3 | DISCUSSION
BD was first described by Professor Hulusi Behçet in 1937 [9]. Since
then, many diagnostic criteria have been established. In 2014, the
revised version of the International Criteria for Behçet’s Disease
(ICBD) was proposed, and a score of ≥ 4 points corresponds to the
diagnosis [1]. The pathergic test and HLA-B51 typing are not
specific, and their positivity is influenced by geographical variations
[4]. Our patient had oral aphthosis (2 points), genito-anal
ulcerations (2 points), and vascular manifestations (1 point),
fulfilling the ICBD criteria.
The age of onset of BD is relatively early. It is usually diagnosed in
the second and third decades of life. Diagnosis before the age of 15 and
after the age of 50 is exceptional [3,10]. In our case, the
diagnosis was made at the age of 24. Both sexes can be similarly
affected, but the prognosis is worse in men. In addition, cardiovascular
manifestations are more common in men [3,7].
Cardiac involvement is a serious complication reported in less than 5%
of cases [10]. It can manifest as pericarditis, myocarditis,
endocarditis, intracardiac thrombosis, myocardial fibrosis, myocardial
infarction and DCM [6]. The most common form is pericarditis. DCM is
less common and can manifest as systolic or diastolic heart failure or
be asymptomatic. In a study conducted in Iran, 4.3% of patients
developed DCM [11,12]. Although rare, BD should not be overlooked in
young adults presenting with heart failure. Signs of BD should be sought
on clinical examination. In our case, special attention to bipolar
aphthosis during the initial hospitalization may have led to an early
diagnosis of BD.
Vascular manifestations of BD (or angio-Behçet) occur in 7 to 32% of
cases. They are dominated by venous involvement, mainly deep vein
thrombosis [13]. The majority of patients present with the first
vascular manifestation within five years of disease onset. In 20% of
cases, vascular involvement occurs simultaneously [7]. In our case,
vascular lesions were discovered at the time of BD diagnosis.
Arterial involvement is less common, occurring in 5-10% of cases. The
frequency is probably underestimated, as autopsy data have reported a
higher proportion [10]. Arterial lesions usually present as
aneurysms and rarely as thrombosis [7]. Pseudoaneurysms are most
common. Arterial aneurysms are often multiple, mainly affecting the
abdominal aorta and femoral arteries [10].
Involvement of the SMA is very rare, accounting for 5.5% of all
visceral aneurysms [8]. A review of the literature by Kakehi et al.
in 2019 found 15 cases [14]. Men were most affected. Abdominal pain
was the main symptom (80%). Lesions consisted of aneurysm (11 cases),
thrombosis (4 cases) and dissection (1 case) and were mostly associated
with other arterial manifestations. In six patients, the discovery of
SMA involvement was concomitant with the diagnosis of BD. In our case,
it was an asymptomatic aneurysm of the SMA.
Pulmonary artery involvement is rare, with an overall incidence of less
than 5%. It usually occurs 3 to 4 years after disease onset
[6,7,15]. Pulmonary artery aneurysms are the most common, followed
by pulmonary embolism. Pulmonary embolism can be isolated (3%) or
associated with aneurysms (25%) [6]. In our case, pulmonary
embolism was suspected four months before the diagnosis of BD. CT scans
showed no direct evidence of pulmonary embolism, but pulmonary
infarction and minimal pleurisy were indirect signs.
The two aneurysms associated with cardiac involvement and pulmonary
embolism represent the originality of our observation. These are
important complications of BD. Atherosclerosis, infection, and
Takayasu’s arteritis are the main differential diagnoses for aortic
involvement. The young age, low tobacco exposure, and absence of
diabetes or dyslipidemia did not suggest an atheromatous origin.
Infectious tests (HIV, viral hepatitis, syphilis, and tuberculosis) were
negative. Classification criteria for Takayasu’s arteritis were not
complete.
A multidisciplinary approach is essential for the management of
cardiovascular complications. The 2018 European League Against
Rheumatism (EULAR) recommendations suggest the combination of high-dose
corticosteroids with cyclophosphamide in the acute phase of
angio-Behçet. Surgical treatment should be performed at a later stage to
reduce the risk of complications. Curative anticoagulation is
recommended for venous thrombosis in the absence of pulmonary artery
aneurysm [16]. There is no consensus on the treatment of cardiac
involvement. The goal is to reduce inflammation to limit lesion
progression and treat symptoms. The efficacy of anti-inflammatory and
immunosuppressive drugs has been demonstrated [12]. However,
treatment is individualized according to the patient’s age and the type
and severity of complications. We chose medical management alone because
the aneurysms were asymptomatic and our local technical platform does
not allow us to perform vascular surgery.