Behçet disease
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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 complications develop in about 20-40% 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
Distribution
Primarily involves four main systems.
- cardiovascular manifestations of Behçet disease - affects 5-30% of cases. Thickening of the aorta and SVC (vasculitis)
- 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. 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
- gastro-intestinal manifestations of Behçet disease - affects 5-60% of cases
- CNS manifestations of Behçet disease - affects 10-50% of cases
Treatment and prognosis
The disease may recur, progress, or resolve (either spontaneously, within a few weeks to several months, or with corticosteroid therapy).
Etymology
Named after Hulusi Behçet (1889-1948), Turkish dermatologist .
Differential diagnosis
-<p><strong>Behçet disease</strong> is a multi-systemic and chronic inflammatory <a title="Vasculitis" 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>-<li>oral ulceration</li>-<li>genital ulceration</li>-<li>ocular manifestations</li>-</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>- +<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>
- +<li>oral ulceration</li>
- +<li>genital ulceration</li>
- +<li>ocular manifestations</li>
- +</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>-<li>-<a title="factor V leiden mutation" href="/articles/factor-v-leiden-mutation">factor V leiden mutation </a><sup>4</sup>-</li>-</ul><h5>Distribution</h5>-<p>Primarily involves four main systems. </p>-<ul>-<li>-<a title="cardiovascular manifestations of Behçet disease " 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)</li>-<li>-<a href="/articles/thoracic-manifestations-of-behcet-disease" title="Thoracic manifestations of Behçet 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>-<li>-<a title="gastro-intestinal manifestations of Behçet disease" href="/articles/gastro-intestinal-manifestations-of-beh-et-disease">gastro-intestinal manifestations of Behçet disease</a> - affects 5-60% of cases </li>-<li>-<a title="CNS manifestations of Behcet disease" href="/articles/cns-manifestations-of-behcet-disease-1">CNS manifestations of Behçet disease </a>- affects 10-50% of cases</li>-</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>Etymology</h4>-<p>Named after <strong>Hulusi Behçet</strong> (1889-1948), Turkish dermatologist .</p>-<h4>Differential diagnosis</h4>-<ul><li><a title="Hughes-Stovin syndrome" href="/articles/hughes-stovin-syndrome">Hughes-Stovin syndrome</a></li></ul>- +</li>
- +<li>
- +<a href="/articles/factor-v-leiden-mutation">factor V leiden mutation </a><sup>4</sup>
- +</li>
- +</ul><h5>Distribution</h5><p>Primarily involves four main systems. </p><ul>
- +<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)</li>
- +<li>
- +<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>
- +<li>
- +<a href="/articles/gastro-intestinal-manifestations-of-beh-et-disease">gastro-intestinal manifestations of Behçet disease</a> - affects 5-60% of cases </li>
- +<li>
- +<a href="/articles/cns-manifestations-of-behcet-disease-1">CNS manifestations of Behçet disease </a>- affects 10-50% of cases</li>
- +</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>Etymology</h4><p>Named after <strong>Hulusi Behçet</strong> (1889-1948), Turkish dermatologist .</p><h4>Differential diagnosis</h4><ul><li><a href="/articles/hughes-stovin-syndrome">Hughes-Stovin syndrome</a></li></ul>
Images Changes:
Image 1 MRI (T2) ( update )
Caption
was changed:
Case 1 -: with CNS involvement
Image 2 CT (C+ arterial phase) ( update )
Caption
was changed:
Case 2 -: with thoracic involvement
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