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Bronchiolitis obliterans syndrome

Background

Overview

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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Guidelines

Key sources

The following summarized guidelines for the management of bronchiolitis obliterans syndrome are prepared by our editorial team based on guidelines from the European Respiratory Society (ERS/EBMT 2024), the British Thoracic Society (BTS 2020), and the European Respiratory Society (ERS/ATS/ISHLT 2014). ...
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Medical management

High-dose corticosteroids: as per ATS/ERS/ISHLT 2014 guidelines, do not use long-term, high-dose corticosteroids in lung transplant recipients with a decline in FEV1 consistent with the onset of BOS.
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  • Tacrolimus

  • Azithromycin

Surgical interventions

Fundoplication: as per ATS/ERS/ISHLT 2014 guidelines, consider referring lung transplant recipients with a decline in FEV1 consistent with the onset of BOS and having confirmed gastroesophageal reflux to an experienced surgeon to evaluate for potential fundoplication of the GEJ.
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  • Re-transplantation

Preventative measures

Primary prevention: as per BTS 2020 guidelines, consider initiating low-dose, long-term azithromycin (250 mg thrice weekly) to prevent BOS after lung transplantation.
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