Sacroiliitis in Behçet Syndrome

2013 ◽  
Vol 19 (6) ◽  
pp. 356 ◽  
Author(s):  
Clarissa Canella ◽  
Flavia Costa ◽  
Ana Beatriz Santos Bacchiega ◽  
Edson Marchiori
2021 ◽  
Vol 12 ◽  
pp. 204062232110267
Author(s):  
Luxi Sun ◽  
Jinjing Liu ◽  
Xiufeng Jin ◽  
Zhimian Wang ◽  
Lu Li ◽  
...  

Background: To investigate the efficacy and safety of biologics in the perioperative management of severe aortic valve regurgitation (AR) caused by Behçet syndrome (BS). Methods: We retrospectively analyzed 20 patients with severe AR caused by BS who were all treated with biologics during the perioperative period of cardiac surgeries in our center between February 2016 and October 2020. Results: A total of 20 patients with severe AR were enrolled, including 19 males and 1 female, with a mean age of 39.1 ± 8.8 years and a median course of 8 [interquartile range (IQR) 5.25–10.00] years. Before biologic administration, 92.9% of the patients who underwent aortic valve replacement had failed conventional therapy and developed postoperative paravalvular leakage (PVL) at a median interval of 4 months. Biologics were administered with background glucocorticoids (GCs) and immunosuppressants during the perioperative period for 22 aortic valve surgeries, including preoperatively with a median interval of 3.5 (IQR 2.75–4.25) months in 13 cases and within 3 months postoperatively in 9 cases. After a median follow up of 21 (IQR 15–32) months, 2 out of 13 cases (15.4%) preoperatively, and 1 out of 9 cases (11.1%) postoperatively treated with biologics developed PVL, and the rest were event free. The Behçet’s Disease Current Activity Form score improved significantly (7 versus 0, median, p < 0.0001). Decrease of erythrocyte sedimentation rate [25.0 (IQR 11.00–36.25) mm/h versus 6.5 (IQR 4.0–8.8) mm/h, p < 0.001], and C-reactive protein [20.77 (IQR 7.19–29.58) mg/l versus 1.53 (IQR 0.94–2.92) mg/l, p = 0.001] were achieved rapidly and effectively. The GC dosage tapered from 40 (IQR 30–60) mg/d to 10 (IQR 5–11.25) mg/d, p < 0.0001. Immunosuppressants were tapered in number and dosage in 6 (30%) and 20 patients (100%), respectively. No serious adverse event was observed. Conclusion: Our study suggests that biologics were effective and well tolerated for the perioperative management of severe and refractory AR caused by BS, which significantly reduced the occurrence of postoperative PVL and had favorable GC- and immunosuppressant-sparing effect.


2019 ◽  
pp. 191-199
Author(s):  
Farida Fortune ◽  
Gulen Hatemi

2014 ◽  
Vol 41 (4) ◽  
pp. 735-738 ◽  
Author(s):  
Vedat Hamuryudan ◽  
Gulen Hatemi ◽  
Koray Tascilar ◽  
Sebahattin Yurdakul ◽  
Cem Mat ◽  
...  

Objective.To test the hypothesis that colchicine use during early disease decreases immunosuppressive use in Behçet syndrome (BS) in the long term.Methods.Patients with BS who participated in a double-blind, placebo-controlled trial of colchicine 16.6 ± 1.1 years ago were evaluated for immunosuppressive use during the posttrial period.Results.We could contact 90/116 patients; 28 (31%) received immunosuppressives during the posttrial period, 14 being from the colchicine arm. Posttrial colchicine use and cumulative duration were similar between patients who received immunosuppressives and those who did not.Conclusion.Continuous use of colchicine, even when initiated at an early disease stage, does not seem to decrease the use of immunosuppressives in the long term.


2020 ◽  
Vol 13 (8) ◽  
pp. e235039
Author(s):  
Nathan R Stein ◽  
Satvik Ramakrishna ◽  
Yerem Yeghiazarians

A young man with a history of early-onset coronary disease presented with an ST-elevation myocardial infarction at the age of 38. He subsequently had recurrent in-stent restenosis requiring repeating interventions and ultimately bypass surgery. After 4 years, he presents with systemic symptoms, new skin lesions and a femoral artery pseudoaneurysm. He is diagnosed with Behçet syndrome, a rare systemic vasculitis characterised by the triad of oral aphthous ulcers, genital ulcers and ocular involvement. Behçet is not associated with premature coronary disease but can have a variety of cardiac complications. Additionally, pathergy, an exaggerated inflammatory response to local injury, is characteristic. We hypothesise that in retrospect, subclinical inflammation and a vascular pathergy likely predisposed him to his cardiac and vascular complications. Here, we review risk factors and presentation of premature coronary artery disease and review the literature on the cardiovascular complications of Behçet syndrome.


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