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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
- Venous disease (in 50-80%); more common than arterial (5-20%)
- Idiopathic systemic necrotizing vasculitis of large, medium, and small vessels
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
- Chung SW et al: Surgical experience of Behcet's disease involving the peripheral artery. Ann Vasc Surg. 69:246-53, 2020
- Ödev K et al: Thoracic complications in Behçet's disease: imaging findings. Can Respir J. 2020:4649081, 2020
- 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
- Emmi G et al: Vascular Behçet's syndrome: an update. Intern Emerg Med. 14(5):645-52, 2019
- Hatemi G et al: One year in review 2019: Behçet's syndrome. Clin Exp Rheumatol. 37 Suppl 121(6):3-17, 2019
- 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
- Adams TN et al: Pulmonary manifestations of large, medium, and variable vessel vasculitis. Respir Med. 145:182-91, 2018
- 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
- 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
- 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
- Ceylan N et al: Pulmonary and vascular manifestations of Behcet disease: imaging findings. AJR Am J Roentgenol. 194(2):W158-64, 2010
- Chae EJ et al: Radiologic and clinical findings of Behçet disease: comprehensive review of multisystemic involvement. Radiographics. 28(5):e31, 2008
- Kim HK et al: Behçet disease complicated by diffuse alveolar damage. J Thorac Imaging. 20(1):55-7, 2005
- Uzun O et al: Pulmonary vasculitis in behcet disease: a cumulative analysis. Chest. 127(6):2243-53, 2005
- Hiller N et al: Thoracic manifestations of Behçet disease at CT. Radiographics. 24(3):801-8, 2004
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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
- Venous disease (in 50-80%); more common than arterial (5-20%)
- Idiopathic systemic necrotizing vasculitis of large, medium, and small vessels
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
- Chung SW et al: Surgical experience of Behcet's disease involving the peripheral artery. Ann Vasc Surg. 69:246-53, 2020
- Ödev K et al: Thoracic complications in Behçet's disease: imaging findings. Can Respir J. 2020:4649081, 2020
- 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
- Emmi G et al: Vascular Behçet's syndrome: an update. Intern Emerg Med. 14(5):645-52, 2019
- Hatemi G et al: One year in review 2019: Behçet's syndrome. Clin Exp Rheumatol. 37 Suppl 121(6):3-17, 2019
- 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
- Adams TN et al: Pulmonary manifestations of large, medium, and variable vessel vasculitis. Respir Med. 145:182-91, 2018
- 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
- 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
- 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
- Ceylan N et al: Pulmonary and vascular manifestations of Behcet disease: imaging findings. AJR Am J Roentgenol. 194(2):W158-64, 2010
- Chae EJ et al: Radiologic and clinical findings of Behçet disease: comprehensive review of multisystemic involvement. Radiographics. 28(5):e31, 2008
- Kim HK et al: Behçet disease complicated by diffuse alveolar damage. J Thorac Imaging. 20(1):55-7, 2005
- Uzun O et al: Pulmonary vasculitis in behcet disease: a cumulative analysis. Chest. 127(6):2243-53, 2005
- Hiller N et al: Thoracic manifestations of Behçet disease at CT. Radiographics. 24(3):801-8, 2004
