Semin Respir Crit Care Med 2013; 34(03): 336-351
DOI: 10.1055/s-0033-1348467
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Bronchiolitis Obliterans Syndrome: The Achilles' Heel of Lung Transplantation

S. Samuel Weigt
1   Division of Pulmonary, Critical Care Medicine, Allergy, and Clinical Immunology, Department of Internal Medicine, The David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California
,
Ariss DerHovanessian
1   Division of Pulmonary, Critical Care Medicine, Allergy, and Clinical Immunology, Department of Internal Medicine, The David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California
,
W. Dean Wallace
2   Department of Pathology and Laboratory Medicine, The David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California
,
Joseph P. Lynch III
1   Division of Pulmonary, Critical Care Medicine, Allergy, and Clinical Immunology, Department of Internal Medicine, The David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California
,
John A. Belperio
1   Division of Pulmonary, Critical Care Medicine, Allergy, and Clinical Immunology, Department of Internal Medicine, The David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California
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Publikationsdatum:
02. Juli 2013 (online)

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Abstract

Lung transplantation is a therapeutic option for patients with end-stage pulmonary disorders. Unfortunately, chronic lung allograft dysfunction (CLAD), most commonly manifest as bronchiolitis obliterans syndrome (BOS), continues to be highly prevalent and is the major limitation to long-term survival. The pathogenesis of BOS is complex and involves alloimmune and nonalloimmune pathways. Clinically, BOS manifests as airway obstruction and dyspnea that are classically progressive and ultimately fatal; however, the course is highly variable, and distinguishable phenotypes may exist. There are few controlled studies assessing treatment efficacy, but only a minority of patients respond to current treatment modalities. Ultimately, preventive strategies may prove more effective at prolonging survival after lung transplantation, but their remains considerable debate and little data regarding the best strategies to prevent BOS. A better understanding of the risk factors and their relationship to the pathological mechanisms of chronic lung allograft rejection should lead to better pharmacological targets to prevent or treat this syndrome.