Abstract / Summary
Background Behçet's syndrome with femoral artery pseudoaneurysm (BS–FAP) is exceedingly rare, and its long-term postoperative recurrence profile remains undefined. We aimed to ascertain event-free survival (EFS) and examine associations of immunosuppressive adherence and five inflammatory biomarkers ( N LR, PLR, SII, SIRI, IBI) with relapse. External validation was performed via systematic review and individual patient data (IPD) meta-analysis. Methods This retrospective study included 13 BS–FAP patients who underwent femoral artery surgery at Peking Union Medical College Hospital (2002–2022). The primary endpoint was EFS, a composite of same-site recurrence, de novo aneurysm, re-intervention, death, or amputation. Five inflammatory indices were derived from preoperative blood counts. EFS was estimated using Kaplan–Meier with log-rank comparisons. A systematic literature search was conducted for descriptive IPD meta-analysis. Results The cohort (median age 34 years, 85% male) underwent endovascular ( n = 6), open ( n = 6), or hybrid ( n = 1) repair. Median follow-up was 60 months (all ≥36 months). Five-year EFS was 69.2% in primary analysis and 76.9% in sensitivity analysis. Endovascular and open approaches showed comparable EFS ( p = 0.992). Elevated NLR, PLR, SIRI, and IBI were associated with lower 5-year EFS (50.0% vs. 85.7%), with SIRI showing the strongest trend ( p = 0.131); SII ( p = 0.787) and ESR ( p = 0.994) showed no trend. Three patients (23.1%) developed de novo aneurysms beyond 5 years, all after immunosuppressive discontinuation. In a supplementary four-level adherence analysis, no patient was fully adherent perioperatively; preoperative-only use ( n = 2) showed the highest disease-specific 5-year EFS (100%). The cohort showed mucocutaneous predominance (oral ulcers 92.3%) and venous involvement in 38.5%. IPD meta-analysis (40 individual cases + 12 series) confirmed a temporal mortality decline from 26%–40% (early open) to 0%–5.8% (contemporary endovascular). Published case reports (median follow-up 6 months) reported 11% recurrence, vs. 31% in our 60-month cohort, confirming systematic underestimation. Conclusion The recurrence burden after BS–FAP surgery is systematically underestimated in short-follow-up literature; surveillance should extend beyond 5 years. Immunosuppressive adherence is a modifiable target for recurrence control. SIRI emerges as the most promising biomarker for risk stratification. Endovascular and open repair offer comparable long-term EFS. This exploratory study supports a comprehensive pathway of “surgical intervention—uninterrupted immunosuppression—extended surveillance,” with adherence incorporated into quality metrics.