A case of Behçet’s disease associated with aortic regurgitation and nephrotic syndrome

1997 ◽  
Vol 7 (3) ◽  
pp. 189-196
Author(s):  
Yuji Akiyama ◽  
Masahiko Tanaka ◽  
Fumihiko Imai ◽  
Shuji Ohno ◽  
Kosei Kobayashi ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Aortic Regurgitation ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behcet's Disease

A case of Behçet’s disease associated with aortic regurgitation and nephrotic syndrome

1997 ◽  
Vol 7 (3) ◽  
pp. 189-196
Author(s):  
Yuji Akiyama ◽  
Masahiko Tanaka ◽  
Fumihiko Imai ◽  
Shuji Ohno ◽  
Kosei Kobayashi ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Aortic Regurgitation ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behcet's Disease

scholarly journals AB0531 GOLIMUMAB IN THE TREATMENT OF SEVERE AND/OR REFRACTORY VASCULO-BEHÇET’S DISEASE: A SINGLE-CENTRE EXPERIENCE IN CHINA

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1562.1-1563
Author(s):  
L. Sun ◽  
J. Liu ◽  
W. Zheng
Keyword(s):  
Aortic Regurgitation ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
High Dose ◽  
Efficacy And Safety ◽  
Behcet's Disease ◽  
Severe Aortic Regurgitation ◽  
Tnf Α

Background:Vascular involvement is one of the leading causes of mortality and morbidity in Behcet’s Disease (BD)1. Surgical treatments are difficult for Vaculo-BD (VBD) patients due to the high risk of serious postoperative complications without effective and promptly perioperative immunotherapy2, 3. Anti-tumor necrosis factor alpha (TNF-α) therapy has been reported as a potential treatment in severe VBD, e.g. infliximab (IFX) and adalimumab (ADA). However, only few case reports are available regarding the fully humanized monoclonal antibody to TNF-α, golimumab (GOL), in the management of VBD4.Objectives:The objective of this study was to report the efficacy and safety of GOL for the treatment of severe and/or refractory VBD.Methods:We retrospectively analyzed the efficacy and safety profile of patients with severe and/or refractory VBD treated with GOL in our medical center between 2018 to 2020.Results:Nine VBD patients (8 male and 1 female) were enrolled, with a mean age and median course of 37±8.6 years and 72 months (range 12 to 300), respectively. Cardiac involvements (severe aortic regurgitation secondary to BD) were presented in 7 patients, including 2 patients with post-operative paravalvular leakage (PVL) after aortic valve replacement surgery. Multiple vascular lesions were documented in the other 2 patients, including one patient with life-threatening multiple pulmonary aneurysms, pulmonary thromboembolism and recurrent deep vein thrombosis, and another patient with abdominal aortic pseudoaneurysm and multiple artery stenosis and occlusion. Prior to GOL therapy, all patients experienced disease progression despite high-dose glucocorticoids combined with multiple immunosuppressants. Moreover, seven patients required effective and fast control of inflammation and a decrease of glucocorticoid dose during the perioperative period. They were treated with GOL, 50mg every 4 weeks, in combination with background low-or medium-dose glucocorticoids and immunosuppressants, for a median of 6 (range 3-15) months. After a mean duration of follow-up of 10 (range 2-6) months, all patients achieved improvement both in clinical symptoms and serum inflammation markers. The ESR level [4.88±4.94 mm/h vs 31.13±31.78mm/h, P<0.01] and CRP level [1.9 (0.11-3.73)mg/L vs 24.3 (0.4-85.57)mg/L, P<0.01] significantly decreased. The dosage of glucocorticoid[10 (0-15) vs 40 (0-100)mg/d, P<0.01] effectively tapered, indicating a potential steroid-sparing effect. No newly-onset aneurysm and recurrent venous thrombosis were observed. Also, one patient had a marked reduction in size and number of pulmonary aneurysms. No post-operative PVL was observed in the five patients after Bentall operation with a median follow-up of 10 months. One patient with severe aortic regurgitation remained stable and without surgical intervention with the treatment of GOL for 16 months. No severe complication occurred in one patient after underwent endovascular repair of abdominal aorta for 8 months. GOL was well-tolerated, and no serious adverse event was observed.Conclusion:Our results suggested that GOL is safe and effective for the treatment of patients with severe and / or refractory VBD. Further controlled studies are warranted to confirm the therapeutic potential of GOL in VBD patients.Disclosure of Interests:None declared

scholarly journals AB0363 PERIOPERATIVE MANAGEMENT WITH BIOLOGICS ON SEVERE AORTIC VALVE REGURGITATION CAUSED BY BEHÇET’S SYNDROME: THE EXPERIENCE FROM A SINGLE CENTER

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1207.1-1207
Author(s):  
L. Sun ◽  
J. Liu ◽  
W. Zheng
Keyword(s):  
Aortic Valve ◽  
Aortic Regurgitation ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Perioperative Management ◽  
Behçet's Disease ◽  
Valve Regurgitation ◽  
Behcet's Disease ◽  
Paravalvular Leakage ◽  
Median Interval

Background:Cardiovascular involvement in Behçet’s syndrome (BS) is associated with poor prognosis and accounts for the leading cause of mortality and morbidity in BS1, 2. Perioperative management of severe aortic valve regurgitation (AR) caused by BS is critical while challenging. AR caused by BS is associated with a high incidence of severe postoperative complications, especially paravalvular leakage (PVL) due to the fragilities of aortic lesions and tissue inflammation, leading to a significantly increased risk of re-operation and life-threatening conditions3-5. For the perioperative management of patients with AR caused by BS, many patients respond inadequately to the combination therapies of glucocorticoids (GCs) and immunosuppressants. Meanwhile, rapid and efficient control of inflammation is critical in preserving cardiac function and surgical management. However, the application of biologics in the perioperative treatment of AR attributable to BS has not yet been evaluated.Objectives:To investigate the efficacy and safety of biologics in the perioperative management of severe AR caused by BS.Methods:We retrospectively analyzed twenty patients with severe AR caused by BS and were treated with biologics during the perioperative cardiac surgeries in our center between February 2016 and October 2020.Results:Twenty patients with severe AR were enrolled, including 19 males and 1 female, with a mean age of 39.1±8.8 years and median course 8 (IQR, 5.25-10) years. Before biologics administration, 92.9% of the patients who underwent aortic valve replacement surgeries had failed conventional therapy and developed postoperative paravalvular leakage (PVL) at a median interval of 4 months. Biologics was administered during the perioperative period of 22 aortic valve surgeries, including preoperatively with a median interval of 3.5 (IQR, 2.75-4.25) months in 13 cases, or within three-month postoperatively in 9 cases, with background glucocorticoids (GCs) and immunosuppressants. After a median follow-up of 21 (IQR, 15-32) months, 11 out of 13 cases (84.6%) preoperatively and 8 out of 9 cases (88.9%) postoperatively treated with biologics were event-free. The BDCAF score improved significantly (7 vs. 0, median, p<0.0001). Significant decrease of ESR (25.0 (IQR, 11-36.25) mm/h vs. 6.5 (IQR, 4-8.8) mm/h, p<0.001), and CRP (20.77 (IQR, 7.19-29.58) mg/L vs. 1.53 (IQR, 0.94-2.92) mg/L, p=0.001) were achieved rapidly and effectively. The dosage of GCs 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 was 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 a favorable GCs- and immunosuppressants- sparing effect.References:[1]Saadoun D, Wechsler B, Desseaux K, et al. Mortality in Behcet’s disease. Arthritis Rheum 2010; 62: 2806-2812.[2]Thomas T, Chandan JS, Subramanian A, et al. Epidemiology, morbidity and mortality in Behcet’s disease: a cohort study using The Health Improvement Network (THIN). Rheumatology (Oxford) 2020; 59: 2785-2795.[3]Ando M, Kosakai Y, Okita Y, et al. Surgical treatment of Behcet’s disease involving aortic regurgitation. Ann Thorac Surg 1999; 68: 2136-2140.[4]Han JK, Kim HK, Kim YJ, et al. Behcet’s disease as a frequently unrecognized cause of aortic regurgitation: suggestive and misleading echocardiography findings. J Am Soc Echocardiogr 2009; 22: 1269-1274.[5]Guo X, Tian Z, Liu Y, et al. Preoperative immunosuppressive therapy reduces paravalvular leakage after aortic valve surgery in patients with aortic regurgitation attributable to Behcet’s disease. Clin Exp Rheumatol 2016; 34: S26-S33.Disclosure of Interests:None declared

scholarly journals Surgical Outcomes in Behcet’s Disease Patients With Severe Aortic Regurgitation

2019 ◽  
Vol 107 (4) ◽  
pp. 1188-1194 ◽  
Author(s):  
Byeongzu Ghang ◽  
Joon Bum Kim ◽  
Sung-Ho Jung ◽  
Cheol Hyun Chung ◽  
Jae Won Lee ◽  
...  
Keyword(s):  
Aortic Regurgitation ◽  
Behçet’S Disease ◽  
Surgical Outcomes ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behcet's Disease ◽  
Severe Aortic Regurgitation

scholarly journals Aortic Regurgitation Presenting with Recurrent Detachment of a Prosthetic Valve, as the First Presenting Symptom of Cardiovascular Behçet's Disease

2018 ◽  
Vol 57 (6) ◽  
pp. 823-827 ◽  
Author(s):  
Tsugumi Takayama ◽  
Takuya Ozawa ◽  
Akiko Sanada ◽  
Tohru Watanabe ◽  
Masahiro Ito ◽  
...  
Keyword(s):  
Aortic Regurgitation ◽  
Behçet’S Disease ◽  
Prosthetic Valve ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behcet's Disease

Valved conduit operation for aortic regurgitation associated with Behçet’s disease

2000 ◽  
Vol 48 (7) ◽  
pp. 424-427 ◽  
Author(s):  
Motomi Ando ◽  
Yosikado Sasako ◽  
Yutaka Okita ◽  
Osamu Tagusari ◽  
Soichiro Kitamura
Keyword(s):  
Aortic Regurgitation ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Behcet's Disease ◽  
Valved Conduit

Aortic Regurgitation Caused by Behçet's Disease: Surgical Experience during an 11-Year Period

2012 ◽  
Vol 27 (1) ◽  
pp. 39-44 ◽  
Author(s):  
Wei-Guo Ma ◽  
Jun Zheng ◽  
Jun-Ming Zhu ◽  
Yong-Min Liu ◽  
Ming Li ◽  
...  
Keyword(s):  
Aortic Regurgitation ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Surgical Experience ◽  
Behcet's Disease
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