Fatal airway disease in an adult with chronic graft-versus-host disease.

Abstract

Chronic graft-versus-host disease (GVHD) is a syndrome of disordered immunity in which a variety of opportunistic respiratory infections have been documented. Restrictive pulmonary disease without advanced fibrotic changes has been also known to occur; obstructive lower airways disease related to chronic GVHD has apparently not been referred to. We report a patient with chronic GVHD and irreversible airways obstruction who died in respiratory failure caused by generalised bronchiolar damage progressing over a few months. Lung function studies showed severe airflow limitation and a leftward shift in the pressure-volume curve indicating a loss of elastic recoil pressure. Postmortem pulmonary lesions were consistent with a necrotising obliterative bron chiolitis. Our purpose is to describe this unusual complication not previously reported in chronic GVHD and discuss the pulmonary mechanics.