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Periodic Fever Syndromes, Autoinflammatory Diseases, Still’s Disease and MAS/HLH

Poster Session C

Session: (2015–2051) Miscellaneous Rheumatic & Inflammatory Diseases Poster III

2028: Behçet’s Syndrome Beyond Endemic Borders: A 24-Year Longitudinal Analysis of Mortality in a Diverse South American Cohort

Tuesday, October 28, 2025

10:30 AM - 12:30 PM Central Time

Location: Hall F1

Abstract Poster Presenter(s)

FS

Fabio Specian, Sr, MD

Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP)
São Paulo, S�o Paulo, Brazil

Disclosure(s): No financial relationships with ineligible companies to disclose

Background/Purpose: Behçet’s syndrome (BS) is a chronic, relapsing vasculitis that contributes substantially to morbidity and mortality, primarily due to vascular and neurological complications. Most of the available mortality data in BS derive from studies conducted in endemic regions. This study aims to evaluate long-term mortality in a multiethnic South American cohort of BS patients.

Methods: This research is a retrospective analysis of a prospective, single-center and multiethnic cohort of BS patients followed between January 2000 and December 2024. We included BS patients diagnosed according to the International Study Group and/or the International Criteria for Behçet's Disease. Mortality was analyzed according to qualitative and quantitative variables using descriptive statistics. Associations with mortality during follow-up were assessed through bivariate Cox regression, with estimation of unadjusted hazard ratios (HRs) and 95% confidence intervals.

Results: In our cohort of 303 patients, the median follow-up time was 117 months (IQR: 55–184 months); 186(61.6%) were female. Eight (2.6%) patients died during the study period. The mean age at BS diagnosis was 31.5(±10.4) in survivors and 33.4(±8) in non-survivors (p=0.662). The mean age at death among the eight patients was 47.6 years (±14.1), and the mean interval between BS diagnosis and death was 14.6 years (±10.3). Causes of death included three cases of malignancy, one of undetermined cause, and four attributed to disease activity. The presence of pulmonary artery aneurysm (HR=28.4; 95% CI: 3.32–243.53; p=0.002), parenchymal central nervous system involvement (HR=6.81; 95% CI: 1.65-28.06; p=0.008), previous use of cyclophosphamide (HR=20.4; 95% CI: 2.38-175; p=0.006) and infliximab (HR=4.87; 95% CI: 1.16-20.44; p=0.031), type 2 diabetes mellitus (DM2) (HR=6.72; 95% CI: 1.67-27.03; p=0.007) and cancer (HR=8.81; 95% CI: 2.02-38.5; p=0.004) were associated with increased mortality risk. Colchicine use was associated with a significantly reduced risk of mortality (HR=0.08; 95% CI: 0.02-0.36; p=0.001). Given the low mortality, only exploratory (unadjusted) analysis of factors associated with death were performed.

Conclusion: Although mortality was low, the relatively large number of patients for a rare disease, the long-term follow-up, and the inclusion of multiple clinical variables provided meaningful insights into mortality risk factors in our cohort. Consistent with the current literature, pulmonary artery aneurysm and central nervous system involvement were strongly associated with increased mortality. The observed association between cyclophosphamide and infliximab use likely reflects treatment of more severe disease. The apparent protective effect of colchicine may be influenced by indication bias, as this drug is frequently used in milder cases. Nonetheless, its known benefits in cardiovascular prevention raise the hypothesis that colchicine could have a broader impact on mortality reduction in Behçet’s syndrome, warranting further investigation.