Management strategies include:
Medications see Medications for Bronchiectasis
Airway clearance program
See Physiotherapy for more information
A prescribed exercise program is important to enhance airways clearance and for general well-being. It should include moderate to high intensity aerobic exercises, strength training and mobility exercises.
See Physiotherapy Exercise for more information
Multidisciplinary team involvement as required
Respiratory physician / GP
The treatment of co-morbidities
Bronchiectasis can complicate most recognised immunodeficiency syndromes. The presumed mechanism is failure of immune clearance resulting in recurrent respiratory tract infections, inflammation and structural damage.
Both primary and secondary immunodeficiency syndromes can result in bronchiectasis. The most common primary immunodeficiency syndrome is Common Variable Immunodeficiency (CVID) where impaired B cell differentiation results in defective production of protective antibodies (immunoglobulins). Examples of secondary immunodeficiency states include medical immunosuppression (for example post organ or bone marrow transplant) and haematological malignancy.
Immunodeficiency presents as recurrent infections, often of the sinuses and respiratory tract. Therefore, the possibility of underlying immunodeficiency should be considered in all patients with bronchiectasis.
Recent Australian and European guidelines recommend testing serum immunoglobulins (IgG, IgA, IgM) as part of the “minimum bundle” of tests for all patients with bronchiectasis, to detect hypogammaglobulinaemia (low immunoglobulin levels). Some patients may require additional tests of immune function such as measurement of vaccine response, which is best done in collaboration with an immunologist. CVID would be diagnosed in the presence of low IgG in combination with low IgA and/or IgM, impaired vaccine response, and the absence of another cause.
Bronchiectasis with hypogammaglobulinaemia presents a rare opportunity to treat the underlying cause of disease. Immunoglobulin replacement may be warranted, and is best managed in collaboration between a respiratory physician and immunologist. Replacement immunoglobulin is prepared from pooled human plasma and contains the typical IgG antibodies found in the normal population. It is most commonly administered via the intravenous route (IVIg), but can be administered subcutaneously (SCIg) at certain centres. An individualised weight-based dose is generally given every 3 to 4 weeks with the goals of attaining a trough IgG level in the middle of the normal range and reducing the frequency of infection.
Replacement immunoglobulin is available from the Australian Red Cross Blood Service, however supply is tightly regulated. See National Blood Authority Australia for Australian supply information.
The Criteria for Clinical Use of Immunoglobulin in Australia have been developed by the National Blood Authority using expert Specialist Working Groups of clinicians to identify the medical conditions and circumstances for which immunoglobulin product is supplied and funded by governments under the national blood arrangements. For use of IVIg outside these indications, approved providers such as hospitals may be able to directly order and fund this product.
An Action Plan
Symptoms and management strategies when well
Change in symptoms and consequent alteration of management
Strategy when unwell
Surgery – may be considered:
In patients with localised disease that have not responded to treatment
To remove an obstructing tumour or foreign body
Where bronchial artery embolization is indicated with massive haemoptysis
In severe cases, lung transplantation may be considered – Lung transplantation for non-cystic fibrosis bronchiectasis