Table 1.

Demographic characteristics and follow-up data.

No.	Sex & Age	Symptoms and time of BD diagnosis	ESR prior to operation (mm/h)	CRP level prior to operation (mg/L)	Involved vessels beyond the aorta	Chief complaint	Duration from admittance to operation	Surgical treatment	Systemic immunosuppressive treatment	Duration of follow-up
1	M, 45	O, G, E Diagnosed after the second recurrence	61	75	-	Continuous back pain for 2 months and sudden exacerbation to unconsciousness within 2 hours	30 minutes	Aneurysmectomy reconstructed by aortic and bi-iliac artery interposition Recurrence at the proximal aortic anastomosis; underwent another aortic interposition 10 months after the first operation Recurrence again at the proximal aortic anastomosis and too close to renal artery to perform EVAR 8 months after the second operation	After rejection of surgery for the second recurrent pseudoaneurysm, prednisone qd 80 mg for 1 month followed by 50 mg for 6 months and 25 mg for 6 months, as well as cyclophosphamide q2w 400 mg for 3 months followed by q4w 400 mg for 3 months	30 months, dead (rupture of recurrent pseudoaneurysm at proximal aortic anastomosis)
2	M, 29	O, G, E1 day Diagnosed prior to the initial open surgery	105	421	Right renal artery pseudoaneurysm	Sudden tearing abdominal pain 15 days prior, followed by continuous dull pain	4 days	Prosthesis bypass from the aorta to the SMA, bilateral renal arteries, and bilateral CIA with aneurysmectomy	Pre-operative prednisone 40 mg once Post-operative prednisone qd 40 mg for 12 months followed by sequential reduction to drug withdrawal	58 months, alive (normal ESR and CRP level)
3	M, 45	O, E, S Diagnosed more than 6 years prior to the initial EVAR	-	-	-	Continuous abdominal pain combined with pulsatile abdominal mass for 1 month, acute enlargement of the mass for 1 day	3 hours	Infra-renal EVAR Recurrence at the proximal end of the endograft accompanied by right femoral anastomotic pseudoaneurysm 6 months after the initial EVAR, patient refused further intervention for aortic recurrence	Pre-operative prednisone for more than 6 years Post-operative prednisone qd 30 mg and azathioprine qd 100 mg for 7 months	7 months, dead (rupture of recurrent pseudoaneurysm at proximal aortic landing zone)
4	M, 33	O, G, S Diagnosed more than 3 years prior to the initial EVAR	10	62.8	Left subclavian aneurysm, Left renal arterial occlusion	Continuous dull epigastric pain for 1 month	6 days	EVAR with right renal artery and SMA chimney stenting Technical failure due to type I endoleakage	Pre-operative prednisone qd 40 mg, Cyclophosphamide q2w 400 mg for 36 months, prednisone qd 60 mg 1 month before operation and continued post-operatively	4 months, dead (rupture of aneurysm due to type Ia endoleak)
5	M, 28	O, G, S Diagnosed approximately 1 year prior to the first recurrence	26	19.6	Right renal artery stenosis	Continuous abdominal and back pain for 3 months	6 days	Infra-renal EVAR (Zenith, 22 mm/80 mm) Recurrent pseudoaneurysm at the distal end of the endograft, followed by another infra-renal EVAR (Aorto-bi-iliac) 80 months after the initial EVAR Recurrent pseudoaneurysm at the proximal end of the endograft above the right renal artery 13 months after the second EVAR; the size was stable based on close follow-up	Prednisone qd 30 mg for 12 months before the first recurrence, continued qd 30 mg post-operatively for another 12 months, qd 15 mg for half a year and qd 10 mg as a maintenance treatment	163 months, alive (regular hemodialysis owing to chronic kidney failure for 65 months)
6	M, 56	O, G, S Diagnosed after the initial EVAR	72	176	Left CIA pseudoaneurysm	Continuous pain in back and lower extremities for 2 months	2 days	Infra-renal EVAR (Medtronic, 25-14 mm /100 mm) combining artificial bypass from the right CFA to the left CFA and plugging of the left CIARupture of recurrent aneurysm 50 months after the initial operation	Prednisone qd 50 mg for 6 months followed by sequential reduction by 5 mg every two weeks after EVAR	50 months, dead (sudden death, rupture of recurrent aneurysm)
7	M, 39	O, G, E Diagnosed after the initial EVAR	45	24.2	-	Back pain for half a year and pulsatile abdominal mass for 20 days	5 days	Infra-renal EVAR (Hercules, 20-20 mm/60 mm) Recurrent pseudoaneurysm at the distal end of the endograft, followed by another infra-renal EVAR(Aorto-bi-iliac) 73 months after the initial EVAR	Prednisone and azathioprine for approximately 3 years after the first EVAR, leflunomide qd 20 mg for 12 months after the second EVAR	108 months, alive (normal ESR of 8 mm/h and CRP level of 6.29 mg/L)
8	M, 35	O, G, P Diagnosed after the initial EVAR	30	66.8	Right CIA occlusion, SMA aneurysm	Intermittent epigastric pain for more than 1 year	2 days	Juxta-renal EVAR (Sinus XL. 22 mm/80 mm, 24 mm/80 mm) combining coil embolization and PTA stenting for the R-CIA	Prednisone qd 50 mg for 6 months followed by sequential reduction by 5 mg every two weeks, Cyclophosphamide q2w 400 mg for 16 months after the initial intervention	16 months, alive (normal ESR of 3 mm/h and slightly elevated CRP level of 11.20 mg/L, new aneurysm at the proximal landing zone of the bare-metal iliac stent)
9	M, 42	O, G, S Diagnosed 3 years prior to the initial EVAR	98	285	Coronary artery circumflex aneurysm, deep venous thrombosis	Intermittent thoracic pain for more than 2 years and abdominal pain for 3 months	1 day	TEVAR (Hercules, 26 mm/80 mm) and infra-renal EVAR (Hercules, 18 mm/80 mm)	Pre-operative prednisone qd 50 mg for 10 months followed by 25 mg for 12 months, 15 mg for 3 months and 7.5 mg for 11 months, as well as Cyclophosphamide q2w 400 mg for 24 monthsPost-operative adalimumab q2w 40 mg combined with prednisone qd 5 mg for 12 months followed by prednisone qd 20 mg and azathioprine 150 mg for 4 months	16 months, alive (normal ESR of 10 mm/h and CRP level of 1.50 mg/L)
10	M, 46	O, G, E Diagnosed after the initial EVAR	74	88	-	Abdominal and back pain for over 10 days, acute deterioration for 12 hours	1 hour	Infra-renal EVAR (Hercules, 18 mm/80 mm)	Post-operative prednisone 30 mg qd for 3 months, and increase to 50 mg up to present, azathioprine 100 mg bid up to present	9 months, alive (normal ESR of 13 mm/h and CRP level of 3.32 mg/L)

Abbreviations: O: ocular lesions; S: skin lesions; G: genital ulceration; E: uveitis; P: positive pathergy test result.