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Wegener Granulomatosis, Airway
Jeffrey P. Kanne, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Wegener granulomatosis (WG)
              • Synonyms

                • Granulomatous polyangiitis, granulomatosis with polyangiitis (newly accepted name for WG)
              • Definitions

                • Multisystemic necrotizing granulomatous vasculitis of small to medium vessels without associated infection

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Kallenberg CG: Pathogenesis of ANCA-associated vasculitides. Ann Rheum Dis. 70 Suppl 1:i59-63, 2011
                                            2. Langford CA: Cyclophosphamide as induction therapy for Wegener's granulomatosis and microscopic polyangiitis. Clin Exp Immunol. 164 Suppl 1:31-4, 2011
                                            3. Niles J: Rituximab in induction therapy for anti-neutrophil cytoplasmic antibody (ANCA) vasculitis. Clin Exp Immunol. 164 Suppl 1:27-30, 2011
                                            4. Schilder AM: Wegener's Granulomatosis vasculitis and granuloma. Autoimmun Rev. 9(7):483-7, 2010
                                            5. Ananthakrishnan L et al: Wegener's granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol. 192(3):676-82, 2009
                                            6. Carruthers D et al: Evidence-based management of ANCA vasculitis. Best Pract Res Clin Rheumatol. 23(3):367-78, 2009
                                            7. Grenier PA et al: Nonneoplastic tracheal and bronchial stenoses. Radiol Clin North Am. 47(2):243-60, 2009
                                            8. Turnbull J et al: Adverse effects of therapy for ANCA-associated vasculitis. Best Pract Res Clin Rheumatol. 23(3):391-401, 2009
                                            9. Jagiello P et al: Complex genetics of Wegener granulomatosis. Autoimmun Rev. 4(1):42-7, 2005
                                            10. Daum TE et al: Tracheobronchial involvement in Wegener's granulomatosis. Am J Respir Crit Care Med. 151(2 Pt 1):522-6, 1995
                                            11. Hoffman GS et al: Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 116(6):488-98, 1992
                                            12. Aberle DR et al: Thoracic manifestations of Wegener granulomatosis: diagnosis and course. Radiology. 174(3 Pt 1):703-9, 1990
                                            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

                                                          • Wegener granulomatosis (WG)
                                                        • Synonyms

                                                          • Granulomatous polyangiitis, granulomatosis with polyangiitis (newly accepted name for WG)
                                                        • Definitions

                                                          • Multisystemic necrotizing granulomatous vasculitis of small to medium vessels without associated infection

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Staging, Grading, & Classification

                                                                      • Gross Pathologic & Surgical Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Kallenberg CG: Pathogenesis of ANCA-associated vasculitides. Ann Rheum Dis. 70 Suppl 1:i59-63, 2011
                                                                                      2. Langford CA: Cyclophosphamide as induction therapy for Wegener's granulomatosis and microscopic polyangiitis. Clin Exp Immunol. 164 Suppl 1:31-4, 2011
                                                                                      3. Niles J: Rituximab in induction therapy for anti-neutrophil cytoplasmic antibody (ANCA) vasculitis. Clin Exp Immunol. 164 Suppl 1:27-30, 2011
                                                                                      4. Schilder AM: Wegener's Granulomatosis vasculitis and granuloma. Autoimmun Rev. 9(7):483-7, 2010
                                                                                      5. Ananthakrishnan L et al: Wegener's granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol. 192(3):676-82, 2009
                                                                                      6. Carruthers D et al: Evidence-based management of ANCA vasculitis. Best Pract Res Clin Rheumatol. 23(3):367-78, 2009
                                                                                      7. Grenier PA et al: Nonneoplastic tracheal and bronchial stenoses. Radiol Clin North Am. 47(2):243-60, 2009
                                                                                      8. Turnbull J et al: Adverse effects of therapy for ANCA-associated vasculitis. Best Pract Res Clin Rheumatol. 23(3):391-401, 2009
                                                                                      9. Jagiello P et al: Complex genetics of Wegener granulomatosis. Autoimmun Rev. 4(1):42-7, 2005
                                                                                      10. Daum TE et al: Tracheobronchial involvement in Wegener's granulomatosis. Am J Respir Crit Care Med. 151(2 Pt 1):522-6, 1995
                                                                                      11. Hoffman GS et al: Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 116(6):488-98, 1992
                                                                                      12. Aberle DR et al: Thoracic manifestations of Wegener granulomatosis: diagnosis and course. Radiology. 174(3 Pt 1):703-9, 1990