Non-CF bronchiectasis: does knowing the aetiology lead to changes in management?

Abstract

The aim of the current study was to review the aetiology of non-cystic fibrosis (CF) bronchiectasis from two tertiary paediatric respiratory units in order to determine how often making a specific aetiological diagnosis leads to a change in management, and to assess the contribution of computed tomography (CT) in determining the underlying diagnosis.The case records of all patients who were diagnosed as having bronchiectasis by CT, currently being seen at the Royal Brompton Hospital and Great Ormond Street Hospital for Children (London, UK), were reviewed. All patients had undergone extensive investigations, and the underlying aetiology and the area of pulmonary involvement (as seen on CT) were recorded. A total of 136 patients were identified; there were 65 young males and the group median (range) age was 12.1 yrs (3.1-18.1). Immunodeficiency, aspiration and primary ciliary dyskinesia accounted for 67% of the cases. In 77 (56%) children, the identification of a cause led to a specific change in management. There was no association between aetiology and the distribution of CT abnormalities.In conclusion, immunodeficiency and other intrinsic abnormalities account for the majority of cases of non-cystic fibrosis bronchiectasis seen in the current authors' units. Computed tomography scans do not contribute towards identifying the aetiology and, most importantly, a specific aetiological diagnosis frequently leads to a change in management.

Changing epidemiology of non-cystic fibrosis bronchiectasis

“…There was no aspiration case due to neurologic reasons. Zaid et al 19 reported rate of BE secondary to aspiration as 22% and Li et al 20 as 18%. In the studies from our country, Karadağ et al 16 reported the rate as 3.6% and Doğru et al 18 as 3.4% but Karakoç et al 17 reported no case of BE secondary to aspiration.…”

Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

“…The incidence of bronchiectasis in other rare PIDs such as hyper-IgE syndrome (<2.5% in children and very rare in adults) [74,84,85], phagocyte defects (<1%-10% in children and <1% in adults) [86,87], and transporter antigen peptide deficiency (rare in children and very rare in adults) [87,88] is low. The microorganisms associated with bronchiectasis in patients with PID are shown in the Table. High-resolution computed tomography (HRCT) is considered a reliable test for assessing bronchiectasis in patients with PID [89][90][91].…”

“…More than 70% of CVID patients develop bronchiectasis and bronchial wall thickening [47,72], indicating that bronchiectasis is a well-recognized complication of CVID. The incidence of bronchiectasis in patients with XLA is almost 32%, although XLA has been associated with up to 3% of cases of childhood bronchiectasis [73][74][75] and is rare in adults. We indicated that the rate and severity of bronchiectasis were greater in CVID patients than in those with XLA, probably because of the earlier diagnosis and treatment of XLA patients [76].…”

Bronquiectasias en pediatría, aproximación diagnóstica y tratamiento

“…Su frecuencia oscila, en adultos y niños, en 0-17%. 18,[40][41][42] Dentro de las adquiridas, además de la inmunodepresión farmacológica, destaca la infección VIH (sida). Las bronquiectasias son más frecuentes en pacientes con neumonía intersticial linfoide, neumonías bacterianas o protozoarias recurrentes, o mayor grado de inmunodepresión, especialmente si los linfocitos CD 4+ son menores de 100/mcL.…”

“…43,44 De las congénitas, las humorales son las que más se asocian a bronquiectasias, destacando la agammaglobulinemia ligada al cromosoma X, la inmunodeficiencia común variable, y déficit de subclases de inmunoglobulinas. 18,[40][41][42] El déficit de IgA, déficit inmunológico más frecuente, se ha asociado a bronquiectasias, pero existe discrepancia al respecto. 45,46 Los gérmenes son clásicamente encapsulados (S. pneumoniae, H. influenzae).…”