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Kirsten M. Williams, MD; Jason W. Chien, MD, MS; Mark T. Gladwin, MD; Steven Z. Pavletic, MD

JAMA. 2009;302(3):306-314.

With improvements in supportive care, both long-term survival following allogeneic hematopoietic stem cell transplantations (HSCTs) and the indications for this procedure have increased. As a result, the number of patients living with long-term toxic effects due to HSCT has increased. A once rare condition of the donor immune cells attacking healthy host tissues, termed chronic graft-vs-host disease, has become a more common phenomenon. When chronic graft-vs-host disease affects the lung tissue, bronchiolitis obliterans syndrome ensues. Recent data suggest that bronchiolitis obliterans syndrome may affect up to 6% of HSCT recipients and dramatically alters survival, with overall survival of only 13% at 5 years. These statistics have not improved since the first presentation of this disease over 20 years ago. Challenges to the progress of medical management of bronchiolitis obliterans syndrome include difficulties and delays in diagnosis and a paucity of data on pathogenesis to direct new therapies. This article critically evaluates the current diagnostic criteria for bronchiolitis obliterans syndrome and reviews the epidemiology, pathogenesis, and available treatments. Improvements in survival will likely require early disease recognition, allowing for therapeutic modulation of disease prior to the development of irreversible airway obliteration.

Author Affiliations: Experimental Transplantation and Immunology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland (Drs Williams and Pavletic); Clinical Research Division, Fred Hutchinson Cancer Research Center and Pulmonary and Critical Care, University of Washington, Seattle (Dr Chien); and Division of Pulmonary, Allergy, and Critical Care Medicine, Hemostasis and Vascular Biology Research Institute, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania (Dr Gladwin).

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