TABLE 1.
Aetiology | Diagnosis | Treatment | Comments |
---|---|---|---|
NTM pulmonary disease [17, 18, 133, 134] | Sputum culture for mycobacteria Microbiological test results compatible with NTM pulmonary disease: 1) the same NTM species is isolated in ≥2 sputum cultures, 2) isolated in ≥1 bronchial wash or lavage or 3) biopsy with mycobacterial histopathological features plus positive culture for NTM (or ≥1 sputum or bronchial washings that are culture positive for NTM) |
Combination of antibiotics for 12 months after sputum culture conversion Decided based on clinical symptoms, progression of radiological signs and knowledge of the infecting NTM species |
ERS 2017 and BTS 2019 guidelines recommend mycobacterial sputum cultures in patients with bronchiectasis ATS/ERS/ESCMID/IDSA 2020 clinical practice guidelines for the treatment of NTM pulmonary disease |
ABPA [17, 18, 135] | Total serum IgE test Aspergillus-specific IgG test Aspergillus-specific IgE test (or skin prick tests for Aspergillus) |
Systemic corticosteroids Antifungal agents |
ERS 2017 and BTS 2019 guidelines recommend ABPA testing in all patients with bronchiectasis |
Immunodeficiency [17, 18] | Serum IgA, IgM and IgG Serum IgG subclass Peripheral blood lymphocyte subpopulations (including T-, B- and NK-cells) Pneumococcal IgG to vaccine response |
Immunoglobulin replacement | ERS 2017 and BTS 2019 guidelines recommend serum IgA, IgM and IgG testing in all patients with bronchiectasis BTS 2019 guideline recommends pneumococcal IgG to vaccine response |
A1AT deficiency [17, 18, 136] | Serum A1AT A1AT genetic testing |
Intravenous augmentation of A1AT in countries where this is available | BTS 2019 guideline recommends A1AT deficiency testing in patients with coexisting basal panacinar emphysema ERS 2017 guideline states the presence of basal emphysema or early-onset airflow obstruction could suggest the need to exclude A1AT deficiency Portuguese 2016 guideline recommends A1AT deficiency testing in all patients with bronchiectasis (estimated prevalence is 1:2191 in Portugal) |
NTM: non-tuberculous mycobacterial; ERS: European Respiratory Society; BTS: British Thoracic Society; ATS: American Thoracic Society; ESCMID: European Society of Clinical Microbiology and Infectious Diseases; IDSA: Infectious Diseases Society of America; ABPA: allergic bronchopulmonary aspergillosis; NK: natural killer; A1AT: α1-antitrypsin.