Despite celiprolol therapy, patients with vascular Ehlers-Danlos syndrome remain at risk of vascular events: A 12-year experience in an Italian referral center

被引:5
作者
Buso, Giacomo [1 ,2 ]
Paini, Anna [1 ]
Agabiti-Rosei, Claudia [1 ]
De Ciuceis, Carolina [1 ]
Bertacchini, Fabio [1 ]
Stassaldi, Deborah [1 ]
Salvetti, Massimo [1 ]
Ritelli, Marco [3 ]
Venturini, Marina [4 ]
Colombi, Marina [3 ]
Muiesan, Maria Lorenza [1 ,5 ]
机构
[1] Univ Brescia, Dept Clin & Expt Sci, Div Internal Med, ASST Spedali Civili Brescia, Brescia, Lombardy, Italy
[2] Univ Lausanne, Lausanne, Switzerland
[3] Univ Brescia, Dept Mol & Translat Med, Div Biol & Genet, Brescia, Italy
[4] Univ Brescia, Dept Clin & Expt Sci, Div Dermatol, ASST Spedali Civili Brescia, Brescia, Lombardy, Italy
[5] Univ Brescia, Dept Clin & Expt Sci, Div Internal Med, ASST Spedali Civili Brescia, C-O Spedali Civili Scala 6 Piano 5, I-25121 Brescia, Lombardy, Italy
关键词
arterial fragility; celiprolol; vascular Ehlers-Danlos syndrome; COL3A1; SYNDROME TYPE-IV; MANAGEMENT;
D O I
10.1177/1358863X231215330
中图分类号
R6 [外科学];
学科分类号
1002 ; 100210 ;
摘要
Background: Vascular Ehlers-Danlos syndrome (vEDS) is an inherited connective tissue disorder characterized by arterial fragility. Celiprolol has been suggested to significantly reduce rates of vascular events in this setting, though real-world evidence is limited. The aim of this study was to report our experience with celiprolol therapy in vEDS management. Methods: Patients with a genetically confirmed diagnosis of vEDS who were referred for outpatient consultation at the Brescia University Hospital between January 2011 and July 2023 were included. At each visit, patients' medical history, results of vascular imaging, and office blood pressure measurements were recorded. Celiprolol therapy was progressively titrated to the maximum tolerated dose of up to 400 mg daily, according to the patients' tolerance. Results: Overall, 26 patients were included. Female sex was prevalent (62%). Mean (SD) age was 37 (16) years. Follow-up duration was 72 (41) months. At the last follow-up visit, all patients were on celiprolol therapy, 80% of whom were taking the maximum recommended dose. The yearly risk of symptomatic vascular events was 8.8%, the majority of which occurred after reaching the maximum recommended dose of celiprolol. No significant predictor of symptomatic vascular events was identified among patients' clinical characteristics. Conclusion: In our cohort, rates of celiprolol use were high and the drug was well tolerated overall. Nonetheless, the risk of symptomatic vascular events remained nonnegligible. Future studies should identify reliable predictors of major adverse events and explore additional therapeutic strategies that could further lower the risk of life-threatening events in this population.
引用
收藏
页码:265 / 273
页数:9
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