Paediatric and adult bronchiectasis: monitoring, cross-infection, role of multi-disciplinary teams and self-management plans

Bronchiectasis is a chronic lung disease associated with structurally abnormal bronchi; clinically manifested by a persistent wet/productive cough, airway infections and recurrent exacerbations. Early identification and treatment of acute exacerbations is an integral part of monitoring and annual review, in both adults and children, to minimise further damage due to infection and inflammation. Common modalities used to monitor disease progression include clinical signs and symptoms, frequency of exacerbations and/or number of hospital admissions, lung function (FEV1 %predicted), imaging (radiological severity of disease) and sputum microbiology (chronic infection with P. aeruginosa). There is good evidence that these monitoring tools can be used to accurately assess severity of disease and predict prognosis in terms of mortality and future hospitalisation. Other tools that are currently used in research settings such as health-related quality of life questionnaires, magnetic resonance imaging and lung clearance index can be burdensome and require additional expertise or resource, which limits their use in clinical practice. Studies have demonstrated that cross-infection, especially with P. aeruginosa between patients with bronchiectasis is possible but infrequent. This should not limit participation of patients in group activities such as pulmonary rehabilitation, and simple infection control measures should be carried out to limit the risk of cross-transmission. A multi-disciplinary approach to care which includes respiratory physicians, chest physiotherapists, nurse specialists and other allied health professionals are vital in providing holistic care. Patient education and personalised self-management plans are also important despite limited evidence it improves quality of life or frequency of exacerbations

Critical care admission trends and outcomes in individuals with bronchiectasis in the UK

Background: There are limited data on admission trends and outcomes of individuals with bronchiectasis admitted to intensive care (ICU). Using national critical care data, we analysed admissions to ICU and estimated outcomes in terms of mortality in individuals with bronchiectasis and chronic obstructive pulmonary disease (COPD) admitted to ICU. Methods: Using data from the Intensive Care National Audit and Research Centre, admissions from bronchiectasis and COPD from 1 January 2009 to 31 December 2013 were extracted. Crude admission rates for bronchiectasis and COPD were calculated and Poisson regression was used to estimate unadjusted annual admission rate ratios. We investigated changes to length of stay on ICU, ICU mortality and in-hospital mortality during the study period. We also compared mortality rates in people with bronchiectasis and COPD aged 70 or above. Results: We found an annual increase of 8% (95% Confidence Interval [CI] 2-15) in the number of ICU admissions from bronchiectasis, whilst the yearly increase in ICU admissions from COPD was 1% (95% CI 0.3-2). ICU and in-hospital mortality was higher in individuals with bronchiectasis compared with those with COPD, especially in people aged 70 years or above. Conclusion: Admission to ICU in people with bronchiectasis are uncommon, but are increasing in frequency over time, and carries a substantial mortality rate. This needs to be considered allocating health care resources and planning respiratory services

Surgical lung biopsy for the diagnosis of interstitial lung disease in England: 1997-2008

Introduction: International guidelines and new targeted therapies for idiopathic pulmonary fibrosis have increased the need for accurate diagnosis of interstitial lung disease, which may lead to more surgical lung biopsies. This study aims to assess the risk of this procedure in patients from the United Kingdom. Methods: We used Hospital Episodes Statistics data from 1997-2008 to assess the frequency of surgical lung biopsy for interstitial lung disease in England. We identified cardiothoracic surgical patients using ICD-10 codes for interstitial lung disease and OPCS-4 codes for surgical lung biopsy. We excluded those with lung resections or lung cancer. We estimated in-hospital, 30-day and 90-day mortality following the procedure, and linked to cause of death using data from the Office of National Statistics. Results: We identified 2,820 patients with interstitial lung disease undergoing surgical lung biopsy during the 12 year period. The number of biopsies increased over the time period studied. In-hospital, 30-day and 90-day mortality were 1.7%, 2.4% and 3.9% respectively. Male sex, increasing age, increasing co-morbidity and open surgery were risk factors for mortality. Discussion: Surgical lung biopsy for interstitial lung disease has a similar mortality to lobectomy for lung cancer, and clinicians and patients should understand the likely risks involved

Increasing global mortality from idiopathic pulmonary fibrosis in the twenty-first century

Rationale: Recent evidence from the United Kingdom suggests that the number of deaths from idiopathic pulmonary fibrosis is increasing, although comparable international data are limited. Objectives: We aimed to collate death certification data from multiple countries to determine global trends in mortality from idiopathic pulmonary fibrosis. Methods: Data were obtained from the national statistics agencies of countries with relevant mortality records. Age-standardised mortality rates were calculated, and Poisson regression modelling was used to calculate rate ratios. Meta-analysis was used to calculate an overall estimate of mortality change over time. Measurements and Main Results: Ten countries provided mortality data on pulmonary fibrosis over a period from 1999 to 2012. Age-standardised mortality ranged between 4 and 10 per 100,000 population for the most recent years of data, being lowest in Sweden (4.68 per 100,000), Spain (5.38 per 100,000) and New Zealand (5.55 per 100,000), and highest in the UK (9.84 per 100,000 in England and Wales, 10.71 per 100,000 in Scotland) and Japan (10.26 per 100,000). Positive associations with male sex and increasing age were consistently observed across all countries. There was an overall 2-3% annual increase in mortality depending on codes used for classification – for broad codes, overall rate ratio 1.03 (95% confidence intervals 1.02-1.04, p<0.001), for narrow codes, overall rate ratio 1.02 (95% confidence intervals 1.01-1.03, p<0.001). Validation in a local cohort showed that idiopathic pulmonary fibrosis was recorded as the underlying cause of death in two-thirds of known cases and anywhere on the death certificate in 80% of cases. Conclusions: Mortality from idiopathic pulmonary fibrosis is increasing steadily worldwide, despite that fact that death certification will almost certainly underestimate true mortality. We estimate that there will be between 28000-65000 deaths in Europe and 13000-17000 deaths in the USA from idiopathic pulmonary fibrosis clinical syndrome in 2014. Variation between countries remains but is less than previously reported

Cardiovascular outcomes following a respiratory tract infection among adults with non-CF bronchiectasis: a general population based study

Rationale: Studies suggest that people with bronchiectasis are at increased risk of cardiovascular co-morbidities. Objectives: We aimed to quantify the relative risk of incident cardiovascular events following a respiratory tract infection amongst people with bronchiectasis. Methods: Using UK electronic primary care records, we conducted a within-person comparison using the self-controlled case series method. We calculated the relative risk of first time cardiovascular events (either first myocardial infarction [MI] or stroke) following a respiratory tract infection compared with the individual’s baseline risk. Results: Our cohort consisted of 895 individuals with non-CF bronchiectasis with a first MI or stroke and at least one respiratory tract infection. There was an increased rate of first time cardiovascular events in the 91 day period after a respiratory tract infection (Incidence Rate Ratio [IRR] 1.56; 95% CI 1.20 to 2.02). The rate of a first cardiovascular event was highest in the first three days following a respiratory tract infection (IRR 2.73, 95% CI 1.41 to 5.27). Conclusions: These data suggest that respiratory tract infections are strongly associated with a transient increased risk of first time MI or stroke amongst people with bronchiectasis. As respiratory tract infections are six times more common in people with bronchiectasis than the general population, the increased risk has a disproportionately greater impact in these individuals. These findings may have implications for including cardiovascular risk modifications in airway infection treatment pathways in this population

Risk factors for cardiovascular disease in people with idiopathic pulmonary fibrosis: a population-based study

OBJECTIVE: People with idiopathic pulmonary fibrosis (IPF) have been shown to be at an increased risk for cardiovascular (CV) disease, but reasons for this are unknown. The aim of this study was to compare the prevalence of common CV risk factors in people with IPF and the general population and establish the incidence of ischemic heart disease (IHD) and stroke after the diagnosis of IPF, controlling for these risk factors. METHODS: We used data from a large, UK primary care database to identify incident cases of IPF and matched general-population control subjects. We compared the prevalence of risk factors for CV disease and prescription of CV medications in people with IPF (before diagnosis) with control subjects from the general population and assessed the incidence of IHD and stroke in people with IPF (after diagnosis) compared with control subjects. RESULTS: We identified 3,211 cases of IPF and 12,307 control subjects. Patients with IPF were more likely to have a record of hypertension (OR, 1.31; 95% CI, 1.19-1.44), and diabetes (OR, 1.20; 95% CI, 1.07-1.34) compared with control subjects; they were also more likely to have been prescribed several CV drugs. The rate of first-time IHD events was more than twice as high in patients than control subjects (rate ratio, 2.32; 95% CI, 1.85-2.93; P &lt; .001), but the incidence of stroke was only marginally higher (P = .09). Rate ratios for IHD and stroke were not altered substantially after adjusting for CV risk factors. CONCLUSIONS: Several CV risk factors were more prevalent in people with IPF; however, this did not account for the increased rate of IHD in this group of patients

Changing causes of death for patients with chronic respiratory disease in England, 2005-2015

Background: Chronic respiratory diseases (CRD) are common, increasing in prevalence, and cause significant morbidity and mortality worldwide. However, we have limited knowledge on causes of death of patients with CRD in the general population. Objective: We evaluated mortality rates and causes of death over time in CRD patients.Methods: We used linked primary care and mortality data to determine mortality rates and the most common causes of death in people with CRD (including asthma, bronchiectasis, chronic obstructive pulmonary disease (COPD), and interstitial lung diseases (ILD)) during 2005-2015 in England. Results: We identified 558,888 CRD patients (451,830 asthma, 137,709 COPD, 19,374 bronchiectasis, 10,745 ILD). The age-standardised mortality rate of patients with CRD was 1,607 per 100,000 persons (asthma=856, COPD=1,503, ILD=2,609, bronchiectasis=1,463). CRD mortality was overall 54% higher than the general population. One third of CRD patients died from respiratory-related causes. Respiratory-related mortality was constant, while cardiovascular-related mortality decreased significantly over time. COPD accounted for the majority of respiratory-related deaths (66% overall) in all patient groups except ILD. Conclusions: CRD patients continue to experience substantial morbidity and mortality due to respiratory diseases. Disease-modifying intervention strategies are needed to improve outcomes for patients with CRD

Presence of a prothrombotic state in people with idiopathic pulmonary fibrosis: a population-based case-control study

BACKGROUND: Laboratory studies suggest that the clotting cascade is activated in fibrotic lungs. Since humans vary in their tendency to clot due to a variety of inherited or acquired defects, we investigated whether a prothrombotic state increases the chance of developing idiopathic pulmonary fibrosis (IPF) and/or worsens the prognosis of IPF. METHODS: We recruited 211 incident cases of IPF and 256 age-and sex-matched general population controls and collected data on medical history, medication, smoking habit, blood samples as well as lung function and high-resolution CT scans done as part of routine clinical care. A prothrombotic state was defined as the presence of at least one inherited or acquired clotting defect or marker of fibrinolytic dysfunction. We used logistic regression to quantify the association between a prothrombotic state and IPF adjusted for age, sex, smoking habit and highly sensitive C reactive protein. Cox regression was used to determine the influence of a prothrombotic state on survival. RESULTS: Cases were more than four times more likely than controls to have a prothrombotic state (OR 4.78, 95% CI 2.93 to 7.80; p<0.0001). Cases with a prothrombotic state were also likely to have more severe disease (forced vital capacity <70% predicted) at presentation (OR 10.79, 95% CI 2.43 to 47.91) and had a threefold increased risk of death (HR 3.26, 95% CI 1.09 to 9.75). CONCLUSIONS: People with IPF are more likely to have a prothrombotic state than general population controls and the presence of a prothrombotic state has an adverse impact on survival