Etiology of Non–Cystic Fibrosis Bronchiectasis in Adults and Its Correlation to Disease Severity

Abstract

Rationale: Testing for underlying etiology is a key part of bronchiectasis management, but it is unclear whether the same extent of testing is required across the spectrum of disease severity.Objectives: The aim of the present study was to identify the etiology of bronchiectasis across European cohorts and according to different levels of disease severity. Methods:We conducted an analysis of seven databases of adult outpatients with bronchiectasis prospectively enrolled at the bronchiectasis clinics of university teaching hospitals in Monza, Italy; Dundee and Newcastle, United Kingdom; Leuven, Belgium; Barcelona, Spain; Athens, Greece; and Galway, Ireland. All the patients at every site underwent the same comprehensive diagnostic workup as suggested by the British Thoracic Society.Measurements and Main Results: Among the 1,258 patients enrolled, an etiology of bronchiectasis was determined in 60%, including postinfective (20%), chronic obstructive pulmonary disease related (15%), connective tissue disease related (10%), immunodeficiency related (5.8%), and asthma related (3.3%). An etiology leading to a change in patient's management was identified in 13% of the cases. No significant differences in the etiology of bronchiectasis were present across different levels of disease severity, with the exception of a higher prevalence of chronic obstructive pulmonary disease-related bronchiectasis (P , 0.001) and a lower prevalence of idiopathic bronchiectasis (P = 0.029) in patients with severe disease.Conclusions: Physicians should not be guided by disease severity in suspecting specific etiologies in patients with bronchiectasis, although idiopathic bronchiectasis appears to be less common in patients with the most severe disease.

Quality standards for the management of bronchiectasis in Italy: a national audit

“…Bronchiectasis was diagnosed by chest radiography in 5% and by bronchography in 1% of the rest of the population, while 1% had a clinical diagnosis alone. The second statement recommends that patients should be investigated for allergic bronchopulmonary aspergillosis (ABPA), common variable immunodeficiency (CVID) and cystic fibrosis (CF), the last of these if indicated, as these are specific and treatable causes of bronchiectasis [11]. In the SIP audit, only 435 (32%) patients were tested for at least one of the above: 17% of the patients had been investigated for ABPA, 22% for CVID and 5.5% for CF.…”

Large and Small Airway Disease Related to Inflammatory Bowel Disease

“…4 In comparison, this was only slightly more frequent than bronchiectasis related to ciliary dysfunction and approximately a third of the cases due to immunodeficiency. Interestingly, while bronchiectasis is usually associated with active IBD, it has also been reported to develop after colectomy.…”

“…Pathologists must therefore remember that in the normal small bronchioles, the epithelial layer is generally in close proximity to the underlying muscular layer, and that any increase in the subepithelial fibrous tissue compartment is abnormal. Since the degree of airway resistance is related to 1/radius, 4 even a tiny degree of narrowing will have a significant effect on pulmonary function. In comparison, when the airway is completely obliterated, an elastic stain may be necessary to show that an area of fibrosis adjacent to a pulmonary artery is actually an airway remnant ( Figure 4, A and B).…”

Suspecting non-cystic fibrosis bronchiectasis: What the busy primary care clinician needs to know

“…Despite identification of these key features on CT and extensive testing, no underlying aetiology is found in a large proportion of patients with bronchiectasis 46. The prevalence of idiopathic bronchiectasis varies amongst cohorts (32%‐66%) likely owing to geographic variations, diagnostic algorithms and other determinants 30, 47, 48, 49, 50, 51…”