohort study (Edinburgh, UK, 2008-2012) enrolling 608 patients. Cox proportional hazard regression was used to identify independent predictors of mortality and hospitalization over 4-year follow-up. The score was validated in independent cohorts from Dundee, UK (n = 218); Leuven, Belgium (n = 253); Monza, Italy (n = 105); and Newcastle, UK (n = 126). Independent predictors of future hospitalization were prior hospital admissions, Medical Research Council dyspnea score greater than or equal to 4, FEV1 < 30% predicted, Pseudomonas aeruginosa colonization, colonization with other pathogenic organisms, and three or more lobes involved on high-resolution computed tomography. Independent predictors of mortality were older age, low FEV1, lower body mass index, prior hospitalization, and three or more exacerbations in the year before the study. The derived BSI predicted mortality and hospitalization: area under the receiver operator characteristic curve (AUC) 0.80 (95% confidence interval, 0.74-0.86) for mortality and AUC 0.88 (95% confidence interval, 0.84-0.91) for hospitalization, respectively. There was a clear difference in exacerbation frequency and quality of life using the St. George's Respiratory Questionnaire between patients classified as low, intermediate, and high risk by the score (P < 0.0001 for all comparisons). In the validation cohorts, the AUC for mortality ranged from 0.81 to 0.84 and for hospitalization from 0.80 to 0.88. The BSI is a useful clinical predictive tool that identifies patients at risk of future mortality, hospitalization, and exacerbations across healthcare systems.

Multidimensional approach to non-cystic fibrosis bronchiectasis: the FACED score

Bronchiectasis is a multidimensional disease and, therefore, its severity or prognosis cannot be adequately quantified by analysing one single variable. The objective of the present study was to develop a multidimensional score that classifies the severity of bronchiectasis according to its prognosis. This is an observational multicentre study including 819 patients diagnosed with non-cystic fibrosis bronchiectasis using high-resolution computed tomography. 397 subjects were selected at random to construct the score while the remaining 422 were used for its validation. The outcome was 5-year all-cause mortality after radiological diagnosis. A logistic regression analysis was used to select the variables included in the final score. The final seven-point score incorporated five dichotomised variables: forced expiratory volume in 1 s % predicted (F, cut-off 50%, maximum value 2 points); age (A, cut-off 70 years, maximum value 2 points); presence of chronic colonisation by Pseudomonas aeruginosa (C, dichotomic, maximum value 1 point); radiological extension (E, number of lobes affected, cut-off two lobes, maximum value 1 point); and dyspnoea (D, cut-off grade II on the Medical Research Council scale, maximum value 1 point) to construct the FACED score. The validation cohort confirmed the score's validity. We conclude that this easy-to-use multidimensional grading system proved capable of accurately classifying the severity of bronchiectasis according to its prognosis.

S124 Derivation and validation of the bronchiectasis severity index: an international multicentre observational study

IntroductionThere are no risk stratification tools for morbidity and mortality in bronchiectasis. As more treatments become available, it is important to identify patients at risk of exacerbations, hospital admissions and...

Matrix Metalloproteinase-1 Polymorphism (-1607G) and Disease Severity in Non-Cystic Fibrosis Bronchiectasis in Taiwan

ObjectivesBronchiectasis is characterized by an irreversible dilatation of bronchi and is associated with lung fibrosis. MMP-1 polymorphism may alter its transcriptional activity, and differentially modulate bronchial destruction and lung fibrosis.DesignTo investigate the association of MMP-1 polymorphisms with disease severity in non-cystic fibrosis (CF) bronchiectasis patients, 51 normal subjects and 113 patients with bronchiectasis were studied. The associations between MMP-1 polymorphisms, lung function, and disease severity evaluated by high resolution computed tomography (HRCT) were analyzed.ResultsThe frequency of MMP-1(-1607G) allele was significantly higher in patients with bronchiectasis than normal subjects (70.8% vs 45.1%, p<0.01). Forced expiratory volume in 1 second (FEV1) was decreased in bronchiectasis patients with 1G/1G (1.2±0.1 L, n = 14) and 1G/2G (1.3±0.1 L, n = 66) genotypes compared to the 2G/2G genotype (1.7±0.1 L, n = 33, p<0.01). Six minute walking distance was decreased in bronchiectasis patients with 1G/1G and 1G/2G compared to that of 2G/2G genotype. Disease severity evaluated by HRCT score significantly increased in bronchiectasis patients with 1G/1G and 1G/2G genotypes compared to that of 2G/2G genotype. Bronchiectasis patients with at least one MMP-1 (-1607G) allele showed increased tendency for hospitalization. Serum levels of pro-MMP-1, active MMP-1 and TGF-β1 were significantly increased in patients with bronchiectasis with 1G/1G and 1G/2G genotype compared with 2G/2G genotype or normal subjects. Under IL-1β stimulation, peripheral blood monocytes from subjects with 1G/2G or 1G/1G genotype secreted higher levels of TGF-β1compared to subjects with 2G/2G genotype.ConclusionThis is the first report to address the influence of MMP-1 polymorphisms on lung function and airway destruction in non-CF bronchiectasis patients. Bronchiectasis patients with MMP-1(-1607G) polymorphism may be more vulnerable to permanent lung fibrosis or airway destruction due to the enhanced MMP-1 and TGF-β1 activity. Upregulated MMP-1 activity results in proteolytic destruction of matrix, and leads to subsequent fibrosis.

Symptoms of airway reflux predict exacerbations and quality of life in bronchiectasis

We have explored the association of the upper airway symptoms related to cough with exacerbation frequency, sputum microbiology and inflammatory markers in patients with non cystic fibrosis bronchiectasis. Patients with bronchiectasis completed the Hull Airway Reflux Questionnaire (HARQ). A score of >13 was taken to indicate the presence of reflux. Patients were followed-up with longitudinal spirometry, sputum culture and Leicester cough questionnaire (LCQ). Myeloperoxidase (MPO), free neutrophil elastase (NE) activity, Interleukin (IL)-8 and Tumour Necrosis Factor (TNF)-α was measured from spontaneous sputum samples. 163 completed the study. 59.5% were female. Mean age was 65.7 years. 73.6% reported airway reflux using HARQ. Patients with airway reflux had more severe cough symptoms as assessed by the LCQ [15.2 (3.5) vs. 19.4 (1.9)], p<0.001. Sputum levels of MPO, NE, IL-8 and TNF-α were all significantly higher in the reflux positive group (p<0.05 for all comparisons). In a multivariable logistic regression, airway reflux was independently associated with cough severity (-3.27, standard error 0.81, p=0.0002). Airway reflux, age, FEV1 % predicted and colonization with Pseudomonas aeruginosa were independently associated with an increased risk of ≥3 bronchiectasis exacerbations in one year. The symptoms of airway reflux independently predictseverity and exacerbation frequency in non cystic fibrosis bronchiectasis.

Mannose-binding lectin deficiency and disease severity in non-cystic fibrosis bronchiectasis: a prospective study

Mannose-binding lectin (MBL) is a key component of innate immunity. MBL deficiency is common (10-30% of the general population depending on the definition used) and has been associated with disease progression in cystic fibrosis. We aimed to assess the effect of MBL deficiency on disease severity in non-cystic fibrosis bronchiectasis. We recruited patients with non-cystic fibrosis bronchiectasis and age-matched and sex-matched controls at a specialist bronchiectasis clinic in Edinburgh, UK. We assessed MBL function with genotyping (low-expressing genotype [deficiency] defined as homozygosity for exon 1 mutations [YO/YO] or compound heterozygosity [XA/YO]; YA/YO and XA/XA genotypes were defined as intermediate-expressing with all other genotypes defined as high-expressing) and serum measurements (deficiency defined with two parameters: <500 ng/mL or <200 ng/mL). We assessed rates of exacerbation, chronic bacterial colonisation, and lung function during 4 years of follow-up. We included 470 patients with bronchiectasis and 414 controls. MBL genotype frequencies and MBL serum concentrations did not differ between patients and controls. 55 (12%) patients with bronchiectasis had low-expressing genotypes. These patients had a mean of 2·7 exacerbations per year (SD 1·8), compared with 1·9 per year (1·2) for 135 patients with intermediate-expressing genotypes and 1·9 per year (1·3) for 280 patients with high-expressing genotypes (p<0·0001). Chronic colonisation with bacteria was most frequent in patients with low-expressing genotypes (47 [85%] patients vs 82 [61%] patients with intermediate-expressing genotypes and 183 [65%] patients with high-expressing genotypes; p=0·0041); especially P aeruginosa colonisation (19 [35%] patients vs 13 [10%] patients and 36 [13%] patients; p<0·0001). Patients with low-expressing genotypes were more likely to be admitted to hospital for severe exacerbations during follow-up (27 [49%] patients vs 42 [31%] patients and 87 [31%] patients; p=0·032). Patients with low-expressing genotypes also had increased scores for radiological severity and worse quality of life compared with the other two groups. MBL serum deficiency (<200 ng/mL) was associated with increased exacerbations, hospital admissions, and radiological severity. When <500 ng/mL was used as the definition of deficiency, the associations with exacerbation frequency and radiological severity were no longer significant. MBL might be an important modifier of disease severity in non-CF bronchiectasis. UK Medical Research Council, UK Chief Scientists Office.

P174 Severity of Bronchiectasis on High Resolution CT Scanning and Its Relationship to Chronic Bacterial Colonisation

IntroductionBacterial colonisation and particularly colonisation with Pseudomonas aeruginosa is associated with a more severe clinical course in bronchiectasis. It is often presumed that patients with more extensive radiological bronchiectasis are...

P176 COPD-Related Bronchiectasis; A Real Clinical Entity with Impact on Disease Course and Outcomes

Introduction and ObjectivesNon-CF bronchiectasis is defined as ‘symptoms of persistent or recurrent bronchial sepsis related to irreversibly damaged and dilated bronchi’ [BTS guidelines 2010]. Radiographic evidence of ‘damaged and dilated...

Vitamin-D deficiency is associated with chronic bacterial colonisation and disease severity in bronchiectasis

IntroductionVitamin-D deficiency has been linked to an increased risk of respiratory infections. The objective of this study was to determine the frequency and clinical importance of vitamin-D deficiency in patients...

Socioeconomic deprivation, readmissions, mortality and acute exacerbations of bronchiectasis

Bronchiectasis is known to cause significant morbidity in children in New Zealand. Little is known of the disease in adults. Our objective was to characterise a cohort of adults who presented to hospital with acute exacerbations of the disease. We retrospectively collected information on all exacerbations treated as inpatients from a single hospital in South Auckland, New Zealand during 2002. We collected information on 307 exacerbations in 152 patients. Twenty-seven per cent were of Maaori ethnic origin, and 44% Pacific. Seventy per cent lived in areas categorised as the 20% most deprived in New Zealand. Comorbid conditions were present in 80% of patients - most commonly chronic obstructive pulmonary disease, asthma, diabetes and cardiac disease. Seventy (46%) patients had at least one readmission and 32 patients (21%) died within 12 months of admission to hospital. Greater deprivation was associated with increased mortality at 12 months after admission after adjusting for other factors (OR 11, 95% CI 2.0-61, P= 0.006). In the subgroup who underwent high-resolution computed tomographic scanning (93), increasing severity of bronchiectasis (modified Bhalla score) was associated with readmission within 12 months (P= 0.004), but not mortality (P= 0.419). We have shown that exacerbations of bronchiectasis in South Auckland are more common in patients who are predominantly of Maaori or Pacific descent and are socioeconomically deprived. Admission to hospital for an exacerbation is associated with high readmission and mortality rates.

Chronic hypersensitivity pneumonitis: high resolution computed tomography patterns and pulmonary function indices as prognostic determinants

To investigate high resolution computed tomography (HRCT) and pulmonary function indices (PFTs) for determining prognosis in patients with chronic fibrotic hypersensitivity pneumonitis (CHP). Case records, PFTs (FEV(1), FVC and DLco) and HRCTs of ninety-two patients with chronic hypersensitivity pneumonitis were evaluated. HRCT studies were scored by two observers for total disease extent, ground-glass opacification, fine and coarse reticulation, microcystic and macrocystic honeycombing, centrilobular emphysema and consolidation. Traction bronchiectasis within each pattern was graded. Using Cox proportional hazards regression models the prognostic strength of individual HRCT patterns and pulmonary function test variables were determined. There were forty two deaths during the study period. Increasing severity of traction bronchiectasis was the strongest predictor of mortality (HR 1.10, P < 0.001, 95%CI 1.04-1.16). Increasing global interstitial disease extent (HR 1.02, P = 0.02, 95%CI 1.00-1.03), microcystic honeycombing (HR 1.09, P = 0.019, 95%CI 1.01-1.17) and macrocystic honeycombing (HR 1.06, P < 0.01, 95%CI 1.01-1.10) were also independent predictors of mortality. In contrast, no individual PFT variable was predictive of mortality once HRCT patterns were accounted for. HRCT patterns, in particular, severity of traction bronchiectasis and extent of honeycombing are superior to pulmonary function tests for predicting mortality in patients with CHP. • HRCT is increasingly used to assess chronic fibrotic hypersensitivity pneumonitis. • HRCT patterns are superior to pulmonary function tests for predicting mortality. • Extensive traction bronchiectasis strongly predicts poor survival in chronic hypersensitivity pneumonitis.

Low serum mannose-binding lectin level is not associated with disease severity in non-cystic fibrosis bronchiectasis

Deficiency of mannose-binding lectin (MBL), a serum protein involved in killing and promoting phagocytosis of pathogens, is associated with respiratory infection and disease progression in a number of acute and chronic lung diseases, including cystic fibrosis (CF)- associated bronchiectasis. No such association has been studied in non-CF bronchiectasis (nCF-Br). One hundred and thirty-three adult patients with nCF-Br were studied. Serum MBL levels were measured and deficiency defined using two cut-off levels, i.e. MBL ≤100 ng/ml and ≤600 ng/ml. Parameters of severity included lung function impairment, annual exacerbation and hospital admission rates, breathlessness, and Pseudomonas aeruginosa and Haemophilus influenzae infection rates. The incidence of MBL deficiency using cut-off levels of 100 ng/ml and 600 ng/ml was 10% and 26% respectively, similar to rates seen in the general population. There was no significant difference in mean FEV(1)% predicted between MBL deficient and sufficient patients at both cut-off levels (≤100 ng/ml: 63.8% vs. 64.6%, P = 0.91; ≤ 600 ng/ml: 66.5% vs. 63.9%, P = 0.56). In addition, exacerbation/hospital admission rates, symptoms of breathlessness and isolation/colonisation rates with P. aeruginosa and H. influenzae were similar in both groups at both cut-off levels. In conclusion, MBL deficiency is not associated with markers of disease severity in patients with nCF-Br.

T2 Single nucleotide polymorphisms in the ficolin-2 gene predispose to Pseudomonas aeruginosa infection and disease severity in non-cystic fibrosis bronchiectasis

<h3>Introduction</h3> Bronchiectasis is associated with a destructive cycle of bacterial infection, airway inflammation and airway structural damage. Ficolins are a family of recently described serum pattern recognition molecules capable of binding to micro-organisms and activating complement through MBL associated serine protease-2 (MASP-2). Their role in chronic lung disease has not previously been investigated. <h3>Methods</h3> Serum levels of Ficolin-2 and MASP-2 were determined in 470 patients with idiopathic non-cystic fibrosis bronchiectasis and 400 matched control subjects by ELISA. Single nucleotide polymorphisms were determined using TaqMan PCR based genotyping. Bacterial binding was determined using ELISA and flow cytometry. Neutrophils from healthy donors were isolated by percoll gradient centrifugation and used in phagocytosis assays with FITC-labelled <i>Pseudomonas aeruginosa</i> strain PA01. <h3>Results</h3> Genotyping success was &gt;95% and all SNP9s were in Hardy–Weinburg equilibrium (p&gt;0.05). Bronchiectasis was associated with a homozygous mutation in the promoter of ficolin-2 (rs3124952) (28.5% vs 19.5%, p=0.002) and a homozygous mutation in exon-8 of the ficolin-2 gene causing impaired binding (rs17549193) (12% vs 8.4%, p=0.04). Low serum levels (&lt;1.6 μg/ml, 2 SD. below the mean for controls) were strongly associated with the promoter polymorphisms (p&lt;0.0001) and with bronchiectasis (18.7% vs 7.8%, p&lt;0.0001). In-vitro, ficolin-2 bound to over 60 strains of <i>P aeruginosa</i>, including clinical isolates and mucoid strains, activating the lectin pathway of complement and promoting C3 and C4 deposition. Ficolin-2 also bound to <i>Streptococcus pneumoniae, Haemophilus influenzae</i> and <i>Staphylococcus aureus</i>. Opsonisation with ficolin-2 promoted phagocytosis of <i>P aeruginosa</i> (PA01) by human neutrophils in a MASP-2 but not c1q dependent manner (p&lt;0.0001) (Abstract T2 figure 1). On multivariable analysis chronic bacterial colonisation (OR=3.5; p&lt;0.0001) and particularly <i>P aeruginosa</i> colonisation (OR=2.8, p=0.0001) were independently associated with ficolin-2 insufficiency. These patients also had more frequent outpatient exacerbations (mean 3.2/yr vs 2.4/yr, p=0.01) and unscheduled hospital admissions for exacerbations (OR=2.3; p&lt;0.0001). <h3>Conclusion</h3> Single nucleotide polymorphisms in the ficolin-2 gene affecting serum levels and carbohydrate binding are associated with non-CF bronchiectasis and increase susceptibility to colonisation with <i>P aeruginosa.</i>

Elevated peripheral airway nitric oxide in bronchiectasis reflects disease severity

Bronchiectasis is characterised by neutrophilic bronchial inflammation and patients are prone to recurrent or chronic bacterial airway infections. Direct measurement of lung inflammation would be useful in order to assess disease activity and guide need for antibiotic treatment and to monitor response. Current methods of monitoring inflammation are invasive, indirect or insensitive. Exhaled nitric oxide (FE(NO)) is a direct simple non-invasive test of inflammation used in other airway diseases. The aim of this study was to test whether peripheral airway nitric oxide (C(alv)) can provide a clinically useful direct measure of inflammation in the lungs of patients with bronchiectasis. Fifty three patients with bronchiectasis were studied when clinically stable and a further 20 patients during an exacerbation of bronchiectasis. FE(NO) was measured by chemiluminescence using a NO analyser. Two models of pulmonary exchange dynamics were used to calculate proximal and peripheral contributions to final FE(NO) concentration. FE(NO) was elevated in bronchiectasis patients compared to 30 healthy controls (p < 0.05). Compartmental modelling reveals that this elevation is due to an increase in peripheral airway NO (bronchiectasis 3.6 ppb (2.1), controls 2.7 ppb (1.5) p < 0.05) whereas proximal airway NO levels are normal (bronchiectasis 777(751) pl/s, controls 582(579) pl/s ns). C(alv) relates to disease severity measured by lung function and HRCT scan and correlates with the quality of life score. There is no change in FE(NO) parameters at exacerbation and following treatment. Peripheral airway NO is elevated and reflects disease severity in bronchiectasis but does not provide information to inform acute treatment decisions.

Fibrotic idiopathic interstitial pneumonias: HRCT findings that predict mortality

The study aims were to identify CT features that predict outcome of fibrotic idiopathic interstitial pneumonia (IIP) when information from lung biopsy data is unavailable. HRCTs of 146 consecutive patients presenting with fibrotic IIP were studied. Visual estimates were made of the extent of abnormal lung and proportional contribution of fine and coarse reticulation, microcystic (cysts ≤4 mm) and macrocystic honeycombing. A score for severity of traction bronchiectasis was also assigned. Using death as our primary outcome measure, variables were analysed using the Cox proportional hazards model. CT features predictive of a worse outcome were coarse reticulation, microcystic and macrocystic honeycombing, as well as overall extent of lung abnormality (p < 0.001). Importantly, increased severity of traction bronchiectasis, corrected for extent of parenchymal abnormality, was predictive of poor prognosis regardless of the background pattern of abnormal lung (HR = 1.04, CI = 1.03-1.06, p < 0.001). On bivariate Cox analysis microcystic honeycombing was a more powerful determinant of a poor prognosis than macrocystic honeycombing. In fibrotic IIPs we have shown that increasingly severe traction bronchiectasis is indicative of higher mortality irrespective of the HRCT pattern and extent of disease. Extent of microcystic honeycombing is a more powerful determinant of outcome than macrocystic honeycombing.

Effect of radiological extent and severity of bronchiectasis on pulmonary function

BackgroundThe aim of this study was to ascertain the effect of the extent and severity of bronchiectasis as determined with high-resolution computed tomography (HRCT) on lung function in patients with pure bronchiectasis, bronchiectasis and asthma, and bronchiectasis and chronic obstructive pulmonary disease (COPD).MethodsOne hundred nineteen patients (71 with pure bronchiectasis, 25 asthmatic patients with bronchiectasis, and 23 COPD patients with bronchiectasis) underwent HRCT and pulmonary function tests. Computed tomography features were scored by the consensus of 2 radiologists.ResultsThere were no statistically significant differences among the 3 patient groups regarding the extent of bronchiectasis, bronchial dilatation degree, bronchial wall thickening, decreased attenuation in the lung parenchyma, or presence of mucus in the large and small airways. In the pure bronchiectasis group, a negative correlation was found between forced vital capacity (FVC) % of predicted, forced expiratory volume in 1 sec (FEV1) % of predicted, the FEV1/FVC ratio and the extent of bronchiectasis, bronchial wall thickening, bronchial wall dilatation, and decreased attenuation. At multivariate analysis the main morphologic changes associated with impairment of FVC and FEV1 were the extent of bronchiectasis and a decreased attenuation in the lung parenchyma. The decrease in the FEV1/FVC ratio was associated with bronchial wall dilatation. No correlation was found between morphologic changes and indices of pulmonary function in the asthma and COPD patients.ConclusionsMorphologic changes associated with bronchiectasis do not influence lung function in patients with asthma and COPD directly, although they do play a role in impairing pulmonary function in patients with bronchiectasis alone.