Bronchiectasis: how to be an orphan with many parents?

Goeminne, Pieter; Soyza, Anthony De

doi:10.1183/13993003.01567-2015

Cited by 46 publications

(26 citation statements)

References 27 publications

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“…based on prior exacerbation history), patient phenotyping or endotyping is beyond the scope of such a document. Likewise, the guideline cannot address treatable comorbidities, environmental, lifestyle and psychological factors, which can also impact on patient outcomes [2,[29][30][31][32]. Providing holistic care for patients with bronchiectasis requires taking these factors into consideration as well.…”

Section: Chronic Pseudomonas Infectionmentioning

confidence: 99%

Treatable traits in bronchiectasis

et al. 2018

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]. The diagnostic label "bronchiectasis" describes the existence of localised and permanent airway dilation, but when used to describe a disease it includes a heterogeneous group of disorders that differ significantly in terms of aetiological, clinical, radiological, functional and microbial features [1]. Using cluster analysis, some previous studies have attempted to identify distinct "clinical phenotypes" in patients with bronchiectasis [2-4] (defined as "a single or combination of disease attributes that describe differences between patients that are related to clinically meaningful outcomes" [5]). By and large, however, these studies did not consider the underlying biology or response to therapy (i.e. the underlying endotype(s) [6]). Potential endotypes in bronchiectasis include immunodeficiency, ciliary dyskinesia, infection (with typical bacteria and non-tuberculous mycobacteria (NTM)), hypersensitivity to fungi and autoimmunity. Importantly, all of them can potentially become therapeutic targets [7]. The main treatment goals in bronchiectasis are to reduce symptoms, prevent exacerbations and lung function decline and, ultimately, improve survival. Unfortunately, most currently available therapeutic options have shown only a modest impact on disease outcomes in randomised clinical trials [8, 9]. For instance, inhaled antibiotic treatments have, so far, produced only modest benefits in terms of reduced exacerbations or improved quality of life, suggesting that endotypes other than airway "infection" are likely to play a relevant pathogenic role [10-12]. Likewise, co-existing airways diseases are common in patients with bronchiectasis and, in fact, up to 50% of these patients have a diagnosis of co-existing asthma or chronic obstructive pulmonary disease (COPD) [13, 14]. All in all, the complexity of bronchiectasis is poorly adapted to the "one size fits all" approach of current clinical guidelines. The coexistence in the same patient of different endotypes, clinical phenotypes and exposures requires a more precise approach to both assessment and therapy. The concept of "treatable traits" was originally proposed in 2016 by AGUSTI et al. [15] in the European Respiratory Journal as a way toward precision medicine of airway diseases. These authors argued that the current airways disease diagnostic labels are imprecise, often overlap and lead to empirical therapy. They proposed that a biomarker-directed approach, based on the recognition of clinical phenotype and endotypes, can help to personalised treatment options which, hopefully, may result in better clinical outcomes. In the original manuscript on treatable traits, bronchiectasis was considered as a potential trait in patients with a diagnostic label of asthma or COPD. Here, we leverage from the treatable traits concept [15] and suggest that patients with bronchiectasis, with or without co-existing COPD and asthma, represent a heterogeneous group of patients who also present multiple treatable traits, many of which go

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Section: Chronic Pseudomonas Infectionmentioning

confidence: 99%

Treatable traits in bronchiectasis

et al. 2018

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“…Bronchiectasis is a lung condition associated with chronic cough and sputum production that is rapidly increasing in prevalence in Europe [2]. It is a neglected disease, but recent initiatives including the European Bronchiectasis Registry and research network (EMBARC) and the European Union supported European Reference Network for Rare Pulmonary Diseases (ERN-LUNG) are beginning to raise the diseases profile and stimulate new research [3,4]. Patient involvement has been, and remains, central to these projects including the recently published European bronchiectasis guidelines which were developed with patients as members of the panel [5].…”

Section: Introductionmentioning

confidence: 99%

Cross-infection risk in patients with bronchiectasis: a position statement from the European Bronchiectasis Network (EMBARC), EMBARC/ELF patient advisory group and European Reference Network (ERN-Lung) Bronchiectasis Network

et al. 2018

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“…This presumption, however, has been challenged recently and some experts believe rightly that bronchiectasis is not an orphan disease. [ 1 ] Recent data from Western countries estimate a much higher prevalence and incidence of bronchiectasis than the threshold of 5/10,000 population for the definition of an orphan disease as defined by the European Union. [ 1 ] A study from the United Kingdom showed an increase in the incidence from 21.2/100,000 person-years in 2004 to 35.2/100,000 person-years in 2013 in women and from 18.2/100,000 person-years in 2004 to 26.9/100,000 person-years in 2013 in men.…”

Section: Introductionmentioning

confidence: 99%

“…[ 1 ] Recent data from Western countries estimate a much higher prevalence and incidence of bronchiectasis than the threshold of 5/10,000 population for the definition of an orphan disease as defined by the European Union. [ 1 ] A study from the United Kingdom showed an increase in the incidence from 21.2/100,000 person-years in 2004 to 35.2/100,000 person-years in 2013 in women and from 18.2/100,000 person-years in 2004 to 26.9/100,000 person-years in 2013 in men. [ 2 ] A recent population-based estimation of bronchiectasis prevalence in Germany estimated an overall prevalence rate of 67/100,000 in 2013 and a higher prevalence in elderly men (75–84 years) of 228/100,000.…”

Section: Introductionmentioning

confidence: 99%

The Saudi Thoracic Society guidelines for diagnosis and management of noncystic fibrosis bronchiectasis

et al. 2017

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This is the first guideline developed by the Saudi Thoracic Society for the diagnosis and management of noncystic fibrosis bronchiectasis. Local experts including pulmonologists, infectious disease specialists, thoracic surgeons, respiratory therapists, and others from adult and pediatric departments provided the best practice evidence recommendations based on the available international and local literature. The main objective of this guideline is to utilize the current published evidence to develop recommendations about management of bronchiectasis suitable to our local health-care system and available resources. We aim to provide clinicians with tools to standardize the diagnosis and management of bronchiectasis. This guideline targets primary care physicians, family medicine practitioners, practicing internists and respiratory physicians, and all other health-care providers involved in the care of the patients with bronchiectasis.

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Bronchiectasis: how to be an orphan with many parents?

Cited by 46 publications

References 27 publications

Treatable traits in bronchiectasis

Treatable traits in bronchiectasis

Cross-infection risk in patients with bronchiectasis: a position statement from the European Bronchiectasis Network (EMBARC), EMBARC/ELF patient advisory group and European Reference Network (ERN-Lung) Bronchiectasis Network

The Saudi Thoracic Society guidelines for diagnosis and management of noncystic fibrosis bronchiectasis

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