Behçet disease
Updates to Article Attributes
Behçet disease is a multi-systemic and chronic inflammatory vasculitis of unknown aetiology.
Epidemiology
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 is has been reported in Turkey. Males are affected 2-5 times more often than females.
Clinical presentation
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 Vascular complications develop in about 20~30% (20-40% of) of patients with Behçet disease.
Pathology
The underlying pathologic process in Behçet disease is vasculitis and perivascular inflammatory infiltrates affecting vessels of differing sizes in various organs.
Associations
- HLA-B514
- factor V leiden mutation 4
- superficial thrombophlebitis8
Distribution
Primarily involves four main systems.
-
cardiovascular manifestations of Behçet disease
--
affects 5-30% of cases
. Thickening -
thickening of the aorta and
SVCsuperior vena cava (vasculitis). Inflammation -
inflammation of the veins lead to thrombosis while arterial involvement includes arterial narrowing and aneurysmal dilatation
.
-
affects 5-30% of cases
-
thoracic manifestations of Behçet disease
- affects-
affects 1-8% of cases
. Pulmonary -
pulmonary arterial aneurysm: fusiform to saccular, commonly multiple and bilateral, located in the lower lobe or main pulmonary arteries
. Subpleural - subpleural alveolar infiltrates, and wedge-shaped or rounded areas of increased density, which represent focal vasculitis and thrombosis resulting in infarction, haemorrhage, and focal atelectasis
-
affects 1-8% of cases
-
gastro-intestinalgastrointestinal manifestations of Behçetdiseasedisease-: affects 5-60% of cases -
CNS manifestations of Behçet disease
- affects: affects 10-50% of cases -
musculoskeletal manifestations of
BehBehçet disease-: >50% develop articular disorders/arthralgia/ 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 Beh Behçet (1889-1948), Turkish dermatologist.
Differential diagnosis
On imaging consider
-<p><strong>Behçet disease</strong> is a multi-systemic and chronic inflammatory <a href="/articles/vasculitis">vasculitis</a> of unknown aetiology.</p><h4>Epidemiology</h4><p>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 is has been reported in Turkey. Males are affected 2-5 times more often than females.</p><h4>Clinical presentation</h4><p>Classic clinical triad of Behçet disease consists of :</p><ol>- +<p><strong>Behçet disease</strong> is a multi-systemic and chronic inflammatory <a href="/articles/vasculitis">vasculitis</a> of unknown aetiology.</p><h4>Epidemiology</h4><p>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.</p><h4>Clinical presentation</h4><p>Classic clinical triad of Behçet disease consists of :</p><ol>
-</ol><p>Additional clinical manifestations include skin, joints, gastrointestinal tract, genitourinary tract, central nervous system, cardiovascular system, and lung involvement.</p><p>Vascular complications develop in about 20-40% of patients with Behçet disease.</p><h4>Pathology</h4><p>The underlying pathologic process in Behçet disease is vasculitis and perivascular inflammatory infiltrates affecting vessels of differing sizes in various organs.</p><h5>Associations</h5><ul>-<li>HLA-B51<sup>4</sup>- +</ol><p>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.</p><h4>Pathology</h4><p>The underlying pathologic process in Behçet disease is vasculitis and perivascular inflammatory infiltrates affecting vessels of differing sizes in various organs.</p><h5>Associations</h5><ul>
- +<li>HLA-B51 <sup>4</sup>
- +<li>
- +<a href="/articles/superficial-thrombophlebitis">superficial thrombophlebitis</a> <sup>8</sup>
- +</li>
-<a href="/articles/cardiovascular-manifestations-of-beh-et-disease">cardiovascular manifestations of Behçet disease </a>- affects 5-30% of cases. Thickening of the aorta and SVC (vasculitis). Inflammation of the veins lead to thrombosis while arterial involvement includes arterial narrowing and aneurysmal dilatation.</li>- +<a href="/articles/cardiovascular-manifestations-of-beh-et-disease">cardiovascular manifestations of Behçet disease</a><ul>
- +<li>
- +<a href="/articles/cardiovascular-manifestations-of-beh-et-disease"></a>affects 5-30% of cases</li>
- +<li>thickening of the aorta and superior vena cava (vasculitis)</li>
- +<li>inflammation of the veins lead to thrombosis while arterial involvement includes arterial narrowing and aneurysmal dilatation</li>
- +</ul>
- +</li>
- +<li>
- +<a href="/articles/behcet-disease-thoracic-manifestations">thoracic manifestations of Behçet disease</a><ul>
-<a href="/articles/thoracic-manifestations-of-behcet-disease">thoracic manifestations of Behçet disease</a> - affects 1-8% of cases. Pulmonary arterial aneurysm: fusiform to saccular, commonly multiple and bilateral, located in the lower lobe or main pulmonary arteries. Subpleural alveolar infiltrates, and wedge-shaped or rounded areas of increased density, which represent focal vasculitis and thrombosis resulting in infarction, haemorrhage, and focal atelectasis</li>- +<a href="/articles/behcet-disease-thoracic-manifestations"></a>affects 1-8% of cases</li>
- +<li>pulmonary arterial aneurysm: fusiform to saccular, commonly multiple and bilateral, located in the lower lobe or main pulmonary arteries</li>
- +<li>subpleural alveolar infiltrates, and wedge-shaped or rounded areas of increased density, which represent focal vasculitis and thrombosis resulting in infarction, haemorrhage, and focal atelectasis</li>
- +</ul>
- +</li>
-<a href="/articles/gastro-intestinal-manifestations-of-beh-et-disease">gastro-intestinal manifestations of Behçet disease</a> - affects 5-60% of cases </li>- +<a href="/articles/gastro-intestinal-manifestations-of-beh-et-disease">gastrointestinal manifestations of Behçet disease</a>: affects 5-60% of cases </li>
-<a href="/articles/cns-manifestations-of-behcet-disease-1">CNS manifestations of Behçet disease </a>- affects 10-50% of cases</li>- +<a href="/articles/behcet-disease-cns-manifestations">CNS manifestations of Behçet disease</a>: affects 10-50% of cases</li>
-<a href="/articles/musculoskeletal-manifestations-of-beh-et-disease">musculoskeletal manifestations of Behçet disease</a> - >50% develop articular disorders/arthralgia<sup>7</sup>- +<a href="/articles/musculoskeletal-manifestations-of-beh-et-disease">musculoskeletal manifestations of Behçet disease</a>: >50% develop articular disorders / arthralgia <sup>7</sup>
-</ul><h4>Treatment and prognosis</h4><p>The disease may recur, progress, or resolve (either spontaneously, within a few weeks to several months, or with corticosteroid therapy).</p><h4>History and etymology</h4><p>It is named after <strong>Hulusi Behçet</strong> (1889-1948), Turkish dermatologist .</p><h4>Differential diagnosis</h4><p>On imaging consider</p><ul><li><a href="/articles/hughes-stovin-syndrome">Hughes-Stovin syndrome</a></li></ul>- +</ul><h4>Treatment and prognosis</h4><p>The disease may recur, progress, or resolve (either spontaneously, within a few weeks to several months, or with corticosteroid therapy).</p><h4>History and etymology</h4><p>It is named after <strong>Hulusi Behçet</strong> (1889-1948), Turkish dermatologist.</p><h4>Differential diagnosis</h4><p>On imaging consider</p><ul><li><a href="/articles/hughes-stovin-syndrome">Hughes-Stovin syndrome</a></li></ul>
References changed:
- 8. Blättler W, Schwarzenbach B, Largiadèr J. Superficial Vein Thrombophlebitis--Serious Concern or Much Ado About Little? Vasa. 2008;37(1):31-8. <a href="https://doi.org/10.1024/0301-1526.37.1.31">doi:10.1024/0301-1526.37.1.31</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/18512540">Pubmed</a>