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Granulomatosis With Polyangiitis (Wegener)
Philip R. Chapman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Granulomatosis with polyangiitis (GPA)
            • Synonyms

              • Wegener granulomatosis
                • Historical eponym Wegener granulomatosis has been discarded by scientific community in favor of GPA
            • Definitions

              • Idiopathic, autoimmune necrotizing granulomatous vasculitis
              • Preferentially affects upper and lower respiratory tracts, kidneys, skin, and joints

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Nwawka OK et al: Granulomatous disease in the head and neck: developing a differential diagnosis. Radiographics. 34(5):1240-56, 2014
                                        2. Cannady SB et al: Sinonasal Wegener granulomatosis: a single-institution experience with 120 cases. Laryngoscope. 119(4):757-61, 2009
                                        3. Fuchs HA et al: Granulomatous disorders of the nose and paranasal sinuses. Curr Opin Otolaryngol Head Neck Surg. 17(1):23-7, 2009
                                        4. Grindler D et al: Computed tomography findings in sinonasal Wegener's granulomatosis. Am J Rhinol Allergy. 23(5):497-501, 2009
                                        5. Erickson VR et al: Wegener's granulomatosis: current trends in diagnosis and management. Curr Opin Otolaryngol Head Neck Surg. 15(3):170-6, 2007
                                        6. Srouji IA et al: Patterns of presentation and diagnosis of patients with Wegener's granulomatosis: ENT aspects. J Laryngol Otol. 121(7):653-8, 2007
                                        7. Lohrmann C et al: Sinonasal computed tomography in patients with Wegener's granulomatosis. J Comput Assist Tomogr. 30(1):122-5, 2006
                                        8. Benoudiba F et al: Sinonasal Wegener's granulomatosis: CT characteristics. Neuroradiology. 45(2): 95-9, 2003
                                        9. Gubbels SP et al: Head and neck manifestations of Wegener's granulomatosis. Otolaryngol Clin North Am. 36(4): 685-705, 2003
                                        10. Langford CA: Wegener's granulomatosis: current and upcoming therapies. Arthritis Res Ther. 5(4): 180-91, 2003
                                        11. Takwoingi YM et al: Wegener's granulomatosis: an analysis of 33 patients seen over a 10-year period. Clin Otolaryngol. 28(3): 187-94, 2003
                                        12. Abdou NI et al: Wegener's granulomatosis: survey of 701 patients in North America. Changes in outcome in the 1990s. J Rheumatol. 29(2): 309-16, 2002
                                        13. Fechner FP et al: Wegener's granulomatosis of the orbit: a clinicopathological study of 15 patients. Laryngoscope. 112(11): 1945-50, 2002
                                        14. Marsot-Dupuch K et al: Wegener granulomatosis involving the pterygopalatine fossa: an unusual case of trigeminal neuropathy. AJNR Am J Neuroradiol. 23(2): 312-5, 2002
                                        15. Metaxaris G et al: Otolaryngologic manifestations of small vessel vasculitis. Auris Nasus Larynx. 29(4): 353-6, 2002
                                        16. Nagai H et al: Clinical review of Wegener's granulomatosis. Acta Otolaryngol Suppl. (547): 50-3, 2002
                                        17. Diamantopoulos II et al: The investigation of nasal septal perforations and ulcers. J Laryngol Otol. 115(7): 541-4, 2001
                                        18. Stein J et al: Nasal cavity squamous cell carcinoma in Wegener's granulomatosis. Arch Otolaryngol Head Neck Surg. 127(6): 709-13, 2001
                                        19. Borges A et al: Midline destructive lesions of the sinonasal tract: simplified terminology based on histopathologic criteria. AJNR Am J Neuroradiol. 21(2): 331-6, 2000
                                        20. Noorduyn LA et al: Sinonasal non-Hodgkin's lymphomas and Wegener's granulomatosis: a clinicopathological study. Virchows Arch A Pathol Anat Histopathol. 418(3): 235-40, 1991
                                        21. Drake-Lee AB et al: A review of the role of radiology in non-healing granulomas of the nose and nasal sinuses. Rhinology. 27(4):231-6, 1989
                                        22. Paling MR et al: Paranasal sinus obliteration in Wegener granulomatosis. Radiology. 144(3):539-43, 1982
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Granulomatosis with polyangiitis (GPA)
                                                  • Synonyms

                                                    • Wegener granulomatosis
                                                      • Historical eponym Wegener granulomatosis has been discarded by scientific community in favor of GPA
                                                  • Definitions

                                                    • Idiopathic, autoimmune necrotizing granulomatous vasculitis
                                                    • Preferentially affects upper and lower respiratory tracts, kidneys, skin, and joints

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Nwawka OK et al: Granulomatous disease in the head and neck: developing a differential diagnosis. Radiographics. 34(5):1240-56, 2014
                                                                              2. Cannady SB et al: Sinonasal Wegener granulomatosis: a single-institution experience with 120 cases. Laryngoscope. 119(4):757-61, 2009
                                                                              3. Fuchs HA et al: Granulomatous disorders of the nose and paranasal sinuses. Curr Opin Otolaryngol Head Neck Surg. 17(1):23-7, 2009
                                                                              4. Grindler D et al: Computed tomography findings in sinonasal Wegener's granulomatosis. Am J Rhinol Allergy. 23(5):497-501, 2009
                                                                              5. Erickson VR et al: Wegener's granulomatosis: current trends in diagnosis and management. Curr Opin Otolaryngol Head Neck Surg. 15(3):170-6, 2007
                                                                              6. Srouji IA et al: Patterns of presentation and diagnosis of patients with Wegener's granulomatosis: ENT aspects. J Laryngol Otol. 121(7):653-8, 2007
                                                                              7. Lohrmann C et al: Sinonasal computed tomography in patients with Wegener's granulomatosis. J Comput Assist Tomogr. 30(1):122-5, 2006
                                                                              8. Benoudiba F et al: Sinonasal Wegener's granulomatosis: CT characteristics. Neuroradiology. 45(2): 95-9, 2003
                                                                              9. Gubbels SP et al: Head and neck manifestations of Wegener's granulomatosis. Otolaryngol Clin North Am. 36(4): 685-705, 2003
                                                                              10. Langford CA: Wegener's granulomatosis: current and upcoming therapies. Arthritis Res Ther. 5(4): 180-91, 2003
                                                                              11. Takwoingi YM et al: Wegener's granulomatosis: an analysis of 33 patients seen over a 10-year period. Clin Otolaryngol. 28(3): 187-94, 2003
                                                                              12. Abdou NI et al: Wegener's granulomatosis: survey of 701 patients in North America. Changes in outcome in the 1990s. J Rheumatol. 29(2): 309-16, 2002
                                                                              13. Fechner FP et al: Wegener's granulomatosis of the orbit: a clinicopathological study of 15 patients. Laryngoscope. 112(11): 1945-50, 2002
                                                                              14. Marsot-Dupuch K et al: Wegener granulomatosis involving the pterygopalatine fossa: an unusual case of trigeminal neuropathy. AJNR Am J Neuroradiol. 23(2): 312-5, 2002
                                                                              15. Metaxaris G et al: Otolaryngologic manifestations of small vessel vasculitis. Auris Nasus Larynx. 29(4): 353-6, 2002
                                                                              16. Nagai H et al: Clinical review of Wegener's granulomatosis. Acta Otolaryngol Suppl. (547): 50-3, 2002
                                                                              17. Diamantopoulos II et al: The investigation of nasal septal perforations and ulcers. J Laryngol Otol. 115(7): 541-4, 2001
                                                                              18. Stein J et al: Nasal cavity squamous cell carcinoma in Wegener's granulomatosis. Arch Otolaryngol Head Neck Surg. 127(6): 709-13, 2001
                                                                              19. Borges A et al: Midline destructive lesions of the sinonasal tract: simplified terminology based on histopathologic criteria. AJNR Am J Neuroradiol. 21(2): 331-6, 2000
                                                                              20. Noorduyn LA et al: Sinonasal non-Hodgkin's lymphomas and Wegener's granulomatosis: a clinicopathological study. Virchows Arch A Pathol Anat Histopathol. 418(3): 235-40, 1991
                                                                              21. Drake-Lee AB et al: A review of the role of radiology in non-healing granulomas of the nose and nasal sinuses. Rhinology. 27(4):231-6, 1989
                                                                              22. Paling MR et al: Paranasal sinus obliteration in Wegener granulomatosis. Radiology. 144(3):539-43, 1982