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Pulmonary Granulomatosis With Polyangiitis
Aletta Ann Frazier, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Wegener granulomatosis: Term no longer in use
            • Synonyms

              • Granulomatosis and polyangiitis (GPA)
            • Definitions

              • Multisystem necrotizing granulomatous vasculitis of small to medium-sized blood vessels (arteries, capillaries, veins)
              • Idiopathic ANCA-associated vasculitis, likely autoimmune
              • Upper respiratory tract, pulmonary, and renal involvement

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Diagnosis

                                      • Treatment

                                        • Natural History & Prognosis

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Almaani S et al: ANCA-associated vasculitis: an update. J Clin Med. 10(7), 2021
                                              2. Selvaraj V et al: COVID-19-induced granulomatosis with polyangiitis. BMJ Case Rep. 14(3), 2021
                                              3. Sun X et al: Clinical features and long-term outcomes of interstitial lung disease with anti-neutrophil cytoplasmic antibody. BMC Pulm Med. 21(1):88, 2021
                                              4. Zhou P et al: Impact of interstitial lung disease on mortality in ANCA-associated vasculitis: A systematic literature review and meta-analysis. Chron Respir Dis. 18:1479973121994562, 2021
                                              5. Lawton A et al: Pulmonary manifestations of systemic vasculitis in childhood. Breathe (Sheff). 16(4):200211, 2020
                                              6. Broncano J et al: CT and MR imaging of cardiothoracic vasculitis. Radiographics. 38(4):997-1021, 2018
                                              7. Feragalli B et al: The lung in systemic vasculitis: radiological patterns and differential diagnosis. Br J Radiol. 89(1061):20150992, 2016
                                              8. Hasleton P et al: Pulmonary vasculitis and pulmonary hemorrhage syndromes. In Spencer's Pathology of the Lung. 6th ed. Cambridge University Press. 713-29, 2013
                                              9. Martinez F et al: Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation. Radiographics. 32(1):51-69, 2012
                                              10. CastaƱer E et al: When to suspect pulmonary vasculitis: radiologic and clinical clues. Radiographics. 30(1):33-53, 2010
                                              11. Ananthakrishnan L et al: Wegener's granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol. 192(3):676-82, 2009
                                              12. Lohrmann C et al: Pulmonary manifestations of wegener granulomatosis: CT findings in 57 patients and a review of the literature. Eur J Radiol. 53(3):471-7, 2005
                                              13. Sheehan RE et al: Computed tomography features of the thoracic manifestations of Wegener granulomatosis. J Thorac Imaging. 18(1):34-41, 2003
                                              14. Frazier AA et al: Pulmonary angiitis and granulomatosis: radiologic-pathologic correlation. Radiographics. 18(3):687-710; quiz 727, 1998
                                              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

                                                          • Wegener granulomatosis: Term no longer in use
                                                        • Synonyms

                                                          • Granulomatosis and polyangiitis (GPA)
                                                        • Definitions

                                                          • Multisystem necrotizing granulomatous vasculitis of small to medium-sized blood vessels (arteries, capillaries, veins)
                                                          • Idiopathic ANCA-associated vasculitis, likely autoimmune
                                                          • Upper respiratory tract, pulmonary, and renal involvement

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • Nuclear Medicine Findings

                                                                • Imaging Recommendations

                                                                  DIFFERENTIAL DIAGNOSIS

                                                                    PATHOLOGY

                                                                    • General Features

                                                                      • Staging, Grading, & Classification

                                                                        • Gross Pathologic & Surgical Features

                                                                          • Microscopic Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Diagnosis

                                                                                  • Treatment

                                                                                    • Natural History & Prognosis

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Almaani S et al: ANCA-associated vasculitis: an update. J Clin Med. 10(7), 2021
                                                                                          2. Selvaraj V et al: COVID-19-induced granulomatosis with polyangiitis. BMJ Case Rep. 14(3), 2021
                                                                                          3. Sun X et al: Clinical features and long-term outcomes of interstitial lung disease with anti-neutrophil cytoplasmic antibody. BMC Pulm Med. 21(1):88, 2021
                                                                                          4. Zhou P et al: Impact of interstitial lung disease on mortality in ANCA-associated vasculitis: A systematic literature review and meta-analysis. Chron Respir Dis. 18:1479973121994562, 2021
                                                                                          5. Lawton A et al: Pulmonary manifestations of systemic vasculitis in childhood. Breathe (Sheff). 16(4):200211, 2020
                                                                                          6. Broncano J et al: CT and MR imaging of cardiothoracic vasculitis. Radiographics. 38(4):997-1021, 2018
                                                                                          7. Feragalli B et al: The lung in systemic vasculitis: radiological patterns and differential diagnosis. Br J Radiol. 89(1061):20150992, 2016
                                                                                          8. Hasleton P et al: Pulmonary vasculitis and pulmonary hemorrhage syndromes. In Spencer's Pathology of the Lung. 6th ed. Cambridge University Press. 713-29, 2013
                                                                                          9. Martinez F et al: Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation. Radiographics. 32(1):51-69, 2012
                                                                                          10. CastaƱer E et al: When to suspect pulmonary vasculitis: radiologic and clinical clues. Radiographics. 30(1):33-53, 2010
                                                                                          11. Ananthakrishnan L et al: Wegener's granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol. 192(3):676-82, 2009
                                                                                          12. Lohrmann C et al: Pulmonary manifestations of wegener granulomatosis: CT findings in 57 patients and a review of the literature. Eur J Radiol. 53(3):471-7, 2005
                                                                                          13. Sheehan RE et al: Computed tomography features of the thoracic manifestations of Wegener granulomatosis. J Thorac Imaging. 18(1):34-41, 2003
                                                                                          14. Frazier AA et al: Pulmonary angiitis and granulomatosis: radiologic-pathologic correlation. Radiographics. 18(3):687-710; quiz 727, 1998