The heart in Behçet's disease
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Overview
The following cardiac abnormalities have been associated with Behcet's disease:
- Intra cardiac thrombosis
- Myocardial infarction
- Pericarditis
- Endocarditis
- Mitral Valve Prolapse
- Endomyocardial fibrosis
- Arrhythmias
- Coronary artery vasculitis
- Myocarditis
Valvular Disease
The cardiac valves may grow vegetations which may subsequently and embolize. Clinically, these lesions are similar to bacterial endocarditis, but cultures are negative and round cell infiltration is most typically observed on histology.
Valvular disease [1], myocardial infarction and aneurysms [1] [1], intracardiac thrombus [1] and endomyocardial fibrosis [1] have been reported in patients with Behcet disease.
Of significance, mitral valve prolapse and dilatation of the proximal aorta were observed in 50% and 30% of patients with Behçet's disease, respectively [1]
Vascular manifestations
- Superficial thrombophlebitis
- Deep venous thrombophlebitis
- Small vessel vasculitis
- Arterial Occlusion and/or Aneurysms
The four types of vascular lesion recognized in Behçet's disease are arterial and venous occlusions, aneurysms and varices. Venous involvement typically includes superficial thrombophlebitis or deep venous thrombosis. Patients with intracranial venous occlusion may present with headache and visual blurring.
Behçet's disease gives rise to a chronic relapsing systemic vasculitis involving arteries and veins of various sizes. The vascular involvement may consist of thrombophlebitis, deep vein thrombosis, and arterial obstruction.
Aneurysms, particularly of the pulmonary arteries, may also develop [1]
Venous involvement usually arises within five years of the initial presentation of Behçet's disease and indeed can be the initial feature of the disease. Superficial and deep vein thrombosis are common features, although embolization is uncommon. Recurrent thrombophlebitis of the legs may lead to stasis dermatitis and crural ulcers.
Thrombosis of the inferior vena cava and hepatic veins in patients with Budd-Chiari syndrome and Behçet's disease carries a poor prognosis. Arterial disease is less common than the venous complications of Behçet's disease but has greater morbidity.
The most frequently affected vessels, in decreasing order, are the pulmonary, femoral, popliteal, subclavian, and carotid arteries. Arterial involvement predisposes to aneurysm formation or arterial occlusion.
Vasculitis of the small and large vessels can cause a variety of symptoms. Vascular manifestations have been reported in 7%-40% of the patients (more frequently in males) with Behçet's disease, and are commonly considered as essential to the pathology of Behçet's disease. Histologic findings include thickening of media, splitting of the elastic fiber and perivascular round cell infiltration.
Occlusion of major veins may cause bleeding, infarction, organ failure or restriction of arm and leg movement. Rupture of a vascular aneurysm may be fatal. Venous involvement is usually limited to occlusion, with the varices rarely affected. Most affected sites of the venous system are the superior vena cava, inferior vena cava, deep femoral vein and subclavian vein. Up to 20% of patients with occlusive venous disease are anticardiolipin antibody positive. With venous occlusion, collateral circulation may develop.
Epidemiology and Demographics
Worldwide, vascular involvement in Behçet's disease is common, although there is some geographic variation in frequency. Vascular complications are slightly less common in children (from 10.5% to 21%) than in adults (from 8% to 26.5%), although the disease can be more severe in children than in adults.
Arterial complications make up 7% of cases (aneurysm and occlusion most common). Patients with arterial manifestations may present with thrombosis or aneurysm formation, with possible fatal rupture. Aneurysm formation carries a worse prognosis than occlusive disease. The subclavian artery and pulmonary artery are most common arteries occluded. Depending on the site, arterial occlusions can have different clinical presentations. Pulseless disease is due to subclavian artery occlusion. Arteritis may involve the aorta or its branches and lead to aneurysm formation.
Obliterating thrombophlebitis, arterial occlusion and aneurysm may occur in vessels of all sizes.
Deep venous thrombophlebitis has been described in about 10%-15% of patients, and superficial thrombophlebitis occurred in 24% of patients in the same study. Symptoms correlate with the vessel involved and may be devastating. For example, Budd-Chiari syndrome is reported in these patients (particularly younger ones), and as many as 32% of the patients with vascular involvement in one series had occlusion of the vena cava. Esophageal varices also have been described.
Other Cardiovascular Manifestations
Hypertension
Hypertension can originate from renal artery stenosis.
Avascular necrosis of the femoral head
Avascular necrosis of the femoral head can be caused by femoral artery stenosis and intermittent claudication may be present.
Pulmonary vasculitis can produce dyspnea, chest pain, cough or hemoptysis.
Aneurysm
Although less common than occlusion, aneurysm formation accounts for most vascular deaths. Common sites of aneurysms are abdominal aorta, femoral artery and thoracic artery.
Treatment
As vascular involvement of Behçet's disease can be significant and prove life threatening, it is vital to find and treat vascular involvement at the earliest possible stage.[1]
References
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
