Which vasculopathy findings are characteristic of Behçet disease?

Updated: Dec 21, 2020
  • Author: Nicole Davey-Ranasinghe, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Behçet disease can cause aneurysms in the pulmonary arterial tree that often prove to be fatal. Pulmonary artery aneurysmal involvement is associated with right-sided cardiac thromboses and can manifest as hemoptysis, cough, chest pain, or dyspnea. [15]

Vasculitis of the small and large vessels can cause a panoply of symptoms depending on location of the lesions.

Arterial disease predominantly affects males and only rarely occurs in women. [34]

Venous involvement (usually in the form of superficial thrombophlebitis) is more common than arterial involvement. [33] Superficial thrombophlebitis presents in a linear fashion with overlying erythema and is often confused with erythema nodosum. In males, formation of these linear areas of vasculopathy leads to sclerosis and stringlike thickening in the affected areas.

Symptoms correlate with the vessel involved and may be devastating. For example, extension of an inferior vena caval clot to the hepatic vein may be the mechanism of Budd-Chiari syndrome in Behçet disease. [34]


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