Tocilizumab and Tofacitinib in the Treatment of Vascular Behçet's Syndrome

Inclusion Criteria:

1． Understand and voluntarily sign an informed consent form prior to any study-related assessments/procedures being conducted.

2. Male and female subjects aged 18-65 years.

3． Fulfill the 2013 International Classification Criteria for Behcet's Disease (ICBD).

4． Patients with aneurysmal dilatation/aneurysm of the descending aorta and/or peripheral arteries confirmed by ultrasonography and/or computed tomography angiography (CTA).

5. Elevated acute phase reactants ESR and hs-CRP.

Exclusion Criteria:

1. Cardiovascular manifestations that cannot be distinguished from giant cellarteritis, Burger's disease, or atherosclerotic aneurysm; infectious aneurysm;

2. Other active organ involvement related to BS that requires intensifiedimmunosuppressive treatment, including gastrointestinal ulcers, uveitis, andparenchymal neurological involvement;

3. Patients with severe aneurysms requiring emergency intervention surgery; patientswith elective surgery indications require the consensus between rheumatologists andvascular surgeons to determine inclusion or exclusion.

4. Severe organ dysfunction, including ALT, AST, and TBIL exceeding the upper limit ofnormal by more than 2 times, serum creatinine ≥ 133 mmol/L, white blood cell count < 3×10^9/L, ANC < 2×10^9/L, hemoglobin < 80g/L, platelet count < 100×10^9/L;

5. Active infection such as active tuberculosis, hepatitis B or C, syphilis, chronicEBV infection, persistent or severe bacterial or viral infection;

6. Primary or secondary immunodeficiency;

7. Malignant tumor;

8. Use of immunosuppressants such as Cyclosporin A (CsA), Azathioprine (AZA),Tacrolimus (TAC), Mycophenolate Mofetil (MMF), or Cyclophosphamide (CTX) within 1month;

9. Use of biologics/small molecule drugs within 5 half-lives (baricitinib within 10days; etanercept within 4 weeks; infliximab within 8 weeks; adalimumab, golimumab,ustekinumab, and abatacept within 10 weeks, secukinumab within 6 months, andpreviously use of tocilizumab and tofacitinib);

10. Pregnant, lactating, or planning a recent pregnancy;

11. Subjects who do not agree to or are unable to comply with regular visits.