Subclavian artery pseudoaneurysm in vascular Behcet's disease repaired using endovascular treatment: A case report of the clinical course over 10 years

Abstract

The vascular involvement in patients with Behcet's disease is defined as vascular Behcet's disease, which can greatly influence the prognosis. However, there are few reports on endovascular treatment (EVT) for subclavian pseudoaneurysms with long-term prognosis over 10 years. We present the case of a 42-year-old man with left subclavian artery pseudoaneurysm due to vascular Behcet's disease who was treated with EVT along with immunosuppressive therapies. Subsequently, 8 years after, the aneurysm recurred in the stent with stent fracture. Therefore, additional EVT was performed. We present the details of the patient's clinical course over 10 years after the EVT.

<Learning objective: Vascular Behcet's disease can greatly influence the prognosis. Therefore, understanding its treatment and management is important. There are few reports on endovascular treatment (EVT) for subclavian pseudoaneurysms with long-term prognosis over 10 years. Long-term management and recurrence monitoring are required for vascular Behcet's disease even after EVT.>

Introduction

Behcet's disease (BD) is a systemic autoinflammatory disease, and in those with vascular involvement is defined as vascular BD. Vascular involvement is present in 25–30% of patients with BD and adversely affects their prognoses. The prevalence of arterial involvement is 12%, which is lower than that of venous involvement (88%). Aortic aneurysms can occur in the arteries throughout the body in BD [1]. Many of these lesions are treated with endovascular treatment (EVT) or surgery [2]. We report the 10-year clinical course following EVT for rupture of the subclavian pseudoaneurysm in a patient with BD.

Case report

A 42-year-old man presented with chief complaint of severe pain in the left clavicle and a pulsatile mass; rheumatoid arthritis was suspected and he was followed up for 1 year without any medication. An aneurysm had not been pointed out by plane computed tomography (CT) at the first visit to our hospital one year previously. After that, he had not visited our hospital. His history included diabetes, dyslipidemia, and smoking. Physical examination at the time of admission revealed left uveitis, arthralgia of the lower extremities, acne, oral ulcers, and a positive needle reaction test. He was diagnosed with BD based on the standard criteria [3]. Contrast-enhanced CT revealed impending rupture of a 6.5 × 4.5 cm pseudoaneurysm with thrombus in the left subclavian artery; the diagnosis of vascular BD was established (Fig. 1A,B). Blood pressure in the right upper arm was 118/78 mmHg and that in the left upper arm was 98/62 mmHg. He was positive for HLA-A24 and A26, while HLA-B was not measurable. The laboratory findings were the following: white blood cells (WBC), 11,900/μL and C-reactive protein (CRP), 15.0 mg/dL. Additionally, antibodies suggestive of collagen disease were not detected. No significant abnormal findings were observed on electrocardiogram, chest X-ray, and echocardiography.

Fig. 1.

Contrast-enhanced computed tomography (CT), intravascular ultrasound (IVUS), and angiogram showed left subclavian artery pseudoaneurysm before and after endovascular treatment (EVT). (A) Contrast-enhanced CT: Left subclavian artery pseudoaneurysm before EVT. (B) 3D-CT: Left subclavian artery pseudoaneurysm before EVT. (C) Selective angiogram of the left subclavian artery via a right common femoral artery showed an aneurysm from the left subclavian artery. (D) IVUS showed a huge defect in the vessel wall at the site of the aneurysm. (E) After an 8.0 × 80 mm endovascular stent graft (Niti-STM ComVi Biliary Stent, Century Medical, Inc., Japan) was deployed. (F) Selective angiogram of the left subclavian artery via a right common femoral artery after stent graft was deployed showed that arterial flow to aneurysm disappeared. (G) Contrast-enhanced CT: Covered stent was patent and had no recurrent aneurysm in the left subclavian artery one year after the first EVT. (H) 3D-CT: Left subclavian artery one year after the first EVT.

Emergency EVT was performed and catheter angiography revealed a subclavian artery aneurysm that was too large to be clearly visualized with contrast media (Fig. 1C). Intravascular ultrasound (IVUS) revealed narrowing of the artery due to compression by the aneurysm and a huge defect in the vessel wall at the site of the aneurysm (Fig. 1D). A covered stent with a diameter of 8.0 × 80 mm expanded polytetrafluoroethylene (ePTFE) (Niti-STM ComVi Biliary Stent, Century Medical, Inc., Japan) was deployed not to cover the entrance of the peripheral artery branch (Fig. 1E,F). Pain and the difference in the blood pressure between the arms disappeared immediately after covered stent placement, and there was no major complication.

Postoperatively, heparin, acetylsalicylic acid (ASA), clopidogrel, and cilostazol were prescribed. Clopidogrel was terminated 3 months after the surgery. Immunosuppressive therapy was started as following: Prednisolone was prescribed from 50 mg and then gradually decreased to 5 mg over a year. Seven years later, CRP increased from 0.76 mg/dl to 12.6 mg/dl with the onset of intestinal BD, then prednisolone was increased to 25 mg. After the start of mesalazine and azathioprine associated with the onset of intestinal BD, prednisolone was gradually decreased to 15 mg before the second EVT. Cyclophosphamide was started at 100 mg after the first EVT and gradually stopped when cyclosporine started 6 months later. Cyclosporine was started from 50 mg 6 months after the first EVT and gradually increased to 100 mg. It was discontinued due to the start of mesalazine and azathioprine. Infliximab started at 400 mg 9 months after the first EVT, gradually increased to 700 mg, and was also used at the time of the second EVT.

Mesalazine 750 mg and azathioprine 50 mg were started 7 years after the first EVT, due to the complication of intestinal BD, and azathioprine was gradually increased to 100 mg. Since then, both had been used together at the time of the second EVT. However, CRP had increased to 9.5 mg/dl at the time of the second EVT.

Contrast-enhanced CT one year after the first EVT revealed good patency of the stent graft without recurrence of aneurysm including at the puncture site (Fig. 1G,H). After that, no abnormality was found on CT. However, seven years after the first EVT, the stent structure broke and an aneurysm recurred in the stent with stent fracture on contrast-enhanced CT (Fig. 2A,B). These were also observed on fluoroscopy during the second EVT angiogram (Fig. 2C,D). A partial defect in the stent structure suggesting stent fracture was also observed in IVUS (Fig. 2E).

Fig. 2.

Contrast-enhanced computed tomography (CT), intravascular ultrasound (IVUS), and angiogram showed that recurrent left subclavian artery aneurysm with stent fracture in the covered stent 8 years after the first endovascular treatment (EVT). (A) Breakage of stent structure and recurrent left subclavian artery aneurysm 8 years after the first EVT. (B) 3D-CT. (C) Fluoroscopy showed the breakage of stent structure. (D) Selective angiogram of the left subclavian artery via a right common femoral artery showed recurrent aneurysm in the covered stent with fracture. (E) IVUS showed a partial defect in the stent structure suggesting stent fracture. (F) Fluoroscopy with a 7.0 × 50 mm endovascular stent graft (GORE^Ⓡ VIABAHN^Ⓡ Endoprosthesis, W. L. Gore & Associates, Inc., USA) deployed on the recurrent left subclavian artery aneurysm. (G) Angiogram with the endovascular stent graft deployed on the recurrent left subclavian artery aneurysm.

A second EVT was performed and a covered stent of 7.0 × 50 mm ePTFE (GORE^Ⓡ VIABAHN^Ⓡ Endoprosthesis, W. L. Gore & Associates, Inc., USA) was deployed within the first covered stent (Fig. 2F,G). Before the second EVT, dual antiplatelet therapy, ASA, and clopidogrel were prescribed and ASA was terminated 1 year later. Two years after the second EVT, prednisolone was gradually decreased from 15 mg to 2.5 mg, and infliximab 700 mg, mesalazine 750 mg, and azathioprine 100 mg were continued without change from the time of the second EVT, and the CRP (0.73 mg/dl) is stable. Two years have passed since the second EVT without recurrence (Fig. 3A,B).

Fig. 3.

Contrast-enhanced computed tomography (CT) showed the left subclavian artery 2 years after the second endovascular treatment (EVT). (A) Left subclavian artery 2 years after the second EVT. (B) 3D-CT.

Discussion

BD is a systemic autoinflammatory disease with a reported 10-year mortality rate of 5%. The 20-year survival rate in the presence of arterial lesions is 73%, which is low compared with the rate of 89% in the absence of arterial lesions, and it is important to search for arterial involvement throughout the body for their prognosis [4]. A previous study reported that the frequency of subclavian aneurysms was approximately 21% in the group of aneurysms excluding intracranial aneurysms [5]. In the current patient, the diagnosis of BD was delayed, which delayed effective active treatment, a rapid onset of left subclavian artery pseudoaneurysm was presumed.

In surgical treatment of vascular BD, graft occlusion is common [6]. Pseudoaneurysm of the anastomotic site is reported as a major complication in approximately 30–50% of postoperative cases [2]. The problem is that re-operation is also difficult in these lesions. Therefore, EVT—which is less invasive—is an alternative to surgical treatment and the reported incidence of pseudoaneurysm after surgery is as low as 14–22% [2]. EVT has also been reported to be effective in terms of safety and long-term prognosis for approximately 5 years [2]. However, there is a report that the graft occlusion rate in non-pulmonary artery lesions is 40% [7]. The indications for EVT and surgical treatment have not been established [8]. Therefore, the selection of procedure should be considered in an individual case, and it seems necessary for both to closely observe the subsequent course. To prevent recurrence of pseudoaneurysm after the surgery, preoperative and postoperative immunosuppressive therapy with azathioprine, cyclophosphamide, and biological agents, such as anti-tumor necrosis factorα antibodies, are recommended [8,9]. Regarding postoperative anticoagulation and antiplatelet therapy, there is insufficient evidence currently; further evidence regarding these therapies and in combination with immunosuppressive therapy is needed [8,9].

In this patient, we chose EVT to avoid complications of surgical treatment. Additionally, the patient had a ruptured pseudoaneurysm and saving his life was the highest priority. Therefore, immunosuppressive therapy, anticoagulant therapy, and antiplatelet therapy were prescribed postoperatively. However, 8 years later, aneurysm recurred in the covered stent with stent fracture.

It has been reported that stent fracture is observed between the thorax and the clavicle and caused by the mobility of upper arm [10]. In addition, in Japan at the time of the first EVT, there was no graft stent for blood vessels that could be used, and the one that could be used for this case was the stent for biliary duct. Therefore, it is possible that the stent was vulnerable to the motion. The clinical course was good in this patient for 2 years after the second EVT and 10 years after the first EVT with antiplatelet and immunosuppressive therapies.

For long-term monitoring, regular whole-body scanning with plane, contrast-enhanced CT and vascular echocardiography may be useful. It seems important to combine the modality and timing according to the symptoms.

There is no case report of a 10-year history of subclavian pseudoaneurysm associated with vascular BD; however, it requires attention to prevent future recurrence of arterial lesions including stent stenosis, occlusion, and stent fracture.

Conclusion

We presented the case report of a patient with vascular BD who was presumed to have a rapid onset of left subclavian artery ruptured pseudoaneurysm within 1 year. We performed life-saving EVT and subsequently prescribed anticoagulant, antiplatelet, and immunosuppressive therapies. Recurrence of aneurysm was observed 8 years later in the covered stent with stent fracture. EVT was repeated 8 years after the first EVT. Good clinical course was observed for 2 years after the second EVT and 10 years after the first EVT with antiplatelet and immunosuppressive therapies.

Funding

None.

Declaration of Competing Interest

None.