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Seminars in Respiratory and Critical... Aug 2021Bronchiectasis is a heterogenous disease with multiple etiologies and associated comorbidities. As bronchiectasis is a complex disease, it is unsound to think of it as a...
Bronchiectasis is a heterogenous disease with multiple etiologies and associated comorbidities. As bronchiectasis is a complex disease, it is unsound to think of it as a single disease particularly when the differing etiologies are likely to be driving bronchiectasis through initial divergent molecular pathways, known as endotypes, that phenotypically present as the same disease due to protracted airway inflammation, but revealing potential differing underlying mechanisms that may have disparity of drug responses. Improved understanding of the cellular immune, inflammatory, and microbiological milieu associated with clinical and radiological features of bronchiectasis has resulted in the recognition of important endotypes and phenotypes that will allow for personalized treatments to improve quality of life and outcomes of patients with bronchiectasis. Here we discuss clinical and radiological phenotypes, as well as emerging molecular endotypes that are possible treatable traits in bronchiectasis.
Topics: Bronchiectasis; Humans; Phenotype; Quality of Life; Radiography
PubMed: 34261179
DOI: 10.1055/s-0041-1730894 -
Ugeskrift For Laeger Nov 2022Rheumatoid arthritis (RA) affects more than 30,000 Danes. In this review, we discuss RA in connection with chronic obstructive pulmonary disease (COPD), bronchiectasis... (Review)
Review
Rheumatoid arthritis (RA) affects more than 30,000 Danes. In this review, we discuss RA in connection with chronic obstructive pulmonary disease (COPD), bronchiectasis and interstitial lung disease (ILD) which are among the most common lung manifestations and are associated with increased mortality. Early suspicion based upon respiratory symptoms should prompt imaging and pulmonary function test. Smoking cessation, vaccination, and rehabilitation are important. COPD and bronchiectasis are treated according to guidelines. Multidisciplinary collaboration in RA-ILD is important and treatment decisions are based on clinical experience and imaging suggesting an inflammatory or fibrotic phenotype.
Topics: Humans; Lung Diseases, Interstitial; Arthritis, Rheumatoid; Lung; Bronchiectasis; Pulmonary Disease, Chronic Obstructive
PubMed: 36345900
DOI: No ID Found -
Canadian Respiratory Journal 2022Primary immunodeficiencies are a group of conditions characterized by developmental or functional alterations in the immune system caused by hereditary genetic defects.... (Review)
Review
Primary immunodeficiencies are a group of conditions characterized by developmental or functional alterations in the immune system caused by hereditary genetic defects. Primary immunodeficiencies may affect either the innate or the adaptive (humoral and cellular) immune system. Pulmonary complications in primary humoral deficiencies are frequent and varied and are associated with high morbidity and mortality rates. The types of complications include bronchiectasis secondary to recurrent respiratory infections and interstitial pulmonary involvement, which can be associated with autoimmune cytopenias, lymphoproliferation, and a range of immunological manifestations. Early detection is key to timely management. Immunoglobulin replacement therapy reduces the severity of disease, the frequency of exacerbations, and hospital admissions in some primary humoral deficiencies. Therefore, the presence of pulmonary disease with concomitant infectious and/or autoimmune complications should raise suspicion of primary humoral deficiencies and warrants a request for immunoglobulin determination in blood. Once diagnosis is confirmed; early immunoglobulin replacement therapy will improve the course of the disease. Further studies are needed to better understand the pathogenesis of pulmonary disease related to primary humoral deficiencies and favor the development of targeted therapies that improve the prognosis of patients.
Topics: Bronchiectasis; Humans; Immunoglobulins; Immunologic Deficiency Syndromes; Lung; Lung Diseases
PubMed: 35440951
DOI: 10.1155/2022/7140919 -
American Journal of Respiratory and... Apr 2023
Topics: Humans; Lung; Bronchiectasis; Thorax
PubMed: 36473273
DOI: 10.1164/rccm.202211-2179ED -
Respirology (Carlton, Vic.) Dec 2020
Topics: Bronchiectasis; Child; Hospitalization; Humans
PubMed: 32608039
DOI: 10.1111/resp.13888 -
European Respiratory Review : An... Mar 2020α-antitrypsin deficiency (AATD) is a hereditary disorder associated with a risk of developing liver disease and pulmonary emphysema, and other chronic respiratory... (Review)
Review
α-antitrypsin deficiency (AATD) is a hereditary disorder associated with a risk of developing liver disease and pulmonary emphysema, and other chronic respiratory disorders (mainly asthma and bronchiectasis); Z variant is the commonest deficient variant of AAT. Determining AAT concentration in serum or plasma and identifying allelic variants by phenotyping or genotyping are fundamental in the diagnosis of AATD. Initial evaluation and annual follow-up measurement of lung function, including post-bronchodilator forced expiratory volume in 1 s and gas transfer inform on disease progression. Lung densitometry is the most sensitive measure of emphysema progression, but must not be use in the follow-up of patients in routine clinical practice. The exogenous administration of purified human serum-derived AAT is the only approved specific treatment for AATD in PiZZ. AAT augmentation therapy is not recommended in PiSZ, PiMZ or current smokers of any protein phenotype, or in patients with hepatic disease. Lung volume reduction and endoscopic bronchial valve placement are useful in selected patients, whereas the survival benefit of lung transplant is unclear. There are several new lines of research in AATD to improve the diagnosis and evaluation of the response to therapy and to develop genetic and regenerative therapies and other treatments.
Topics: Asthma; Bronchiectasis; Genetic Predisposition to Disease; Humans; Lung; Phenotype; Prognosis; Pulmonary Disease, Chronic Obstructive; Pulmonary Emphysema; Risk Assessment; Risk Factors; alpha 1-Antitrypsin Deficiency
PubMed: 32051168
DOI: 10.1183/16000617.0073-2019 -
Frontiers in Immunology 2020Bronchiectasis, the presence of bronchial wall thickening with airway dilatation, is a particularly challenging complication of primary antibody deficiencies. While... (Review)
Review
Bronchiectasis, the presence of bronchial wall thickening with airway dilatation, is a particularly challenging complication of primary antibody deficiencies. While susceptibility to infections may be the primary factor leading to the development of bronchiectasis in these patients, the condition may develop in the absence of known infections. Once bronchiectasis is present, the lungs are subject to a progressive cycle involving both infectious and non-infectious factors. If bronchiectasis is not identified or not managed appropriately, the cycle proceeds unchecked and yields advanced and permanent lung damage. Severe symptoms may limit exercise tolerance, require frequent hospitalizations, profoundly impair quality of life (QOL), and lead to early death. This review article focuses on the appropriate identification and management of bronchiectasis in patients with primary antibody deficiencies. The underlying immune deficiency and the bronchiectasis need to be treated from combined immunology and pulmonary perspectives, reflected in this review by experts from both fields. An aggressive multidisciplinary approach may reduce exacerbations and slow the progression of permanent lung damage.
Topics: Bronchiectasis; Humans; Primary Immunodeficiency Diseases
PubMed: 32296433
DOI: 10.3389/fimmu.2020.00522 -
American Journal of Respiratory and... Dec 2023
Topics: Humans; Cohort Studies; Bronchiectasis; Precision Medicine
PubMed: 37917354
DOI: 10.1164/rccm.202310-1827ED -
Respiratory Research Dec 2022Bronchiectasis and bronchiolitis are differential diagnoses of asthma; moreover, they are factors associated with worse asthma control.
RATIONALE
Bronchiectasis and bronchiolitis are differential diagnoses of asthma; moreover, they are factors associated with worse asthma control.
OBJECTIVE
We determined clinical courses of bronchiectasis/bronchiolitis-complicated asthma by inflammatory subtypes as well as factors affecting them.
METHODS
We conducted a survey of refractory asthma with non-cystic fibrosis bronchiectasis/bronchiolitis in Japan. Cases were classified into three groups, based on the latest fractional exhaled NO (FeNO) level (32 ppb for the threshold) and blood eosinophil counts (320/µL for the threshold): high (type 2-high) or low (type 2-low) FeNO and eosinophil and high FeNO or eosinophil (type 2-intermediate). Clinical courses in groups and factors affecting them were analysed.
RESULTS
In total, 216 cases from 81 facilities were reported, and 142 were stratified: 34, 40 and 68 into the type 2-high, -intermediate and -low groups, respectively. The frequency of bronchopneumonia and exacerbations requiring antibiotics and gram-negative bacteria detection rates were highest in the type 2-low group. Eighty-seven cases had paired latest and oldest available data of FeNO and eosinophil counts; they were analysed for inflammatory transition patterns. Among former type 2-high and -intermediate groups, 32% had recently transitioned to the -low group, to which relatively low FeNO in the past and oral corticosteroid use contributed. Lastly, in cases treated with moderate to high doses of inhaled corticosteroids, the frequencies of exacerbations requiring antibiotics were found to be higher in cases with more severe airway lesions and lower FeNO.
CONCLUSIONS
Bronchiectasis/bronchiolitis-complicated refractory asthma is heterogeneous. In patients with sputum symptoms and low FeNO, airway colonisation of pathogenic bacteria and infectious episodes are common; thus, corticosteroids should be carefully used.
Topics: Humans; Nitric Oxide; Asthma; Eosinophils; Bronchiectasis; Adrenal Cortex Hormones; Exhalation
PubMed: 36539765
DOI: 10.1186/s12931-022-02289-y -
Laeknabladid Jul 2020Bronchiectasis is a disease that is characterized by permanent bronchial dilation. This can be localized or diffuse in the lungs. The disease can occur at any age and... (Review)
Review
Bronchiectasis is a disease that is characterized by permanent bronchial dilation. This can be localized or diffuse in the lungs. The disease can occur at any age and causes cough, sputum production and repeated infections. It is more common in women and incidence increases with age. Bronchiectasis is characterized by repeated episodes of worsening symptoms that are usually caused by respiratory infections. The cause of bronchiectasis can be unknown but it can be caused by respiratory diseases and diseases outside the chest. Examples of such diseases are asthma, chronic obstructive pulmonary disease, rheumatoid arthritis in addition to immune deficiency. Disease profile is therefore different for each patient. Bronchiectasis is diagnosed with computerized tomography of the chest in addition to clinical symptoms. Workup to diagnose other diseases that could be causing it is therefore important. For that detailed history, physical examination and additional investigations are appropriate. Patients with bronchiectasis have decreased health related quality of life and increased mortality. Treatment focuses on treatment of underlying diseases, airway clearance and treatment of infections. Pulmonary rehabilititation is also important. Regular follow-up is important. This is a review on bronchiectasis that is intended for a spectrum of physicians, because bronchiectasis can be seen in primary care, hospitals and out of hospital.
Topics: Bronchiectasis; Bronchoscopy; Cost of Illness; Humans; Predictive Value of Tests; Prognosis; Quality of Life; Risk Factors; Tomography, X-Ray Computed
PubMed: 32608358
DOI: 10.17992/lbl.2020.0708.592