Constrictive Bronchiolitis Obliterans: The Fibrotic Airway Disorder

Gary R. Epler

Disclosures

Expert Rev Resp Med. 2007;1(1):139-147. 

In This Article

Management & Treatment

The management of CB usually begins with an empirical course of high-dose corticosteroid therapy in the order of 1 mg/kg for.[50] This approach will treat the inflammatory component and in some situations may be curative if the process is the inflammatory intraluminal polypoid process. For the purely fibrotic lesion, the corticosteroid should be stopped shortly after stabilization as adverse reactions and possible interference with the natural healing process outweigh the benefit. However, additional empirical use of corticosteroid therapy can be beneficial for future intermittent exacerbations.

Bronchodilator inhalers and inhaled corticosteroids are beneficial for a small percentage of patients with CB and can be administered during the early stages of the disease to determine efficacy. Rapid and aggressive treatment of respiratory infections is fundamental to the management of this disorder. After stabilization, a pulmonary rehabilitation program with an ongoing home exercise program is very beneficial for this group of patients, who are able to improve their muscular conditioning as well as their sense of well being.

For development of the BOS among lung-transplant recipients, treatment consists of increasing the dose of immune suppression agents, adding new immune-suppressive or immune-modulating therapies. The regimen generally includes increasing dosage of a corticosteroid, a calcineurin inhibitor, such as cyclosporine or tacrolimus, and a purine synthesis inhibitor, such as azathioprine or mycophenolate mofetil.[35,36] For individuals with the purely fibrotic bronchiolar lesion who have not responded to corticosteroid therapy, antifibrotic agents could be utilized; however, this would be on an anecdotal basis, because controlled clinical studies have not yet established an effective agent.

A 3-month empirical course of a macrolide, such as azithromycin 250 mg three-times weekly, can be considered in certain situations, especially if there is an inflammatory component to the lesion as this agent is well tolerated by patients, has few adverse reactions at this dosage and has been shown to be beneficial in individuals with an inflammatory airway lesion.[51] This agent will probably not be effective for a purely fibrotic CB lesion and therefore can be stopped after 3 months if there has been no improvement. Finally, although this option should be avoided until it is clear the patient has no other choice, for severe and lifethreatening progressive illness, lung transplantation is an effective and successful lifesaving treatment.

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