Aetiology of bronchiectasis in adults: A systematic literature review

Abstract

While identifying the underlying aetiology is a key part of bronchiectasis management, the prevalence and impact of identifying the aetiologies on clinical management remain unclear. We aimed to determine the etiological spectrum of bronchiectasis, and how often etiological assessment could lead to the changes in patients' management. A comprehensive search was conducted using MEDLINE (via PubMed) and EMBASE for observational studies published before October 2015 reporting aetiologies in adults with bronchiectasis. Of the 8216 citations identified, 56 studies including 8608 adults with bronchiectasis were relevant for this systematic review. The crude prevalence for the identified aetiologies ranged from 18% to 95%, which possibly resulted from the differences in the geographic regions and diagnostic workup. Post-infective (29.9%), immunodeficiency (5%), chronic obstructive pulmonary disease (3.9%), connective tissue disease (3.8%), ciliary dysfunction (2.5%), allergic bronchopulmonary aspergillosis (2.6%) were the most common aetiologies. In 1577 patients (18.3%), identifying the aetiologies led to changes in patient's management. Aetiologies varied considerably among different geographic regions (P < 0.001). Intensive investigations of these aetiologies might help change patient's management and therefore should be incorporated into routine clinical practice.

British Thoracic Society Guideline for bronchiectasis in adults

“…67 In adults, almost all cases of immune deficiency are likely to involve defects in antibody production. Recognition of significant antibody syndromes such as common variable immune deficiency (CVID) is important as immunoglobulin replacement therapy can reduce exacerbation frequency and improve clinical outcomes.…”

“…23 62–66 Early studies were conducted in specialist centres but more recently studies of adult patients have been conducted in general chest clinics; online appendix 5 summarises those most relevant to the UK population and includes a systematic review of 56 studies covering 8608 patients from across the world. 67 …”

Lady Windermere syndrome

“…22,23 Non-cystic fibrosis bronchiectasis in adults often has no obvious cause or is a sequalae of infections but can occur in patients with immunodeficiency, chronic obstructive pulmonary disease, connective tissue disease, allergic bronchopulmonary aspergillosis (ABPA), and ciliary dyskinesia. 21,24 Lady Windermere syndrome (LWS), a unique subtype of M. avium pulmonary disease, is associated with bronchiectasis, but it is unknown whether bronchiectasis precedes M. avium infection or is a result of infection. 25 Airway involvement in bronchiectasis is usually diffuse, but some diseases are associated with more focal distributions of bronchiectasis in the lung.…”

Chronic Respiratory Abnormalities in the Multi-Morbid Frail Elderly

“…The prevalence and impact of airflow limitation and/or COPD has been extensively studied and reviewed in asthma and obstructive sleep disorders 14,57,58 , bronchiectasis 59,60 , lung cancer 14,61 , interstitial lung diseases and pulmonary hypertension 62,63 , tuberculosis 64 , and human immunodeficiency virus (HIV) 65 , and will not be addressed in this review.…”