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Vasculitis
Siva P. Raman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Diagnostic Checklist

        TERMINOLOGY

        • Definitions

          • General term describing diverse group of diseases characterized by inflammation/necrosis of blood vessels
            • Classified by size of blood vessel involved into small vessel, medium vessel, and large vessel vasculitis
            • Large vessel: Takayasu arteritis
            • Medium vessel: Polyarteritis nodosa
            • Small vessel: Henoch-Schönlein purpura, lupus vasculitis, Behçet disease, Wegener granulomatosis
          • Takayasu arteritis: Chronic granulomatous inflammatory vasculitis affecting aorta and its main branches
          • Polyarteritis nodosa: Fibrinoid necrotizing vasculitis involving small and medium vessels with formation of multiple branch-point aneurysms
          • Henoch-Schönlein purpura: Hypersensitivity-related acute vasculitis affecting small vessels with deposition of IgA complexes in skin, joints, kidneys, and GI tract
          • Wegener granulomatosis: Granulomatous vasculitis of respiratory tract and kidneys
          • Lupus vasculitis: Complex autoimmune disease with associated necrotizing vasculitis affecting small vessels
          • Behçet disease: Necrotizing vasculitis of small vessels affecting multiple organs

        IMAGING

        • Imaging Recommendations

          • General Features

            • Radiographic Findings

              • Ultrasonographic Findings

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • Staging, Grading, & Classification

                    • Microscopic Features

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Image Interpretation Pearls

                                Selected References

                                1. Collins JD: MR Imaging of the mesenteric vasculature. Radiol Clin North Am. 58(4):797-813, 2020
                                2. Hansen NJ: Computed tomographic angiography of the abdominal aorta. Radiol Clin North Am. 54(1):35-54, 2016
                                3. Kalfa M et al: Segmental arterial mediolysis mimics systemic vasculitis. Eur J Rheumatol. 3(3):136-8, 2016
                                4. Muto G et al: Large vessel vasculitis in elderly patients: early diagnosis and steroid-response evaluation with FDG-PET/CT and contrast-enhanced CT. Rheumatol Int. 34(11):1545-54, 2014
                                5. Prieto-González S et al: Positron emission tomography assessment of large vessel inflammation in patients with newly diagnosed, biopsy-proven giant cell arteritis: a prospective, case-control study. Ann Rheum Dis. 73(7):1388-92, 2014
                                6. Schmidt WA: Imaging in vasculitis. Best Pract Res Clin Rheumatol. 27(1):107-18, 2013
                                7. Chu LC et al: Vascular complications of Ehlers-Danlos syndrome: CT findings. AJR Am J Roentgenol. 198(2):482-7, 2012
                                8. Hokama A et al: Endoscopic and radiographic features of gastrointestinal involvement in vasculitis. World J Gastrointest Endosc. 4(3):50-6, 2012
                                9. Pipitone N et al: Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update. Rheumatology (Oxford). 47(4):403-8, 2008
                                10. Haubitz M: ANCA-associated vasculitis: diagnosis, clinical characteristics and treatment. Vasa. 36(2):81-9, 2007
                                11. Otsuka H et al: FDG-PET/CT for diagnosis and follow-up of vasculitis. J Med Invest. 54(3-4):345-9, 2007
                                12. Roemer FW et al: Unusual manifestation of small-vessel vasculitis. AJR Am J Roentgenol. 186(2):586-7, 2006
                                13. Jayne D: Current attitudes to the therapy of vasculitis. Kidney Blood Press Res. 26(4):231-9, 2003
                                14. Geboes K et al: Vasculitis and the gastrointestinal tract. Acta Gastroenterol Belg. 65(4):204-12, 2002
                                15. Chung SY et al: Radiologic findings of Behçet syndrome involving the gastrointestinal tract. Radiographics. 21(4):911-24; discussion 924-6, 2001
                                16. Wiest R et al: Clinical image: occult large vessel vasculitis diagnosed by PET imaging. Rheumatol Int. 20(6):250, 2001
                                17. Ha HK et al: Radiologic features of vasculitis involving the gastrointestinal tract. Radiographics. 20(3):779-94, 2000
                                18. Kawashima A et al: CT evaluation of renovascular disease. Radiographics. 20(5):1321-40, 2000
                                19. Rha SE et al: CT and MR imaging findings of bowel ischemia from various primary causes. Radiographics. 20(1):29-42, 2000
                                20. Hunder G: Vasculitis: diagnosis and therapy. Am J Med. 100(2A):37S-45S, 1996
                                21. Jennette JC et al: The pathology of vasculitis involving the kidney. Am J Kidney Dis. 24(1):130-41, 1994
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Diagnostic Checklist

                                      TERMINOLOGY

                                      • Definitions

                                        • General term describing diverse group of diseases characterized by inflammation/necrosis of blood vessels
                                          • Classified by size of blood vessel involved into small vessel, medium vessel, and large vessel vasculitis
                                          • Large vessel: Takayasu arteritis
                                          • Medium vessel: Polyarteritis nodosa
                                          • Small vessel: Henoch-Schönlein purpura, lupus vasculitis, Behçet disease, Wegener granulomatosis
                                        • Takayasu arteritis: Chronic granulomatous inflammatory vasculitis affecting aorta and its main branches
                                        • Polyarteritis nodosa: Fibrinoid necrotizing vasculitis involving small and medium vessels with formation of multiple branch-point aneurysms
                                        • Henoch-Schönlein purpura: Hypersensitivity-related acute vasculitis affecting small vessels with deposition of IgA complexes in skin, joints, kidneys, and GI tract
                                        • Wegener granulomatosis: Granulomatous vasculitis of respiratory tract and kidneys
                                        • Lupus vasculitis: Complex autoimmune disease with associated necrotizing vasculitis affecting small vessels
                                        • Behçet disease: Necrotizing vasculitis of small vessels affecting multiple organs

                                      IMAGING

                                      • Imaging Recommendations

                                        • General Features

                                          • Radiographic Findings

                                            • Ultrasonographic Findings

                                              DIFFERENTIAL DIAGNOSIS

                                                PATHOLOGY

                                                • Staging, Grading, & Classification

                                                  • Microscopic Features

                                                    CLINICAL ISSUES

                                                    • Presentation

                                                      • Demographics

                                                        • Natural History & Prognosis

                                                          • Treatment

                                                            DIAGNOSTIC CHECKLIST

                                                            • Image Interpretation Pearls

                                                              Selected References

                                                              1. Collins JD: MR Imaging of the mesenteric vasculature. Radiol Clin North Am. 58(4):797-813, 2020
                                                              2. Hansen NJ: Computed tomographic angiography of the abdominal aorta. Radiol Clin North Am. 54(1):35-54, 2016
                                                              3. Kalfa M et al: Segmental arterial mediolysis mimics systemic vasculitis. Eur J Rheumatol. 3(3):136-8, 2016
                                                              4. Muto G et al: Large vessel vasculitis in elderly patients: early diagnosis and steroid-response evaluation with FDG-PET/CT and contrast-enhanced CT. Rheumatol Int. 34(11):1545-54, 2014
                                                              5. Prieto-González S et al: Positron emission tomography assessment of large vessel inflammation in patients with newly diagnosed, biopsy-proven giant cell arteritis: a prospective, case-control study. Ann Rheum Dis. 73(7):1388-92, 2014
                                                              6. Schmidt WA: Imaging in vasculitis. Best Pract Res Clin Rheumatol. 27(1):107-18, 2013
                                                              7. Chu LC et al: Vascular complications of Ehlers-Danlos syndrome: CT findings. AJR Am J Roentgenol. 198(2):482-7, 2012
                                                              8. Hokama A et al: Endoscopic and radiographic features of gastrointestinal involvement in vasculitis. World J Gastrointest Endosc. 4(3):50-6, 2012
                                                              9. Pipitone N et al: Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update. Rheumatology (Oxford). 47(4):403-8, 2008
                                                              10. Haubitz M: ANCA-associated vasculitis: diagnosis, clinical characteristics and treatment. Vasa. 36(2):81-9, 2007
                                                              11. Otsuka H et al: FDG-PET/CT for diagnosis and follow-up of vasculitis. J Med Invest. 54(3-4):345-9, 2007
                                                              12. Roemer FW et al: Unusual manifestation of small-vessel vasculitis. AJR Am J Roentgenol. 186(2):586-7, 2006
                                                              13. Jayne D: Current attitudes to the therapy of vasculitis. Kidney Blood Press Res. 26(4):231-9, 2003
                                                              14. Geboes K et al: Vasculitis and the gastrointestinal tract. Acta Gastroenterol Belg. 65(4):204-12, 2002
                                                              15. Chung SY et al: Radiologic findings of Behçet syndrome involving the gastrointestinal tract. Radiographics. 21(4):911-24; discussion 924-6, 2001
                                                              16. Wiest R et al: Clinical image: occult large vessel vasculitis diagnosed by PET imaging. Rheumatol Int. 20(6):250, 2001
                                                              17. Ha HK et al: Radiologic features of vasculitis involving the gastrointestinal tract. Radiographics. 20(3):779-94, 2000
                                                              18. Kawashima A et al: CT evaluation of renovascular disease. Radiographics. 20(5):1321-40, 2000
                                                              19. Rha SE et al: CT and MR imaging findings of bowel ischemia from various primary causes. Radiographics. 20(1):29-42, 2000
                                                              20. Hunder G: Vasculitis: diagnosis and therapy. Am J Med. 100(2A):37S-45S, 1996
                                                              21. Jennette JC et al: The pathology of vasculitis involving the kidney. Am J Kidney Dis. 24(1):130-41, 1994