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Behcet Syndrome
Aletta Ann Frazier, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Synonyms

              • Behçet disease (BD), Silk Road disease
            • Definitions

              • Idiopathic systemic necrotizing vasculitis of large, medium, and small vessels
                • Venous disease (in 50-80%); more common than arterial (5-20%)
                  • Wide geographic variations in frequency
                • Oral and genital mucosal ulcers, uveitis
                • Arteritis: Pulmonary arteries, aorta ± branches, coronaries (rare)
                  • Iliac/femoral arteries may also be affected
                • Thoracic manifestations: Vascular aneurysms ± in situ thrombosis
                  • Lung hemorrhage, infarction
                  • Mediastinal lymphadenopathy, mediastinitis, fibrosis
                  • Cardiac thrombus, inflammation
                  • Pleural nodules, effusions

            IMAGING

            • Radiographic Findings

              • 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

                                      Selected References

                                      1. Chung SW et al: Surgical experience of Behcet's disease involving the peripheral artery. Ann Vasc Surg. 69:246-53, 2020
                                      2. Ödev K et al: Thoracic complications in Behçet's disease: imaging findings. Can Respir J. 2020:4649081, 2020
                                      3. Becatti M et al: Behçet's syndrome as a tool to dissect the mechanisms of thrombo-inflammation: clinical and pathogenetic aspects. Clin Exp Immunol. 195(3):322-33, 2019
                                      4. Emmi G et al: Vascular Behçet's syndrome: an update. Intern Emerg Med. 14(5):645-52, 2019
                                      5. Hatemi G et al: One year in review 2019: Behçet's syndrome. Clin Exp Rheumatol. 37 Suppl 121(6):3-17, 2019
                                      6. Vural U et al: Coronary Involvement in Behçet's disease: what are its risks and Prognosis? (Rare Cases and Literature Review). Braz J Cardiovasc Surg. 34(6):749-58, 2019
                                      7. Adams TN et al: Pulmonary manifestations of large, medium, and variable vessel vasculitis. Respir Med. 145:182-91, 2018
                                      8. Argyropoulou OD et al: Accelerated atheromatosis and arteriosclerosis in primary systemic vasculitides: current evidence and future perspectives. Curr Opin Rheumatol. 30(1):36-43, 2018
                                      9. Protogerou AD et al: Non-invasive vascular biomarkers in patients with Behçet's disease: review of the data and future perspectives. Clin Exp Rheumatol. 35 Suppl 108(6):100-07, 2017
                                      10. Hasleton P et al: Pulmonary vasculitis and pulmonary hemorrhage syndromes. In Spencer's Pathology of the Lung. 6th ed. Cambridge University Press. 711-66, 2013
                                      11. Ceylan N et al: Pulmonary and vascular manifestations of Behcet disease: imaging findings. AJR Am J Roentgenol. 194(2):W158-64, 2010
                                      12. Chae EJ et al: Radiologic and clinical findings of Behçet disease: comprehensive review of multisystemic involvement. Radiographics. 28(5):e31, 2008
                                      13. Kim HK et al: Behçet disease complicated by diffuse alveolar damage. J Thorac Imaging. 20(1):55-7, 2005
                                      14. Uzun O et al: Pulmonary vasculitis in behcet disease: a cumulative analysis. Chest. 127(6):2243-53, 2005
                                      15. Hiller N et al: Thoracic manifestations of Behçet disease at CT. Radiographics. 24(3):801-8, 2004
                                      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

                                                • Synonyms

                                                  • Behçet disease (BD), Silk Road disease
                                                • Definitions

                                                  • Idiopathic systemic necrotizing vasculitis of large, medium, and small vessels
                                                    • Venous disease (in 50-80%); more common than arterial (5-20%)
                                                      • Wide geographic variations in frequency
                                                    • Oral and genital mucosal ulcers, uveitis
                                                    • Arteritis: Pulmonary arteries, aorta ± branches, coronaries (rare)
                                                      • Iliac/femoral arteries may also be affected
                                                    • Thoracic manifestations: Vascular aneurysms ± in situ thrombosis
                                                      • Lung hemorrhage, infarction
                                                      • Mediastinal lymphadenopathy, mediastinitis, fibrosis
                                                      • Cardiac thrombus, inflammation
                                                      • Pleural nodules, effusions

                                                IMAGING

                                                • Radiographic Findings

                                                  • 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

                                                                          Selected References

                                                                          1. Chung SW et al: Surgical experience of Behcet's disease involving the peripheral artery. Ann Vasc Surg. 69:246-53, 2020
                                                                          2. Ödev K et al: Thoracic complications in Behçet's disease: imaging findings. Can Respir J. 2020:4649081, 2020
                                                                          3. Becatti M et al: Behçet's syndrome as a tool to dissect the mechanisms of thrombo-inflammation: clinical and pathogenetic aspects. Clin Exp Immunol. 195(3):322-33, 2019
                                                                          4. Emmi G et al: Vascular Behçet's syndrome: an update. Intern Emerg Med. 14(5):645-52, 2019
                                                                          5. Hatemi G et al: One year in review 2019: Behçet's syndrome. Clin Exp Rheumatol. 37 Suppl 121(6):3-17, 2019
                                                                          6. Vural U et al: Coronary Involvement in Behçet's disease: what are its risks and Prognosis? (Rare Cases and Literature Review). Braz J Cardiovasc Surg. 34(6):749-58, 2019
                                                                          7. Adams TN et al: Pulmonary manifestations of large, medium, and variable vessel vasculitis. Respir Med. 145:182-91, 2018
                                                                          8. Argyropoulou OD et al: Accelerated atheromatosis and arteriosclerosis in primary systemic vasculitides: current evidence and future perspectives. Curr Opin Rheumatol. 30(1):36-43, 2018
                                                                          9. Protogerou AD et al: Non-invasive vascular biomarkers in patients with Behçet's disease: review of the data and future perspectives. Clin Exp Rheumatol. 35 Suppl 108(6):100-07, 2017
                                                                          10. Hasleton P et al: Pulmonary vasculitis and pulmonary hemorrhage syndromes. In Spencer's Pathology of the Lung. 6th ed. Cambridge University Press. 711-66, 2013
                                                                          11. Ceylan N et al: Pulmonary and vascular manifestations of Behcet disease: imaging findings. AJR Am J Roentgenol. 194(2):W158-64, 2010
                                                                          12. Chae EJ et al: Radiologic and clinical findings of Behçet disease: comprehensive review of multisystemic involvement. Radiographics. 28(5):e31, 2008
                                                                          13. Kim HK et al: Behçet disease complicated by diffuse alveolar damage. J Thorac Imaging. 20(1):55-7, 2005
                                                                          14. Uzun O et al: Pulmonary vasculitis in behcet disease: a cumulative analysis. Chest. 127(6):2243-53, 2005
                                                                          15. Hiller N et al: Thoracic manifestations of Behçet disease at CT. Radiographics. 24(3):801-8, 2004