Non-cystic fibrosis bronchiectasis (NCFBE) is a chronic respiratory disease characterized by a clinical syndrome of chronic productive cough and recurrent respiratory infections in the presence of abnormal and permanent dilation of the bronchi. Recent epidemiological studies have clearly shown that the prevalence and incidence of NCFBE are quickly rising both in high- and low-income countries. With the increase of prevalence, bronchiectasis brings huge medical and economic burden to the society. In this study, the investigator will perform biomarker assessments and multi-omics analysis on NCFBE patients and healthy participants in China to validate the link of disease pathways to pathophysiological features and uncover the molecular endotypes behind clinical phenotypesof Chinese patients with NCFBE.
Background/Rationale: Non-cystic fibrosis bronchiectasis (NCFBE) is a chronic respiratory disease characterized by a clinical syndrome of chronic productive cough and recurrent respiratory infections in the presence of abnormal and permanent dilation of the bronchi. Recent epidemiological studies have clearly shown that the prevalence and incidence of NCFBE are quickly rising both in high- and low-income countries. With the increase of prevalence, bronchiectasis brings huge medical and economic burden to the society. Recently published Chinese Bronchiectasis Registry study (BE-China) data showed that Chinese bronchiectasis patients exhibit unique clinical characteristics when compared to western countries. The proportion of post-infective causes and tuberculosis in China was twice that of the European Multicenter Bronchiectasis Audit and Research Collaboration (EMBARC) cohort. Moreover, Chinese patients had a lower FEV1% of predicted value, and a higher proportion of obstruction compared to the EMBARC cohort. Pseudomonas aeruginosa (PsA) was the most common pathogen in both cohorts. Chinese patients exhibited a higher frequency of hospitalization compared to the EMBARC cohort (57.2% vs 26.4%); however, unlike the frequency of hospitalization, Chinese patients had fewer exacerbations in the year before enrollment compared to the EMBARC cohort, with most having only one exacerbation. The proportion of patients with three or more exacerbations was much higher in the EMBARC cohort than in China (12.3% vs 38.8%). Except for Aspergillus fumigatus, the positive culture rates of other pathogens were much higher in the EMBARC cohort than in China. Significant difference in aetiology and clinical phenotypes of NCFBE has been demonstrated by many registry studies conducted in different geographical regions including EMBARC and BE-China. However, the biological processes and mechanisms driving the disease development, so-called "endotypes", have not been fully investigated within the patient populations in these studies. It is remaining unknown that if these differences reported in clinical phenotypes were truly caused by or linked to different endotypes in NCFBE. In this study, the investigator will perform biomarker assessments and multi-omics analysis on NCFBE patients and healthy participants in China to validate the link of disease pathways to pathophysiological features and uncover the molecular endotypes behind clinical phenotypesof Chinese patients with NCFBE.
Study Type
OBSERVATIONAL
Enrollment
320
This is a longitudinal multi-center, observational, translational study which includes patients with a physician diagnosis of NCFBE by chest HRCT and healthy controls (at baseline only). This study will consist of a baseline visit, a 6-month (site visit or telephone visit) and a 12-month visit as well as planned unscheduled visits for exacerbation events and one optional visit for bronchoscopy.Healthy participants will be only enrolled in the baseline visit and bronchoscopy visit.
Beijing Chaoyang Hospital Affiliated to Capital Medical University
Beijing, Beijing Municipality, China
ACTIVE_NOT_RECRUITINGBeijing Hospital
Beijing, Beijing Municipality, China
RECRUITINGPeking University People's Hospital
Beijing, Beijing Municipality, China
ACTIVE_NOT_RECRUITINGThe Second Affiliated Hospital of Chongqing Medical University
Chongqing, Chongqing Municipality, China
FEV1/FVC%
Evaluation of lung function
Time frame: 12 month
FEF25-75 L/s
Evaluation of small airway function
Time frame: 12 month
FENO ppb
Evaluation of airway inflammation: FENO.
Time frame: 12 month
HRCT
Evaluation of lung structure profile and change through radiological parameters.
Time frame: 12 month
cell percentage (%)
Measurement of immune cell (including but not limited to neutrophils and eosinophils) percentages in blood
Time frame: 12 month
Gene expression read counts by RNAseq
Functional and transcriptional characterization of airway immune cells, bronchial epithelial cells andsmooth muscle cells (optional).
Time frame: 12 month
Molecular deliverables
MUC5AC/5B in sputum
Time frame: 12 month
on-set age(years-old)
Risk factor assessment: on-set age(years-old)
Time frame: 12 month
Sex(M/F)
Risk factor assessment: Sex(M/F)
Time frame: 12 month
body mass index(kg/m^2)
Risk factor assessment: body mass index(kg/m\^2)
Time frame: 12 month
comorbidities
Risk factor assessment: comorbidities
Time frame: 12 month
medical history
Risk factor assessment: medical history, especially TB history
Time frame: 12 month
smoking status(never/current/former)
Risk factor assessment: smoking status(never/current/former)
Time frame: 12 month
smoking pack years(pack/year)
Risk factor assessment: smoking pack years(pack/year)
Time frame: 12 month
Sputum microbiology (CFU)
Bronchiectasis aetiology evaluation
Time frame: 12 month
Historical Exacerbation
Risk factor assessment: exacerbation number in the previous year
Time frame: 12 month
BSI score
Evaluation of ronchiectasis disease severity: BSI score(0-4 mild,5-8 moderate, ≥9 severe)
Time frame: 12 month
QoL-B-RSS
Evaluation of quality of life: Patient reported outcome: QoL-B-RSS (0-100, higher score stands for lower symptom burden and higher quality of life)
Time frame: 12 month
BHQ
Evaluation of quality of life: Patient reported outcome: BHQ(10-70,higher score stands for higher symptom burden and pooer quality of life )
Time frame: 12 month
BEST
eDiary: BEST(MCID 4 points may standfor an exacerbation.)
Time frame: 12 month
Treatment pattern
Evaluation of treatment pattern: inhaled antibiotic, macrolide, and mucoactive drugs,etc.
Time frame: 12 month
Exacerbation assessment
Exacerbation assessment: number of exacerbations per patient per year
Time frame: 12 month
Cell counts (10^9/L)
Measurement of immune cell (including but not limited to neutrophils and eosinophils) counts in blood
Time frame: 12 month
Exacerbation assessment about hospitalization
Exacerbation assessment: number of exacerbations lead to hospitalization per patient per year
Time frame: 12 month
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The Second Affiliated Hospital of Fujian Medical University
Quanzhou, Fujian, China
RECRUITINGGansu Provincial Hospital
Lanzhou, Gansu, China
ACTIVE_NOT_RECRUITINGShenzhen People's Hospital
Shenzhen, Guangdong, China
ACTIVE_NOT_RECRUITINGAffiliated Hospital of Guangdong Medical University"
Zhanjiang, Guangdong, China
RECRUITINGThe First Affiliated Hospital of Guangxi Medical University
Nanning, Guangxi, China
RECRUITINGGuizhou Provincial People's Hospital
Guiyang, Guizhou, China
RECRUITING...and 28 more locations