CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 2, March/April 2014
AFRICA
65
Arterial system involvement is rare and may be seen as arterial
thrombosis and aneurysm. Two-thirds of arterial involvement
cases consist of aneurysm and the remaining one-third are
occlusive arteritis. Vascular invasive interventions mostly end up
with relapsing aneurysms at either the proximal or distal ends
of grafts. Postoperative aneurysm progress ranges from one to
12 months. Even catheterisation for vascular imaging may cause
aneurysms, and recurrent surgery increases the mortality rate
and morbidity in patients with this disease.
In patients with Behcet’s disease, cardiac involvement
can be seen as pericarditis, acute myocardial infarction and
cardiomyopathy. The involvement of heart valves is rare and is
reported as case reports in the literature.
16
Behcet’s disease vascular involvement increases the risk for
mortality. Serious vascular complications including ischaemic
cerebrovascular events, ischaemic bowel perforation, Budd-
Chiari syndrome and perforation of aneurysms occur in 8% of
patients and cause death in most patients. The most common
vascular event is lower-extremity venous thrombosis. Thrombosis
impairing venous return to the great vessels causes superior vena
cava syndrome and Budd-Chiari syndrome.
16,17
Dural venous
thrombosis leads to intracranial hypertension.
18,19
Patients with Behcet’s disease have a tendency to thrombosis
because of an imbalance between procoagulant and anticoagulant
factors. Lee
et al.
reported 90% of the 171 patients in one
study and 3% in another had thrombosis.
20
Antithrombin 3,
protein C and protein S deficiencies increase susceptibility to
hypercoagulation. Prothrombin G20210A polymorphism and
factor V Leiden mutation (506Arg/gln) have been observed in
idiopathic deep-vein thrombosis.
16,17,21
Superior and inferior vena cava thrombosis have been reported
at 9 and 2.5%, respectively.
11
In our study we observed lower-
extremity deep-vein thrombosis in three cases. One patient had
a thrombosis in the superior vena cava and internal jugular vein
and three had vena saphena magna thrombosis.
Most commonly, arterial aneurysms are seen in the abdominal
aorta, and in decreasing frequency, in the femoral artery,
popliteal artery and pulmonary artery, respectively. In our study
we did not detect any cases of aneurysm. We did find arterial
occlusion in three (1%) patients, one of which was occlusion at
the iliac level. In one study, prevalence was reported as 2.2% in
a series including 450 cases.
22
Arterial involvement is most common in the form of aneurysm
and pseudo-aneurysm formation, and occasionally arterial stenosis.
Arterial occlusion can result in organ failure and sometimes
causes infarct. The involvement of major arteries usually occurs
after an average of 5.8 years and mainly involves lower-extremity
arteries, and rarely upper-extremity arteries. Ranked in ascending
order of prevalence, the right pulmonary artery, femoral, popliteal,
subclavian and carotid arteries are involved.
Histopathological studies reveal non-specific vasculitis.
Mononuclear and neutrophilic infiltration, endothelial
proliferation, destruction of the elastic lamina, fibrinoid necrosis
and thrombus are pathological evidence of the disorder seen
at the tissue level. Combined treatment with corticosteroids,
anticoagulants and immunosuppressive therapy is necessary.
22-24
In a study by Hong
et al.
, it was reported that carotid arterial
intimal thickness increased compared to the control group,
which included normal healthy subjects (carotid artery intima–
media thickness in Behçet’s disease patients without significant
cardiovascular involvement).
25
Uveitis or retinal vasculitis was
found to correlate with increased carotid intimal thickness.
While Balile
et al.
found increasing age and serum cholesterol
levels in patients was directly proportional, with steroid use, a
correlation was not found.
27
In our study, in patients with carotid
artery intimal thickening, there was no correlation between
serum cholesterol levels, age and the use of steroid eye ointment,
and none of the patients had retinal vasculitis.
In a study conducted in Korea, an increase in regional arterial
segmental stiffness was reported and it reached statistical
significance.
28
The exact pathophysiology of heart valve
involvement causing insufficiency is unclear in patients with
Behcet’s disease. Inflammation causes destruction of the valve
tissue and dilatation of the ascending aorta and sinus Valsalva
aneurysm. Histological findings varied from normal tissue to
fibrosis independent of inflammation.
Accumulation of inflammatory cells in the adventitia and
media is seen in inflammatory cases. Neutrophils, lymphocytes,
plasma cells, less frequently histiocytes and eosinophilic cells,
and sometimes giant cells are observed. Fibrous thickening
of the intima and adventitia can be traced. However, fibrous
changes to the elastic membrane has also been reported.
29
Valves
with aortic regurgitation were found in six patients in our study
but fibrous thickening was not observed in the structure of the
valve, and the aortic annulus was normal except in two patients.
Dilated cardiomyopathy has been reported in patients with
Behcet’s disease. In our study, two patients presented with
increased left ventricular diameter. Heart valve involvement is
rare but if present, increases the mortality rate.We believe patients
should be followed up with echocardiography annually. In our
study, we detected increased incidence of venous insufficiency.
To lower thrombosis risk in patients with Behcet’s disease,
accompanying venous insufficiency should be diagnosed and
treated early.
Conclusion
Rates of heart and vascular involvement in Behcet’s disease
range widely in the literature but this involvement increases the
risk of mortality and morbidity, requiring a multidisciplinary
approach.
References
1.
Lehner T, Welsh KI, Batchelor JR. The relationship of HLA-B and DR
phenotypes to Behçet’s syndrome. Recurrent oral ulceration and the
class of immune complexes.
Immunology
1982;
47
: 581–587.
2.
Behçet’s Disease Research Committee of Japan. Skin hypersensitivity
to streptococcal antigens and the introduction of systemic symptoms
by the antigens in Behçet’s disease – a multicenter study.
J Rheumatol
1989;
16
: 506–511.
3.
Sezer TN. The isolation of a virus as the cause of Behçet’s disease.
Am
J Ophthalmol
1953;
36
: 301–315.
4.
Hamzoi K, Ayed K, Slim A. Natural killer cell activity, interferon
gamma and antibodies to herpes viruses in patients with Behçet’s
disease.
Clin Exp Immunol
1990;
79
: 28–30.
5.
Münke H, Stöckmann F, Ramadori G. Possible association between
Behçet’s syndrome and chronic hepatitis C virüs infection.
N Engl J
Med
1995;
332
: 400–401.
6.
Özaran K, Aydıntug O, Tokgöz G,
et al
. Serum levels of IL-8 in patients
