Successful Treatment of Vasculo-Behcet’s Disease Presenting as Recurrent Pseudoaneurysms: the Importance of Medical Treatment
A 33-year-old Chinese male was admitted to the surgical ward due to a pulsating subcutaneous mass in the right subclavicular region over a 2-month period. The patient denied any history of trauma or manipulation. The patient felt progressive and intermittent numbness of the right upper extremity caused by the rapidly growing mass. A computed tomography (CT) angiogram revealed a right subclavian artery pseudoaneurysm of 4.5 cm × 3.5 cm (Fig. a), which was soon resected and an expanded polytetrafluoroethylene (e-PTFE) vascular graft was performed. Histology of the arterial wall revealed inflammatory cell infiltrate (Fig. ).\nTwo months later, the patient developed a symptomatic recurrence at the surgical site. A CT angiography revealed that there were both proximal and distal anastomotic pseudoaneurysms, 0.76 cm × 0.87 cm and 4.45 cm × 4.67 cm, respectively, at the right subclavian artery (Fig. b). The two anastomotic pseudoaneurysms were also excised.\nFour months after the second operation, the patient suffered from persistent severe left lumbago that responded poorly to analgesics. An abdominal CT angiography revealed aneurysmal dilatation of the upper abdominal aorta and the celiac trunk (Fig. c). Moreover, the left kidney and adrenal gland were atrophied due to compression by aneurysmal dilatation. Consequently, the patient underwent another operation.\nOne month after the third operation, the patient had a pseudoaneurysm of 2.35 cm × 1.74 cm in the right thoracoacromial artery (Fig. d). Because the patient developed multiple and recurrent aneurysms over a short time span, the patient was admitted to the dermatological ward instead of reoperation.\nAfter admission, a detailed review of the patient’s history revealed recurrent oral and genital aphthous ulcers of 10-year duration. The ulcers occurred at least eight times in a year and were described as multiple painful lesions, each of which had a diameter of 2–5 mm. The patient also had a history of recurrent pseudofolliculitis on the neck and back for 7 years. Physical examinations showed a pulsatile swelling in the right axilla. Multiple pigmented macules were seen on the neck and back (Fig. ). The pathergy test was positive. No oral or genital lesions were detected. Neither eye disease nor joint problems were evident. Laboratory investigation revealed erythrocyte sedimentation rate (ESR) of 119 mm/h (reference range 0–15 mm/h) and C-reactive protein (CRP) of 70 mg/L (reference range 0–8 mg/L). Serum immunoglobulin electrophoresis showed that immunoglobulin G (IgG) and immunoglobulin M (IgM) were elevated. Antinuclear antibody and other autoantibodies, such as antineutrophil cytoplasmic antibody and anticardiolipin antibody, were all negative. A thorough checkup of the vascular system of the whole body did not reveal any new significant abnormality except for a pseudoaneurysm in the right thoracoacromial artery. A diagnosis of vasculo-Behcet’s disease was made based on recurrent oral and genital aphthous ulcers, pseudofolliculitis, pseudoaneurysms, and pathergy test. As the disease was active, as evidenced by high ESR, CRP, IgG, IgM, positive pathergy test, and development of fresh aneurysm, the patient was initiated on immunosuppressive therapy using oral prednisone 45 mg (1 mg/kg) daily and intravenous pulse cyclophosphamide 1,200 mg (12 mg/kg/day for 2 days) every 2 weeks. A month later, the pseudoaneurysm in the right thoracoacromial artery was hardened and the CT angiography revealed that the pseudoaneurysm was shrinking. The levels of ESR, CRP, IgG, and IgM were gradually reduced to the normal range. The patient was discharged on a regimen of azathioprine 100 mg (2 mg/kg) daily and prednisone 45 mg daily. The dosages of prednisone and azathioprine were tapered gradually. After 16 months, prednisone was reduced to 10 mg every other day. This dose of prednisone was then continued. After 20 months, azathioprine was reduced to 50 mg/day and discontinued after 30 months. To date, the pseudoaneurysm has regressed without recurrence for approximately 2 years and the patient continues to take prednisone 10 mg every other day.