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Vasculitis
Siva P. Raman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Diagnostic Checklist

        TERMINOLOGY

        • Definitions

          • General term describing a 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

                PATHOLOGY

                • Staging, Grading, & Classification

                  • Microscopic Features

                    CLINICAL ISSUES

                    • Presentation

                      • Demographics

                        • Natural History & Prognosis

                          • Treatment

                            DIAGNOSTIC CHECKLIST

                            • Image Interpretation Pearls

                              Selected References

                              1. 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. ePub, 2014
                              2. 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
                              3. Schmidt WA: Imaging in vasculitis. Best Pract Res Clin Rheumatol. 27(1):107-18, 2013
                              4. Hokama A et al: Endoscopic and radiographic features of gastrointestinal involvement in vasculitis. World J Gastrointest Endosc. 4(3):50-6, 2012
                              5. 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
                              6. Haubitz M: ANCA-associated vasculitis: diagnosis, clinical characteristics and treatment. Vasa. 36(2):81-9, 2007
                              7. Otsuka H et al: FDG-PET/CT for diagnosis and follow-up of vasculitis. J Med Invest. 54(3-4):345-9, 2007
                              8. Roemer FW et al: Unusual manifestation of small-vessel vasculitis. AJR Am J Roentgenol. 186(2):586-7, 2006
                              9. Jayne D: Current attitudes to the therapy of vasculitis. Kidney Blood Press Res. 26(4):231-9, 2003
                              10. Geboes K et al: Vasculitis and the gastrointestinal tract. Acta Gastroenterol Belg. 65(4):204-12, 2002
                              11. Chung SY et al: Radiologic findings of Behçet syndrome involving the gastrointestinal tract. Radiographics. 21(4):911-24; discussion 924-6, 2001
                              12. Wiest R et al: Clinical image: occult large vessel vasculitis diagnosed by PET imaging. Rheumatol Int. 20(6):250, 2001
                              13. Ha HK et al: Radiologic features of vasculitis involving the gastrointestinal tract. Radiographics. 20(3):779-94, 2000
                              14. Kawashima A et al: CT evaluation of renovascular disease. Radiographics. 20(5):1321-40, 2000
                              15. Rha SE et al: CT and MR imaging findings of bowel ischemia from various primary causes. Radiographics. 20(1):29-42, 2000
                              16. Hunder G: Vasculitis: diagnosis and therapy. Am J Med. 100(2A):37S-45S, 1996
                              17. 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 a 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

                                            PATHOLOGY

                                            • Staging, Grading, & Classification

                                              • Microscopic Features

                                                CLINICAL ISSUES

                                                • Presentation

                                                  • Demographics

                                                    • Natural History & Prognosis

                                                      • Treatment

                                                        DIAGNOSTIC CHECKLIST

                                                        • Image Interpretation Pearls

                                                          Selected References

                                                          1. 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. ePub, 2014
                                                          2. 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
                                                          3. Schmidt WA: Imaging in vasculitis. Best Pract Res Clin Rheumatol. 27(1):107-18, 2013
                                                          4. Hokama A et al: Endoscopic and radiographic features of gastrointestinal involvement in vasculitis. World J Gastrointest Endosc. 4(3):50-6, 2012
                                                          5. 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
                                                          6. Haubitz M: ANCA-associated vasculitis: diagnosis, clinical characteristics and treatment. Vasa. 36(2):81-9, 2007
                                                          7. Otsuka H et al: FDG-PET/CT for diagnosis and follow-up of vasculitis. J Med Invest. 54(3-4):345-9, 2007
                                                          8. Roemer FW et al: Unusual manifestation of small-vessel vasculitis. AJR Am J Roentgenol. 186(2):586-7, 2006
                                                          9. Jayne D: Current attitudes to the therapy of vasculitis. Kidney Blood Press Res. 26(4):231-9, 2003
                                                          10. Geboes K et al: Vasculitis and the gastrointestinal tract. Acta Gastroenterol Belg. 65(4):204-12, 2002
                                                          11. Chung SY et al: Radiologic findings of Behçet syndrome involving the gastrointestinal tract. Radiographics. 21(4):911-24; discussion 924-6, 2001
                                                          12. Wiest R et al: Clinical image: occult large vessel vasculitis diagnosed by PET imaging. Rheumatol Int. 20(6):250, 2001
                                                          13. Ha HK et al: Radiologic features of vasculitis involving the gastrointestinal tract. Radiographics. 20(3):779-94, 2000
                                                          14. Kawashima A et al: CT evaluation of renovascular disease. Radiographics. 20(5):1321-40, 2000
                                                          15. Rha SE et al: CT and MR imaging findings of bowel ischemia from various primary causes. Radiographics. 20(1):29-42, 2000
                                                          16. Hunder G: Vasculitis: diagnosis and therapy. Am J Med. 100(2A):37S-45S, 1996
                                                          17. Jennette JC et al: The pathology of vasculitis involving the kidney. Am J Kidney Dis. 24(1):130-41, 1994