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Bronchiolitis Obliterans Syndrome
Carlos S. Restrepo, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Lung transplantation
                  • Chronic lung allograft dysfunction (CLAD)
                  • Bronchiolitis obliterans syndrome (BOS)
                  • Restrictive allograft syndrome (RAS)
                  • Neutrophilic reversible allograft dysfunction (NRAD)
                  • Fibrous BOS (fBOS)
                • Hematopoietic stem cell transplantation (HSCT): BOS, graft-vs.-host disease (GVHD)
                • Diffuse alveolar damage (DAD)
              • Definitions

                • BOS: Broadly used and well-known term; often incorrectly used to signify chronic rejection
                  • Evolving evidence shows that BOS is a form of CLAD
                • CLAD: Broad term describing spectrum of heterogeneous chronic lung abnormalities in lung transplant recipients
                  • Phenotypes
                    • RAS: Restrictive pulmonary function
                      • Late new-onset DAD (> 3 months post transplantation) suggests near-future development of RAS
                    • BOS: Includes chronic rejection but alloimmune independent factors (gastroesophageal reflux, aspiration, and infection) contribute to pathogenesis
                      • NRAD: Allograft dysfunction characterized by reversible airway neutrophilia responsive to azithromycin
                      • fBOS: BOS subtype not responsive to azithromycin
                • HSCT: In this setting, BOS refers to new-onset obstructive pulmonary dysfunction
                  • Histologically identical to BOS after lung transplantation

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Royer PJ et al: Chronic lung allograft dysfunction: A systematic review of mechanisms. Transplantation. 100(9):1803-14, 2016
                                        2. Jagasia MH et al: National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: I. The 2014 Diagnosis and Staging Working Group Report. Biol Blood Marrow Transplant. ePub, 2014
                                        3. Meyer KC et al: An international ISHLT/ATS/ERS clinical practice guideline: diagnosis and management of bronchiolitis obliterans syndrome. Eur Respir J. 44(6):1479-503, 2014
                                        4. Bergeron A et al: Bronchiolitis obliterans syndrome after allogeneic hematopoietic SCT: phenotypes and prognosis. Bone Marrow Transplant. 48(6):819-24, 2013
                                        5. Sato M: Chronic lung allograft dysfunction after lung transplantation: the moving target. Gen Thorac Cardiovasc Surg. 61(2):67-78, 2013
                                        6. Verleden SE et al: Neutrophilic reversible allograft dysfunction (NRAD) and restrictive allograft syndrome (RAS). Semin Respir Crit Care Med. 34(3):352-60, 2013
                                        7. de Jong PA et al: Thin-section computed tomography findings before and after azithromycin treatment of neutrophilic reversible lung allograft dysfunction. Eur Radiol. 21(12):2466-74, 2011
                                        8. Todd JL et al: Bronchiolitis obliterans syndrome: the final frontier for lung transplantation. Chest. 140(2):502-8, 2011
                                        9. Gunn ML et al: High-resolution CT findings of bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation. J Thorac Imaging. 23(4):244-50, 2008
                                        10. Konen E et al: Bronchiolitis obliterans syndrome in lung-transplant recipients: can thin-section CT findings predict disease before its clinical appearance? Radiology. 231(2):467-73, 2004
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Lung transplantation
                                                        • Chronic lung allograft dysfunction (CLAD)
                                                        • Bronchiolitis obliterans syndrome (BOS)
                                                        • Restrictive allograft syndrome (RAS)
                                                        • Neutrophilic reversible allograft dysfunction (NRAD)
                                                        • Fibrous BOS (fBOS)
                                                      • Hematopoietic stem cell transplantation (HSCT): BOS, graft-vs.-host disease (GVHD)
                                                      • Diffuse alveolar damage (DAD)
                                                    • Definitions

                                                      • BOS: Broadly used and well-known term; often incorrectly used to signify chronic rejection
                                                        • Evolving evidence shows that BOS is a form of CLAD
                                                      • CLAD: Broad term describing spectrum of heterogeneous chronic lung abnormalities in lung transplant recipients
                                                        • Phenotypes
                                                          • RAS: Restrictive pulmonary function
                                                            • Late new-onset DAD (> 3 months post transplantation) suggests near-future development of RAS
                                                          • BOS: Includes chronic rejection but alloimmune independent factors (gastroesophageal reflux, aspiration, and infection) contribute to pathogenesis
                                                            • NRAD: Allograft dysfunction characterized by reversible airway neutrophilia responsive to azithromycin
                                                            • fBOS: BOS subtype not responsive to azithromycin
                                                      • HSCT: In this setting, BOS refers to new-onset obstructive pulmonary dysfunction
                                                        • Histologically identical to BOS after lung transplantation

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • CT Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Royer PJ et al: Chronic lung allograft dysfunction: A systematic review of mechanisms. Transplantation. 100(9):1803-14, 2016
                                                                              2. Jagasia MH et al: National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: I. The 2014 Diagnosis and Staging Working Group Report. Biol Blood Marrow Transplant. ePub, 2014
                                                                              3. Meyer KC et al: An international ISHLT/ATS/ERS clinical practice guideline: diagnosis and management of bronchiolitis obliterans syndrome. Eur Respir J. 44(6):1479-503, 2014
                                                                              4. Bergeron A et al: Bronchiolitis obliterans syndrome after allogeneic hematopoietic SCT: phenotypes and prognosis. Bone Marrow Transplant. 48(6):819-24, 2013
                                                                              5. Sato M: Chronic lung allograft dysfunction after lung transplantation: the moving target. Gen Thorac Cardiovasc Surg. 61(2):67-78, 2013
                                                                              6. Verleden SE et al: Neutrophilic reversible allograft dysfunction (NRAD) and restrictive allograft syndrome (RAS). Semin Respir Crit Care Med. 34(3):352-60, 2013
                                                                              7. de Jong PA et al: Thin-section computed tomography findings before and after azithromycin treatment of neutrophilic reversible lung allograft dysfunction. Eur Radiol. 21(12):2466-74, 2011
                                                                              8. Todd JL et al: Bronchiolitis obliterans syndrome: the final frontier for lung transplantation. Chest. 140(2):502-8, 2011
                                                                              9. Gunn ML et al: High-resolution CT findings of bronchiolitis obliterans syndrome after hematopoietic stem cell transplantation. J Thorac Imaging. 23(4):244-50, 2008
                                                                              10. Konen E et al: Bronchiolitis obliterans syndrome in lung-transplant recipients: can thin-section CT findings predict disease before its clinical appearance? Radiology. 231(2):467-73, 2004