Patients often present with recurrent pulmonary infections, including a chronic daily productive cough with mucopurulent sputum production.
Diagnostic testing for a potential aetiology of unexplained bronchiectasis should be performed.
A sputum sample should be obtained when the patient is in a stable state and during acute exacerbations. Systemic antibiotics directed towards prior culture results should be administered.
Daily airway clearance is essential for treatment success.
Maintenance aerosolised antibiotics may be used for treatment of severe bronchiectasis or recurrent Pseudomonas aeruginosa infections.
Surgical therapy, including lung transplantation, should be considered for patients who continue to deteriorate despite optimal medical management.
Bronchiectasis is the permanent dilation of bronchi due to the destruction of the elastic and muscular components of the bronchial wall. It is often caused as a consequence of recurrent and/or severe infections secondary to an underlying disorder. The majority of patients will present with a chronic cough and sputum production.
History and exam
Key diagnostic factors
- presence of risk factors
- sputum production
- crackles, high-pitched inspiratory squeaks and rhonchi
Other diagnostic factors
- weight loss
- pleuritic chest pain
- cystic fibrosis
- host immunodeficiency
- previous infections
- congenital disorders of the bronchial airways
- primary ciliary dyskinesia
- alpha-1 antitrypsin deficiency
- connective tissue disease
- inflammatory bowel disease
- aspiration or inhalation injury
- focal bronchial obstruction
- tall, thin, white females, aged 60 or over
1st investigations to order
- high-resolution chest CT
- sputum culture and sensitivity
- serum alpha-1 antitrypsin phenotype and level
- serum immunoglobulins
- sweat chloride test
- rheumatoid factor
- specific IgE or skin prick test to Aspergillus fumigatus
- serum HIV antibody
- nasal nitric oxide (NNO)
- pulmonary function tests
Investigations to consider
- bronchial biopsy and electron microscopy of cilia
- cystic fibrosis transmembrane regulator (CFTR) protein gene mutation testing
- swallow study
- pH monitoring of oesophagus
- 6-minute walk test
acute exacerbation: mild to moderate underlying disease
acute exacerbation: severe underlying disease or not responding/resistant to oral antibiotics
3 or more exacerbations per year despite maintenance therapy
Anne E. O'Donnell, MD
Professor of Medicine
Chief, Division of Pulmonary, Critical Care and Sleep Medicine
The Nehemiah and Naomi Cohen Chair in Pulmonary Disease Research
Georgetown University Medical Center
AEOD has received scientific consulting fees from the following companies: Bayer, Xellia, Horizon, Grifols, Insmed, and Electromed. AEOD is the Principal Investigator for trials sponsored by the following companies (research funding provided directly to Georgetown University): Bayer, Insmed, Aradigm, Parion, Zambon. AEOD has been a faculty member at the annual meetings of CHEST (American College of Chest Physicians), American Thoracic Society, and European Respiratory Society. AEOD is the author of a reference cited in this topic.
Dr Anne E. O'Donnell would like to gratefully acknowledge Dr Pamela J. McShane and Dr Sangeeta M. Bhorade, previous contributors to this topic.
PJM and SMB declare that they have no competing interests.
Philip W. Ind, BA (Cantab), MB BChir, MA (Cantab), FRCP
Honorary Senior Lecturer
Imperial College Healthcare Trust
PWI declares that he has no competing interests.
Ware Kuschner, MD
Associate Professor of Medicine
US Department of Veterans Affairs
Palo Alto Health Care System
WK declares that he has no competing interests.
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