Superior Mesenteric Artery Vasculitis in Behçet's Disease: A Case Report and Literature Review

Abstract

A 55-year-old male presented with abdominal pain that had begun about 5 days ago. Physical examination revealed oral aphtha, genital aphthosis, and pseudofolliculitis, and the patient was diagnosed with incomplete Behçet's disease (BD). Contrast-enhanced computed tomography (CECT) showed dilation of the superior mesenteric artery and mesenteric infiltration of inflammation, indicating vasculo-BD. The symptoms were improved by 3-day of intravenous methylprednisolone pulse therapy followed by oral prednisolone. A literature review suggested that vasculo-BD should be included as a differential diagnosis in cases with unexplained abdominal pain, arterial dilation, and mesenteric invasion, and CECT examination and steroid therapy should be considered.

Successful conservative treatment of an isolated inflammatory superior mesenteric artery aneurysm

“…Abdominal pain, inflammatory markers and radiological presentations were all improved after steroid therapy. These findings were also consistent with inflammatory aneurysms [ 10 ]. In addition, other potential causes, such as infection, trauma and arterial dissection, were excluded.…”

“…The diagnosis of an inflammatory SMA aneurysm was established based on clinical judgement. First, our patient had abdominal pain, which is the most common symptom in patients with inflammatory aneurysms [ 10 ]. Second, the inflammatory markers were elevated, which was consistent with inflammatory aneurysms [ 3 , 4 ].…”

“…For inflammatory SMA aneurysms, there is no consensus on therapeutic management because of the rarity of the disease. In the literature, most previous cases were treated surgically with or without anti-inflammatory treatment [ 3–6 , 10 ]. Anti-inflammatory treatment was not used due to unrecognized inflammation or aneurysm rupture [ 3 , 5 ].…”

Ultrasonographic measurement of the vascular wall thickness and intima-media thickness in patients with Behçet’s disease with symptoms or signs of vascular involvement: A cross-sectional study

“…[ 22 ] Although US, computed tomography, MRI-venography, and angiography are used for the diagnosis of vascular involvement in BD patients, we could not find an appropriate imaging recommendation in the literature for its screening. [ 23 ] However, Doppler US is a practical, easy-to-access, cost-effective and non-ionizing examination with increasing use in rheumatology clinics in recent years. [ 10 ] In a study by Alibaz-Oner et al,[ 10 ] when the lower extremity VWTs were evaluated with Doppler US, it was emphasized that a cut-off value of 0.49 mm obtained for common femoral vein was highly sensitive and specific to differentiate BD from vasculitis and spondyloarthropathies.…”

The Diagnosis and Management of Vasculo-Behçet's Disease

“…The abdominal aorta is the most common site of aneurysm in Behçet's disease, while the ulnar, celiac, subclavian, left anterior descending, tibioperoneal, iliac and superior mesenteric arteries have been reported as rare sites of aneurysm (8). Kakehi et al reported a new-onset case of Behçet's disease with aneurysmal lesions in the superior mesenteric artery (9). The patient was accompanied by upper abdominal pain in addition to oral aphtha, genital ulcers and folliculitis-like rashes.…”