Behçet disease is a multisystemic and chronic inflammatory vasculitis of unknown etiology.
The mean age at which Behçet disease occurs is 20-30 years. The disease is most prevalent in the Mediterranean region, Middle East and East Asia. The highest incidence has been reported in Turkey. Males are affected 2-5 times more often than females.
The classic clinical triad of Behçet disease consists of:
- oral ulceration
- genital ulceration
- ocular manifestations
Additional clinical manifestations include skin, joints, gastrointestinal tract, genitourinary tract, central nervous system, cardiovascular system, and lung involvement. Vascular complications develop in ~30% (20-40%) of patients with Behçet disease.
The underlying pathologic process in Behçet disease is vasculitis, and perivascular inflammatory infiltrates affecting vessels of differing sizes in various organs.
Primarily involves four main systems:
cardiovascular manifestations of Behçet disease
- affects 5-30% of cases
- thickening of the aorta and superior vena cava (vasculitis)
- inflammation of the veins leads to thrombosis while arterial involvement includes arterial narrowing and aneurysmal dilatation
- pericarditis is the most common cardiac manifestation occurring in 39% of cases 9
thoracic manifestations of Behçet disease
- affects 1-8% of cases
- pulmonary arterial aneurysm: fusiform to saccular, commonly multiple and bilateral, located in the lower lobe or main pulmonary arteries (most common pulmonary manifestation of Behçet disease)
- subpleural alveolar infiltrates, and wedge-shaped or rounded areas of increased density, which represent focal vasculitis and thrombosis resulting in infarction, hemorrhage, and focal atelectasis
- gastrointestinal manifestations of Behçet disease: affects 5-60% of cases
- CNS manifestations of Behçet disease: affects 10-50% of cases
- musculoskeletal manifestations of Behçet disease: >50% develop articular disorders or arthralgia 7
Treatment and prognosis
The disease may recur, progress, or resolve (either spontaneously, within a few weeks to several months, or with corticosteroid therapy).
History and etymology
It is named after Hulusi Behçet (1889-1948), a Turkish dermatologist.
On imaging consider:
- 1. Hiller N, Lieberman S, Chajek-Shaul T et-al. Thoracic manifestations of Behçet disease at CT. Radiographics. 2004;24 (3): 801-8. doi:10.1148/rg.243035091 - Pubmed citation
- 2. Chung SY, Ha HK, Kim JH et-al. Radiologic findings of Behçet syndrome involving the gastrointestinal tract. Radiographics. 21 (4): 911-24. Radiographics (full text) - Pubmed citation
- 3. Marchiori E, Zanetti G, Mano CM. Fulminant evolution of Behçet disease. AJR Am J Roentgenol. 2010;195 (4): W311-2. doi:10.2214/AJR.10.4602 - Pubmed citation
- 4. Chae EJ, Do KH, Seo JB et-al. Radiologic and clinical findings of Behçet disease: comprehensive review of multisystemic involvement. Radiographics. 28 (5): e31. doi:10.1148/rg.e31 - Pubmed citation
- 5. Tunaci M, Ozkorkmaz B, Tunaci A et-al. CT findings of pulmonary artery aneurysms during treatment for Behçet's disease. AJR Am J Roentgenol. 1999;172 (3): 729-33. AJR Am J Roentgenol (abstract) - Pubmed citation
- 6. Ceylan N, Bayraktaroglu S, Erturk SM et-al. Pulmonary and vascular manifestations of Behcet disease: imaging findings. AJR Am J Roentgenol. 2010;194 (2): W158-64. doi:10.2214/AJR.09.2763 - Pubmed citation
- 7. Resnick DL. Diagnosis of Bone and Joint Disorders. Saunders. (2002) ISBN:0721689213. Read it at Google Books - Find it at Amazon
- 8. Blättler W, Schwarzenbach B, Largiadèr J. Superficial vein thrombophlebitis-serious concern or much ado about little?. VASA. 2008;37 (1): 31-8. doi:10.1024/0301-1522.214.171.124 - Pubmed citation
- 9. Broncano J, Vargas D, Bhalla S, Cummings KW, Raptis CA, Luna A. CT and MR Imaging of Cardiothoracic Vasculitis. (2018) Radiographics : a review publication of the Radiological Society of North America, Inc. 38 (4): 997-1021. doi:10.1148/rg.2018170136 - Pubmed