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Bronchiolitis obliterans syndrome

Definition
BOS is a form of chronic lung allograft dysfunction characterized by progressive airflow obstruction in the absence of acute rejection, infection, or other coexistent conditions.
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Pathophysiology
BOS is caused by immune-mediated injury (alloimmune T-cell reactivity, humoral immunity, autoimmunity, pulmonary innate immunity) and environmental insults.
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Disease course
BOS manifests as progressive dyspnea and cough, with imaging features of constrictive bronchiolitis, airway distortion, and subepithelial fibrosis.
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Prognosis and risk of recurrence
Severe BOS is associated with a mortality of 66.6%.
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Key sources
The following summarized guidelines for the management of bronchiolitis obliterans syndrome are prepared by our editorial team based on guidelines from the British Thoracic Society (BTS 2020) and the European Respiratory Society (ERS/ATS/ISHLT 2014).
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Guidelines

1.Medical management

High-dose corticosteroids: consider avoiding long-term, high-dose corticosteroids for lung transplant recipients who develop a decline in FEV1 consistent with the onset of BOS.
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  • Tacrolimus

  • Azithromycin

2.Surgical interventions

Fundoplication: consider referral to an experienced surgeon to evaluate for potential fundoplication of the gastro-oesophageal junction in lung transplant recipients who develop a decline in FEV1 consistent with the onset of BOS and have confirmed gastro-oesophageal reflux.
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  • Re-transplantation

3.Preventative measures

Primary prevention: as per ERS 2020 guidelines, consider offering low-dose, long-term azithromycin (250 mg thrice weekly) for the prevention of BOS after lung transplantation.
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