Constrictive Bronchiolitis Obliterans: The Fibrotic Airway Disorder

Gary R. Epler

Disclosures

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

In This Article

Summary & Conclusions

CB is concentric submucosal fibrosis of the bronchiolar airway leading to narrowing and obliteration. Shortness of breath is the most common presenting symptom and early inspiratory crackles are heard by lung auscultation. Pulmonary function demonstrates irreversible airflow obstruction with decreased FEV1:FVC ratio, although a restrictive pattern may be seen. The chest radiograph shows normal lungs or hyperinflation, while the chest CT shows air trapping by the expiratory view, a mosaic pattern, bronchiolectasis and small airway wall thickening. Corticosteroid therapy is effective for the inflammatory component of the illness and for exacerbations. Immunosuppressive agents may be effective in some individuals. Antifibrotic agents may be effective in the future. Lung transplantation is an option for life-threatening, disabling disease.

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