Background/Purpose:

Takayasu’s arteritis (TA) and Behçet’s syndrome (BS) are both
systemic vasculitis of an unknown etiology, each with unique involvement
pattern. TA affects aorta and its main branches causing narrowing or occlusions.
BS is characterized by recurrent skin –mucosa lesions and uveitis. Arterial
involvement is rare in BS and manifests usually as aneurysms or in situ
thrombosis.

We describe here 8 TA patients with concomitant BS.

Methods:

We reviewed the charts of patients
diagnosed with BS and TA for information regarding patients’ gender, age at
diagnosis of BS and TA, BS manifestations,
symptoms prior to TA diagnosis, involved vessels and the used drugs. The diagnosis of TA
was based on the finding of typical homogenous arterial wall thickening.

Results:

We identified 8 (0.1%)
patients among 9000 BS patients. Table summarizes
demographic and clinical characteristics of these patients. Their mean age at
the time of diagnosis of BS was 31.6 ± 11.5 yrs, and
at the time of diagnosis of TA was 37.5 ± 10.8. F/M ratio was 3/1. TA
preceded BS in 4 cases (6, 6, 12, 15 yrs) and occurred
simultaneously in the remaining 4. Skin-mucosa lesions were the most common
finding, followed by uveitis (5/8), and
arthritis (3/8). Initial symptoms of TA were fatigue and fever in 2 patients,
absent pulse in 2, fatigue in 1, arm claudication in 1. The remaining 2 were
diagnosed as TA while being evaluated for the extent of vascular disease for
BS. Subclavian (5/8) and carotid arteries (5/8) were the most commonly involved
arteries.  In addition to prednisolone, the
initial agent was methotrexate in 4 patients, azathioprine in 3 and
cyclophosphamide in 1. At the end of follow-up (1, 2, 2, 3, 7, 9, 18, 21 yrs),
4 patients had a stable disease following the first
treatment, 3 had to switch to infliximab and 1 had to switch to azathioprine
after methotrexate. BS manifestations resolved in 6 patients while recurrent
arthritis persisted in 2. Six patients were still on immunosuppressive therapy
due to TA, while the other 2 were off treatment. None had died.

Conclusion:

 TA may be associated with BS. Similar
associations of TA have been reported with ulcerative colitis, Crohn’s disease,
and ankylosing spondylitis. Whether it is a true association or mere
co-existence is always debated. Interestingly, in this hybrid setting, both TA
and BS followed their own course: while BS abated in time, TA continued its
persistent activity. 

Table: Demographic and
clinical characteristics of patients with TA and BS

A: arthritis;
AZA, azathioprine; BT, brachiocephalic trunk; 
CAR: carotid artery; CT: celiac trunk; CYC: cyclophosphamide; EN:
erythema nodosum; F: female; G: genital ulcers; INF:
infliximab; M: male; MTX: methotrexate; O: oral ulcers; PP: papulo-pustular
lesions, PAA: pulmonary artery aneurysm; RA: renal artery; SBC: subclavian
artery; SFA: superficial femoral artery, SMA: superior mesenteric artery; STM:
superficial thrombophlebitis; ThAo: Thoracic
aorta; U: uveitis.