Frequency Of Pulmonary Exacerbations Research Articles

Respiratory rehabilitation techniques for patients with cystic fibrosis: a protocol for a systematic review and network meta-analysis

IntroductionCystic fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, primarily affecting the respiratory and digestive systems. Respiratory rehabilitation...

Correlation Between Vitamin E Levels and Cholesterol, Vitamin D, and Frequency of Pulmonary Exacerbations in Children With Cystic Fibrosis.

Introduction Children with cystic fibrosis (CF) have lipidmaldigestion due to pancreatic insufficiency, which causes malabsorption of fat-soluble vitamins. The primary objective of this study was to assess the prevalence of vitamin E deficiency among children with CF. The secondary objectivewas to examine the correlation between vitamin E levels with demographic data, laboratory findings, and the number of pulmonary exacerbations. Furthermore, the study aimed to identify potential predictors of vitamin E deficiency in this population. Methods A prospective cohort study was conducted from July 1, 2017, to April 30, 2019. Medical records of children diagnosed with CF at the Department of Pediatrics, Salmaniya Medical Complex, Bahrain were reviewed. Patients who didn't receive fat-soluble vitamin supplementation for at least three days were recruited for the study.Light-protected blood samples were tested for vitamin E and D levelsand fasting serum cholesterol levels. Patients with vitamin E deficiency were compared with those without regarding demography, laboratory results, and number of pulmonary exacerbations. Results Of 109 patients with CF, 35 (32.1%) fulfilled the inclusion criteria. Eighteen (51.4%) were males. The mean age was 6.8 ± 4.7 years. Eleven (31.4%) patients were symptomatic. Vitamin E and D were deficient in nine (25.7%) and 28/34 (82.4%) patients, respectively. Cholesterol was low in 29 (82.9%). The mean vitamin E level in the deficient group was significantly lower (3.2 ± 1.7 mg/L) than that (10.3 ± 3.1 mg/L) of the vitamin E-sufficient group (P< 0.0001). A significant negative correlation was noted between vitamin E levels and white blood cell (WBC) count (r = -0.408; P = 0.015). However, no correlation was found between vitamin E levels andcholesterol, vitamin D levels, or the number of pulmonary exacerbations. Vitamin E-deficient patients had lower weight at presentation (P = 0.045), hemoglobin level (P = 0.001), and salbutamol use (P = 0.022), but higher reticulocyte percentage (P = 0.034) and WBC count (P = 0.001) compared to the vitamin E-sufficient group. Conclusion Vitamin E deficiency is common among patients with CF in Bahrain and may increase the risk of hemolytic anemia. This deficiency did not seem to affect the frequency of pulmonary exacerbations. Management of vitamin E deficiency in patients with CF should be hastened to avoid irreversible complications.

Adapting the cystic fibrosis care model: Perspectives from people with CF, caregivers, and members of CF care teams

BackgroundRapidly emerging clinical trends offer the opportunity to amend guidance on issues pertaining to CF care delivery. A national survey was conducted to gather perspectives on CF care including potential adaptations to the care model to best meet the needs of this population. MethodsA survey instrument was developed to capture perspectives on CF care. People with CF (pwCF), including those post lung transplant, caregivers and care teams were surveyed. Descriptive statistics were calculated to characterize respondents and responses. ResultsIn-person, routine visits with the CF care teams were valued by survey respondents. However, reduced in-person visit frequency from the standard three-month interval was supported for individuals in a stable state of health. This was particularly true for pwCF ages two or older and on a modulator. Lung function, pulmonary exacerbation frequency, and transition periods were noted to influence preference for visit frequency. Integrating telehealth with remote monitoring in between visits was broadly supported. For shared care between CF teams and other medical providers (transplant teams and primary care providers (PCP)), good communication, easily accessible health records, and convenient locations were important. ConclusionsSurvey findings support adapting CF care based on individual needs and life transitions. Themes identified can inform future areas of study and resource development to support successful modification of the CF care model and shared decision-making between patients and their care providers.

Long-Term Clinical Efficacy of Elexacaftor-Tezacaftor-Ivacaftor in People With Cystic Fibrosis and Preexisting Advanced Lung Disease at Treatment Initiation

BackgroundElexacaftor-tezacaftor-ivacaftor (ETI) is associated with increased forced expiratory volume in the first second (FEV1), decreased exacerbation frequency, and increased body mass index (BMI) in people with Cystic Fibrosis (pwCF) [1, 2]. Landmark clinical trials excluded patients with advanced lung disease (ALD) as defined by FEV1<40%. We have previously reported an improvement in ppFEV1 of 7.9% after 3 months of ETI in people with CF and ALD (ACFLD)[3]. The long-term effects of ETI on this cohort are unknown. This study reports the efficacy of ETI following 24 months of treatment in people with ACFLD. Study DesignWe conducted a retrospective cohort study of adult patients with FEV1<40% and/or other high-risk features defined by the 2019 CFF lung transplant guidelines who were started on ETI between September 2019 and February 2020. Response to therapy was assessed with repeat spirometry measured at 12 and 24 months. All measurements were taken outside of an acute exacerbation. Demographics and clinical data including BMI and pulmonary exacerbation frequency were extracted from the medical record. Results57 of 64 people with ACFLD showed improved lung function with a mean change in ppFEV1 of 6.74% (p = <0.001, 95% CI 4.25 – 9.23%) between baseline and 24 months of treatment. BMI increased by a mean change of 1.55 kg/m2 (p = <0.001, 95% CI 0.93 – 2.18 kg/m2) during this interval. The annual exacerbation rate between the year before ETI and 24 months on ETI declined with a median of 1 fewer per year (p = 0.0007). InterpretationPeople with ACFLD experienced a significant increase in lung function at 24 months on ETI, although less than those with higher baseline lung function compared to prior studies [1, 2]. They also had an increase in BMI and a decline in the rate of annual pulmonary exacerbations.

Clinical Significance of Mucor in Airway Culture of Immunocompetent Patients With Chronic Lung Disease.

Mucor within the airways of immunocompromised patients often signifies an invasive life-threatening infection. However, its significance in immunocompetent patients with chronic lung diseases is less clear. We aimed to assess the clinical implication of mucor in airway-secretion cultures of these patients. A single-center retrospective cohort study was performed. Patients with cystic fibrosis (CF), primary ciliary dyskinesia (PCD) or non-CF/non-PCD bronchiectasis followed in our Pediatric Pulmonary Institute, with sputum or bronchoalveolar lavage cultures growing Mucorales molds in the years 2010-2022, were included. Demographic and clinical parameters such as body mass index and spirometry values (forced expiratory volume at 1 second) were collected and compared with values up to 12 months prior to and following the index (positive culture) visit. A total of 27 patients of whom 22 (82%) patients were with CF, 3 with PCD (11%) and 2 (7%) with non-CF/non-PCD bronchiectasis were included. Median age was 21.8 (14.9-32.1) years, with forced expiratory volume at 1 second of 62.8% ± 21.9% at the index visit. None of the patients developed disseminated disease, none had clinical or radiological evidence of fungal disease and none required antifungal therapy. Throughout the 12 months prior to and following the positive cultures, no significant changes were noted in body mass index, forced expiratory volume at 1 second, frequency of pulmonary exacerbations, days of hospitalization or days of antibiotic treatment. Evidence of mucor in airway cultures of immunocompetent patients with chronic lung disease does not necessarily signify clinical deterioration nor suggests invasive fungal disease. Larger, long-term prospective studies are required to obviate the need for a thorough evaluation in these patients.

Predicting lung function decline in cystic fibrosis: the impact of initiating ivacaftor therapy

BackgroundModulator therapies that seek to correct the underlying defect in cystic fibrosis (CF) have revolutionized the clinical landscape. Given the heterogeneous nature of lung disease progression in the post-modulator era, there is a need to develop prediction models that are robust to modulator uptake.MethodsWe conducted a retrospective longitudinal cohort study of the CF Foundation Patient Registry (N = 867 patients carrying the G551D mutation who were treated with ivacaftor from 2003 to 2018). The primary outcome was lung function (percent predicted forced expiratory volume in 1 s or FEV1pp). To characterize the association between ivacaftor initiation and lung function, we developed a dynamic prediction model through covariate selection of demographic and clinical characteristics. The ability of the selected model to predict a decline in lung function, clinically known as an FEV1-indicated exacerbation signal (FIES), was evaluated both at the population level and individual level.ResultsBased on the final model, the estimated improvement in FEV1pp after ivacaftor initiation was 4.89% predicted (95% confidence interval [CI]: 3.90 to 5.89). The rate of decline was reduced with ivacaftor initiation by 0.14% predicted/year (95% CI: 0.01 to 0.27). More frequent outpatient visits prior to study entry and being male corresponded to a higher overall FEV1pp. Pancreatic insufficiency, older age at study entry, a history of more frequent pulmonary exacerbations, lung infections, CF-related diabetes, and use of Medicaid insurance corresponded to lower FEV1pp. The model had excellent predictive accuracy for FIES events with an area under the receiver operating characteristic curve of 0.83 (95% CI: 0.83 to 0.84) for the independent testing cohort and 0.90 (95% CI: 0.89 to 0.90) for 6-month forecasting with the masked cohort. The root-mean-square errors of the FEV1pp predictions for these cohorts were 7.31% and 6.78% predicted, respectively, with standard deviations of 0.29 and 0.20. The predictive accuracy was robust across different covariate specifications.ConclusionsThe methods and applications of dynamic prediction models developed using data prior to modulator uptake have the potential to inform post-modulator projections of lung function and enhance clinical surveillance in the new era of CF care.

Reliability of comorbidity indices as predictive indicators for frequent severe chronic obstructive pulmonary disease exacerbations.

The relationship between comorbidities and chronic obstructive pulmonary disease (COPD) is two-sided. As the number of comorbidities increases, frequency of acute exacerbations of COPD (AECOPD) consequently increases. Comorbidity indices can be used to evaluate comorbidities while managing COPD patients. We aimed to compare comorbidity indices such as the Charlson comorbidity index (CCI), comorbidities in COPD index (COMCOLD) and COPD specific comorbidity test (COTE) regarding exacerbation frequency. Participants hospitalized for AECOPD were included in this bidirectional case-control study. Exacerbation severity, frequency, further exacerbations over a one-year follow-up period and CCI, COMCOLD, and COTE scores were recorded. High and low comorbidity groups were compared regarding AECOPD frequency, severity, and further exacerbations. Ninety-two patients were enrolled. The frequency of AECOPD was significantly higher in high-comorbidity groups (p= 0.026 for CCI; 0.015 for COTE; 0.012 for COMCOLD) than that in low-comorbidity groups. Severe AECOPD was significantly higher in all high-comorbidity groups according to the indices. Median number of exacerbations during the one-year follow-up period was significantly higher in the high-comorbidity groups defined by CCI [0 (0-4) vs. 1 (0-4), p<0.001 and COMCOLD 0 (0-4) vs. 1 (0-3), p= 0.007]. Comorbidities are among the most important risk factors for AECOPD. Managing comorbidities begins with their identification, followed by appropriate interventions. Therefore, using at least one comorbidity index during assessment ensures that comorbidities are not overlooked during diagnostic and therapeutic processes. CCI, COTE, and COMCOLD comorbidity indices can be used in predicting COPD exacerbations.

Real-world effectiveness of elexacaftor/tezacaftor/ivacaftor on the burden of illness in adolescents and adults with cystic fibrosis

BackgroundElexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) has been shown to be safe and efficacious in people with cystic fibrosis (CF) aged ≥2 years. Here, we describe results from an observational study assessing change in burden of illness following initiating ELX/TEZ/IVA in real-world settings. MethodsThis US-based, multicenter, observational study used data from electronic medical records to evaluate real-world burden of illness before and after ELX/TEZ/IVA initiation in people with CF aged ≥12 years heterozygous for F508del and a minimal function mutation (F/MF) or an uncharacterized CFTR mutation. Endpoints included absolute change from baseline in percent predicted forced expiratory volume in 1 s (ppFEV1), body mass index (BMI) and BMI-for-age z-score, glycated hemoglobin (HbA1c), and numbers of pulmonary exacerbations (PEx). ResultsOverall, 206 people with CF were enrolled (mean [SD] age 22.5 [11.1] years; 192 [93.2%] with F/MF genotype). Mean follow-up was 15.6 (SD, 1.6) months. Improvements in ppFEV1 (7.3 [95% CI: 5.7, 8.8] percentage points) were observed from baseline through follow-up. Increases in BMI (1.40 [95% CI: 1.07, 1.77] kg/m2) and BMI-for-age z-score (0.14 [95% CI: 0.00, 0.28]) were also observed from baseline at 12 months. The estimated annualized rate of any PEx was 1.31 at baseline and 0.61 over follow-up (rate ratio 0.47 [95% CI: 0.39, 0.55]), with annualized rates of PEx requiring antibiotics and hospitalizations of 0.55 and 0.88 in the baseline period and 0.12 and 0.36 over follow-up (rate ratios 0.22 [95% CI: 0.15, 0.31] and 0.41 [95% CI: 0.32, 0.51]), respectively. Absolute change in HbA1c was −0.22 (95% CI: -0.38, −0.06) from baseline through follow-up. ConclusionsELX/TEZ/IVA treatment was associated with improved lung function, increased BMI, reduced frequency of PEx, and improved (i.e., reduced) HbA1c. These results confirm the broad clinical benefits of ELX/TEZ/IVA seen in clinical trials and show the potential for ELX/TEZ/IVA to improve markers of glucose metabolism.

Diminished airway host innate response in people with cystic fibrosis who experience frequent pulmonary exacerbations.

Pulmonary exacerbations are clinically impactful events that accelerate cystic fibrosis (CF) lung disease progression. The pathophysiological mechanisms underlying an increased frequency of pulmonary exacerbations have not been explored. To compare host immune response during intravenous antibiotic treatment of pulmonary exacerbations in people with CF who have a history of frequent versus infrequent exacerbations. Adults with CF were recruited at onset of antibiotic treatment of a pulmonary exacerbation and were categorised as infrequent or frequent exacerbators based on their pulmonary exacerbation frequency in the previous 12 months. Clinical parameters, sputum bacterial load and sputum inflammatory markers were measured on day 0, day 5 and at the end of treatment. Shotgun proteomic analysis was performed on sputum using liquid chromatography-mass spectrometry. Many sputum proteins were differentially enriched between infrequent and frequent exacerbators (day 0 n=23 and day 5 n=31). The majority of these proteins had a higher abundance in infrequent exacerbators and were secreted innate host defence proteins with antimicrobial, antiprotease and immunomodulatory functions. Several differentially enriched proteins were validated using ELISA and Western blot including secretory leukocyte protease inhibitor (SLPI), lipocalin-1 and cystatin SA. Sputum from frequent exacerbators demonstrated potent ability to cleave exogenous recombinant SLPI in a neutrophil elastase dependent manner. Frequent exacerbators had increased sputum inflammatory markers (interleukin (IL)-1β and IL-8) and total bacterial load compared to infrequent exacerbators. A diminished innate host protein defence may play a role in the pathophysiological mechanisms of frequent CF pulmonary exacerbations. Frequent exacerbators may benefit from therapies targeting this dysregulated host immune response.

Improved recognition of lung function decline as signal of cystic fibrosis pulmonary exacerbation: a Cystic Fibrosis Learning Network Innovation Laboratory quality improvement initiative

IntroductionCystic fibrosis (CF) is a systemic autosomal recessive condition characterised by progressive lung disease. CF pulmonary exacerbations (PEx) are episodes of worsening respiratory status, and frequent PEx are a risk...

Chronic Rhinosinusitis with Nasal Polyposis in People with Cystic Fibrosis

Cystic fibrosis (CF) is an autosomal recessive disorder that results in deranged ion transport and affects multiple organ systems, including the upper and lower respiratory tracts. People with CF (PwCF) often develop chronic rhinosinusitis (CRS) with or without nasal polyposis. CRS can significantly decrease quality of life for PwCF and can lead to more frequent pulmonary exacerbations. The management of CRS in PwCF is different from that in individuals without CF. Novel therapies have emerged in the last several years that have drastically altered the progression of both pulmonary and sinonasal disease in people with CF. It is critical for providers who manage CF-related CRS to understand the unique characteristics and challenges that coincide with this disease process. This review article aims to provide readers with an overview of the pathophysiology of CF and to summarize best practice strategies for the management of CF-related CRS.

Clinical significance of BPI-ANCA in patients with cystic fibrosis: a single center prospective study

Recurrent pulmonary exacerbation due to infection and inflammation remain the major cause of mortality and morbidity in patients with cystic fibrosis (CF). Increased levels of BPI-ANCA have been linked to Pseudomonas colonization and pulmonary exacerbations in patients with CF. The majority of these studies were done in Europe, and it is unclear whether similar findings are true in CF patients who lives in United States. In our single center study of 47 patients with CF, the prevalence of BPI-ANCA was 19% at baseline and 15% at annual follow-up visit. Overall, there were no statistical differences noted in FEV1 and frequency of pulmonary exacerbations in CF patients who were positive for BPI-ANCA compared to those who were negative for BPI-ANCA. The role of BPI-ANCA in patients with CF still remains unclear.

730 Longitudinal impact of pulmonary exacerbation frequency on lung function in children with cystic fibrosis

730 Longitudinal impact of pulmonary exacerbation frequency on lung function in children with cystic fibrosis

Dynamic Hyperinflation While Exercising-A Potential Predictor of Pulmonary Deterioration in Cystic Fibrosis.

Lung function deterioration in cystic fibrosis (CF) is typically measured by a decline in the forced expiratory volume in one second (FEV1%), which is thought to be a late marker of lung disease. Dynamic hyperinflation (DH) is seen in obstructive lung diseases while exercising. Our aim was to assess whether DH could predict pulmonary deterioration in CF; a secondary measure was the peak VO2. A retrospective study was conducted of people with CF who performed cardiopulmonary exercise tests (CPETs) during 2012-2018. The tests were classified as those demonstrating DH non-DH. Demographic, genetic, and clinical data until 12.2022 were extracted from patient charts. A total of 33 patients aged 10-61 years performed 41 valid CPETs with valid DH measurements; sixteen (39%) demonstrated DH. At the time of the CPETs, there was no difference in the FEV1% measurements between the DH and non-DH groups (median 83.5% vs. 87.6%, respectively; p = 0.174). The FEV1% trend over 4 years showed a decline in the DH group compared to the non-DH group (p = 0.009). A correlation was found between DH and the lung clearance index (LCI), as well as the FEV1% (r = 0.36 and p = 0.019 and r = -0.55 and p = 0.004, respectively). Intravenous (IV) antibiotic courses during the 4 years after the CPETs were significantly more frequent in the DH group (p = 0.046). The peak VO2 also correlated with the FEV1% and LCI (r = 0.36 and p = 0.02 and r = -0.46 and p = 0.014, respectively) as well as with the IV antibiotic courses (r = -0.46 and p = 0.014). In our cohort, the DH and peak VO2 were both associated with lung function deterioration and more frequent pulmonary exacerbations. DH may serve as a marker to predict pulmonary deterioration in people with CF.

Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF Registry.

Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) improves multiple clinical outcomes in people with cystic fibrosis (pwCF) with at least one F508del allele. This study evaluated the real-world impact of ETI on lung function, nutritional status, pulmonary exacerbation frequency, and sweat chloride concentrations in a large group of pwCF. This observational cohort study used data from the German CF Registry for pwCF who received ETI therapy and were followed up for a period of 12 months. The study included 2645 pwCF from 67 centres in Germany (mean age 28.0±11.5 years). Over the first year after ETI was initiated, percent predicted forced expiratory volume in 1s (ppFEV1) increased by 11.3% (95% confidence interval [CI] 10.8-11.8, p<0.0001), body mass index (BMI) z-score increased by 0.3 (95% CI 0.3-0.4, p<0.0001) in individuals aged 12 to <18 years and BMI in adults increased by 1.4kg/m2 (95% CI 1.3-1.4, p<0.0001), pulmonary exacerbations decreased by 75.9% (p<0.0001) and mean sweat chloride concentration decreased by 50.9mmol/L (95% CI -52.6,-49.3, p<0.0001). Improvements in ppFEV1 over the first year of therapy were greater in pwCF who had not previously received cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy (12.6% [95% CI 11.9-13.4] vs. 9.7% [95% CI 9.0-10.5] in those with prior CFTR modulator treatment. These real-world data are consistent with the findings of randomised clinical trials, and support the use of ETI as a highly effective treatment option for pwCF who have at least one F508del allele. None.

Pulmonary Function Tests in the Evaluation of Early Lung Disease in Cystic Fibrosis.

Properly evaluating respiratory system dysfunction is essential in children with cystic fibrosis (CF). This prospective study aimed to assess the course of early lung disease based on multiple breath nitrogen washout (MBNW), impulse oscillometry (IOS), and conventional techniques, such as spirometry and body plethysmography. Over a 2 year recruitment period, subjects with CF aged 7-18 performed pulmonary function tests (PFTs). Moreover, the nutritional and microbiological status, frequency of pulmonary exacerbations (PExs), and patients' health-related quality of life (HRQoL) were assessed. The mean age of the children (n = 69) was 14.09 ± 3.26 years; F/M 37/32. Spirometry-based diagnoses of normal lung function (forced expiratory volume in 1 s, FEV1 ≥ 90%pred), mild (FEV1 70-89%pred) and moderate (FEV1 40-69%pred) lung diseases were established in 34 (49.3%), 25 (36.2%), and 10 (14.5%) patients, respectively. An elevated lung clearance index (LCI > 6.98) was observed in 85% of the subjects with normal FEV1. The presence of Pseudomonas aeruginosa infection (n = 16) and the number of PExs treated with IV antibiotics were associated with significantly worse PFT results. MBNW and IOS are more helpful tools than conventional techniques in assessing early lung disease in CF. LCI is a more useful parameter for detecting functional abnormalities than FEV1 in school-age children.

P224 Eligibility of CFTR Modulator [HB1] drugs in patients registered in cystic fibrosis registry of Turkey

CFTR modulatory treatments have resulted in decrease in the frequency of pulmonary exacerbations, increase in quality of life and prolongation of lifespan in CF patients. However, since these drugs are not covered by health insurance in Turkey, eligible patients may sometimes have access to the therapy through lawsuits. In this study, we aimed to determine the eligibility of patients in our country for the modulators.

P184 Sex differences in annual pulmonary exacerbations in people with cystic fibrosis

Pulmonary exacerbations (PEx) are a major contributor to disease progression in people with cystic fibrosis (CF). While studies have demonstrated that females with CF experiencing PEx have worse clinical outcomes compared to males, there is currently a lack of epidemiologic studies comparing the frequency of PEx in the current era. We therefore utilized the Cystic Fibrosis Foundation Patient Registry (CFFPR) to evaluate whether the burden of PEx differs between females and males with CF.

P250 Minimum inhibitory concentration targeted antibiotic dose optimization in patients with cystic fibrosis

Frequent pulmonary exacerbations of Cystic Fibrosis (CF) reduce survival and quality of life even among patients receiving highly effective modulator therapy suggesting the need for better treatments. Our primary objective was to discern whether antibiotics matched with bacterial culture susceptibilities and meeting minimum inhibitory concentration (MIC)-based target serum levels improve pulmonary exacerbation treatment outcomes. MIC directed antibiotic dose selection and optimization therapy is standard practice at our center.

Pulmonary exacerbations in insured patients with bronchiectasis over 2 years.

Patients with bronchiectasis experience persistent symptoms and frequent pulmonary exacerbations; this study investigated the frequency of exacerbations and all-cause hospitalisation. This longitudinal, retrospective, claims database study (IBM® MarketScan®) identified patients aged ≥18 years from 1 July 2015 through 30 September 2018. Exacerbations were identified by bronchiectasis inpatient claim or a healthcare interaction, followed by antibiotic prescription within 7 days. Patients with ≥36 months of continuous health plan enrolment (12 months preceding the first bronchiectasis claim, i.e., baseline period and ≥24 months of follow-up) were included. Patients with cystic fibrosis at baseline were excluded. A multivariable logistic regression model identified baseline factors associated with having ≥2 exacerbations over the 2-year follow-up period. In total, 14 798 patients with bronchiectasis were identified; 64.5% were female, 82.7% were aged ≥55 years and 42.7% had ≥2 exacerbations at baseline. Having ≥2 exacerbations after 2 years was positively associated with chronic macrolide use, long-acting β2 agonist use, gastro-oesophageal reflux disease, heart failure and Pseudomonas aeruginosa. Frequent exacerbations (≥2) at baseline were significantly associated with greater likelihood of experiencing ≥2 exacerbations during the first and second year's follow-up (unadjusted odds ratios 3.35 (95% CI 3.1-3.6) and 2.96 (95% CI 2.8-3.2), respectively). The proportion of patients experiencing ≥1 all-cause hospitalisation cumulatively increased from 41.0% in the first year of follow-up to 51.1% over 2 years' follow-up. Frequent exacerbations in patients with bronchiectasis may increase the likelihood of future exacerbations over 2 years of follow-up, with increased hospitalisation rates over time.