Risk factors for morbidity and death in non-cystic fibrosis bronchiectasis: a retrospective cross-sectional analysis of CT diagnosed bronchiectatic patients

Goeminne, Pieter; Scheers, Hans; Decraene, Ann; Seys, Sven; Dupont, Lieven

doi:10.1186/1465-9921-13-21

Cited by 95 publications

(76 citation statements)

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“…Infectious complications are associated with mortality in patients with MPA over the first year after diagnosis (26), and maintenance therapy with corticosteroids increases the incidence rate of infections (27). Furthermore, BE is closely related to respiratory infection, and the exacerbation rate is 1.94 times per year (28). In the present study, two patients (28.6%) with BE were colonized by P. aeruginosa.…”

Section: Discussionmentioning

confidence: 46%

Characteristics and prognosis of microscopic polyangiitis with bronchiectasis

et al. 2017

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Background: Major pulmonary manifestations associated with microscopic polyangiitis (MPA) include diffuse alveolar hemorrhage (DAH) and interstitial pneumonia (IP).We previously showed bronchiectasis (BE) was one of the pulmonary complications of MPA. However, clinical features of BE patients with MPA are not fully understood. We investigated the characteristics and prognosis of BE patients with MPA.Methods: Forty-five MPA patients were retrospectively studied. The patients were divided into two groups:patients with BE and those without BE.Results: Thirty-one of 45 patients (69%) had pulmonary involvement including IP (23/45, 51%), BE (7/45, 16%), and DAH (5/45, 11%). There were no differences between the patients with BE versus those without with regard to clinical characteristics and initial treatments. However, the prognosis for patients with BE was better than those without BE during the first year after diagnosis, but it was worse between 1 and 5 years, which was statistically significant. Two BE patients died between 1 and 5 years as a result of pneumonia.Conclusions: BE as a complication of MPA might be related to lower mortality in the acute phase and higher mortality in the chronic phase compared to other pulmonary manifestations. More attention to pulmonary infection is needed for patients with BE during the chronic phase.

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Section: Discussionmentioning

confidence: 46%

Characteristics and prognosis of microscopic polyangiitis with bronchiectasis

et al. 2017

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“…Additional variables are likely to be associated with mortality beyond those included in the current BSI score. These may include time since diagnosis of bronchiectasis or the presence of pulmonary hypertension, neither of which was recorded routinely in this study (20). In addition, our study was primarily of patients with idiopathic and postinfective bronchiectasis and would not be powered to detect anything other than very large effects on survival in bronchiectasis due to less common etiologies, such as allergic bronchopulmonary aspergillosis or rheumatological diseases.…”

Section: Discussionmentioning

confidence: 99%

“…The Bronchiectasis Severity Index (BSI) was validated in independent cohorts of patients with bronchiectasis from four centers: Dundee (n = 218) and Newcastle (n = 126) in the UK (19), Leuven in Belgium (n = 253) (20), and Monza in Italy (n = 105). Details of data collection in each of these studies are described in the online supplement.…”

Section: Validation Cohortsmentioning

confidence: 99%

The Bronchiectasis Severity Index. An International Derivation and Validation Study

Chalmers

Goeminne²,

Aliberti

et al. 2014

Am J Respir Crit Care Med

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Rationale: 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.Objectives: This study describes the derivation and validation of the Bronchiectasis Severity Index (BSI).Methods: Derivation of the BSI used data from a prospective cohort study (Edinburgh, UK, 2008 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).Measurements and Main Results: Independent predictors of future hospitalization were prior hospital admissions, Medical Research Council dyspnea score greater than or equal to 4, FEV 1 , 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 FEV 1 , 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. Conclusions:The BSI is a useful clinical predictive tool that identifies patients at risk of future mortality, hospitalization, and exacerbations across healthcare systems.

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“…While a myriad of infecting organisms have been identified, the archetypal pathogen of suppurative lung diseases is Pseudomonas aeruginosa (King et al, 2007;Goeminne et al, 2012). During the initial phase of infection, P. aeruginosa is postulated to utilize various virulence factors, including proteases, lipases and elastases, which help to facilitate initial infection (Gillham et al, 2009;Chalmers & Hill, 2013).…”

Section: Introductionmentioning

confidence: 99%

Virulence adaptations of Pseudomonas aeruginosa isolated from patients with non-cystic fibrosis bronchiectasis

et al. 2016

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Pseudomonas aeruginosa is a major pathogen in chronic lung diseases such as cystic fibrosis (CF) and non-cystic fibrosis bronchiectasis (nCFB). Much of our understanding regarding infections in nCFB patients is extrapolated from findings in CF with little direct investigation on the adaptation of P. aeruginosa in nCFB patients. As such, we investigated whether the adaptation of P. aeruginosa was indeed similar between nCFB and CF. From our prospectively collected biobank, we identified 40 nCFB patients who had repeated P. aeruginosa isolates separated by !6 months and compared these to a control population of 28 CF patients. A total of 84 nCFB isolates [40 early (defined as the earliest isolate in the biobank) and 41 late (defined as the last available isolate in the biobank)] were compared to 83 CF isolates (39 early and 44 late). We assessed the isolates for protease, lipase and elastase production; mucoid phenotype; swarm and swim motility; biofilm production; and the presence of the lasR mutant phenotype. Overall, we observed phenotypic heterogeneity in both nCFB and CF isolates and found that P. aeruginosa adapted to the nCFB lung environment similarly to the way observed in CF isolates in terms of protease and elastase expression, motility and biofilm formation. However, significant differences between nCFB and CF isolates were observed in lipase expression, which may allude to distinct characteristics found in the lung environment of nCFB patients. We also sought to determine virulence potential over time in nCFB P. aeruginosa isolates and found that virulence decreased over time, similar to CF. INTRODUCTIONBronchiectasis is a pathologic diagnosis defined by permanent dilation and widening of the respiratory airways and is common in chronic suppurative lung diseases like cystic fibrosis (CF) and non-cystic fibrosis bronchiectasis (nCFB) (Weycker et al., 2005). Patients with bronchiectasis may present with common symptoms including sputum production, recurrent respiratory infections and airway obstruction manifesting in thickened bronchial walls, establishment of chronic infections and increased levels of inflammatory markers (Mhanna et al., 2001;Seitz et al., 2010;Bergin et al., 2013;Gupta et al., 2015).Bronchiectasis arises as a consequence of complications induced by mutations in the CF transmembrane conductance regulator CFTR. However, multiple other non-CFTR mechanisms exist that culminate in bronchiectasis, termed nCFB. Typically considered an 'orphan disease', the incidence of nCFB has risen by 8.7 % annually between (Barker & Bardana, 1988Seitz et al., 2012) and is estimated to cost over $630 million annually in the US healthcare system. Causes of bronchiectasis include immune dysregulation (including autoimmune disorders), obstruction of the airways and complications from infections or injuries (Al-Shirawi et al., 2006;McShane et al., 2012).In patients with CF and nCFB, accumulation of thick mucus in the lungs and impaired mucociliary clearance allow respiratory infections (Martens et al., 2011). ...

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Risk factors for morbidity and death in non-cystic fibrosis bronchiectasis: a retrospective cross-sectional analysis of CT diagnosed bronchiectatic patients

Cited by 95 publications

References 34 publications

Characteristics and prognosis of microscopic polyangiitis with bronchiectasis

Characteristics and prognosis of microscopic polyangiitis with bronchiectasis

The Bronchiectasis Severity Index. An International Derivation and Validation Study

Virulence adaptations of Pseudomonas aeruginosa isolated from patients with non-cystic fibrosis bronchiectasis

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