Behçet’s disease is most common among the
Mediterranean, Middle and Far East Asia populations
along the old silk route. The etiology is still unclear,
but likely to be multi-factorial. Although the usual onset of disease is in the third decade of life, it
can occur at any age.[
1] Men and younger patients
often have more severe disease than women and
older patients. Behçet’s disease has various clinical
features and progresses with unpredictable attacks and
remissions. Vasculitis is considered to underlie these
clinical manifestations.[
1,
2] Vascular involvement may
be seen in up to 50% of patients depending on the
population studied. Involvement in BD can be arterial
or, more commonly, venous and may involve both
systems in a single patient. The most frequent type
of vascular manifestation is superficial and deep vein
thrombosis, mostly in the lower extremities. After the
first vascular lesion, usually venous thrombosis, the
risks are increased for other vascular lesions. Although
rare, arterial involvement is the main predictor of
morbidity and mortality. Arterial lesions are often
isolated, but may be multiple and frequently coexist
with venous thromboses. Arterial manifestation
includes true aneurysm or pseudoaneurysm formation
and less commonly stenosis or occlusion. The main
locations of the arterial lesions are the aorta followed
by the pulmonary and femoral arteries. Other
reported arterial lesions include subclavian, common
carotid, coronary, brachial, radial, ulnar, common
iliac, external iliac, tibial, renal, cerebral, axillary,
and splenic arteries.[
1,
2,
5-
7] Our case had a history of
deep vein thrombosis five years ago and claudication
thereafter.
The diagnosis of disease is primarily based on
clinical criteria, as there are no specific laboratory
or histopathological findings. The ESR and CRP are usually moderately elevated; however, they do
not correlate well with the disease activity. Previous
studies recommended immunosuppressive therapy to
normalize the ESR and CRP values preoperatively
or before endovascular treatment.[6,7] Postoperative
therapy was also reported to be critically important to
prevent recurrent aneurysmal disease or formation of
pseudoaneurysms at the site of artery access.[8,9]
Furthermore, vasculo-BD patients usually have
a dramatic course and prognosis. Ischemia of the
arterial wall due to an inflammatory obliteration of
the vasa vasorum may result in true or false aneurysm
formation with an increased incidence of rupture and
bleeding. The rupture of a aneurysm is a major cause of
death.[5,6] Arterial complications may lead to difficult
surgical problems, as surgical repair may provoke
development of new aneurysms at the site of vascular
interference or anastomosis and may be complicated by
the graft thrombosis in high percentage of patients. To
avoid surgical complications, endovascular treatment
have been recommended increasingly, since it is less
invasive and has fewer complications.[5,8-10]
Although arterial stenosis or occlusion in BD is
rare, nearly every major artery has been reported to
be involved by this disease.[6,11-13] Some of them were
treated by balloon angioplasty and stent implantation
to improve symptoms. In a previous study, long-term
results in the recanalization of chronic iliac occlusions
were acceptable compared to conventional surgical
techniques.[14] However, experience with endovascular
treatment of the iliac artery stenosis or occlusion in
BD and their long-term results are very limited. Only
two cases of endovascular treatment of an isolated
common iliac artery stenosis or bilateral aortoiliac
bifurcation stenosis in patients with BD have been
reported previously.[3,4] To the best of our knowledge,
our case, who presented with aortoiliac occlusion in
BD, is the first case of this type reported. Because
of severe claudication symptoms, angioplasty and
stent implantation was performed for management of
aortoiliac occlusion. At the time of the procedure, she
was on systemic immunosuppressives and her ESR and
CRP values were close to the upper of normal ranges.
Endovascular treatment was successful, in our case,
without any complication related to angiography and
angioplasty.
Arterial punctures or insertion of a vascular sheath
for angiography may induce either a thrombosis or a
pseudoaneurysm formation in the puncture site.[15] As a result, CT angiography was obtained after six months
for discovery of a new pseudoaneurysm, stenosis
or occlusion. No aortoiliac or femoral aneurysm
appeared. There was no significant interval change in
the residual stenosis on the left side.
In conclusion, arterial stenosis or occlusion
are extremely rare, particularly at the aortoiliac
bifurcation or common iliac arteries due to Behçet’s
disease. Endovascular stent implantation with
the ‘‘kissing stents’’ technique is an appropriate
therapeutic choice for these patients. Not only the
vascular or endovascular interventions, but also
medical treatment strategy for the Behçet’s disease
should be taken into consideration.
Declaration of conflicting interests
The author declared no conflicts of interest with respect
to the authorship and/or publication of this article.
Funding
The author received no financial support for the research
and/or authorship of this article.
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