Prognostic analysis of Behçet’s disease with aortic regurgitation or involvement

Abstract Background Aortic regurgitation is the most common cardiovascular damage in Chinese patients with Behçet’s disease (BD) and is usually associated with aortic disease. These patients are easily misdiagnosed, and their prognosis is poor, even after surgical treatment. This study aimed to analyse potential factors that can improve the prognosis of BD patients with aortic regurgitation and/or aortic involvement. Methods Twenty-two patients with diagnosed or suspected BD as well as aortic regurgitation and/or aortic involvement in our hospital from 2012 through 2017 were collected in this study. Their clinical characteristics were listed, and the diagnosis of BD was evaluated by two different criteria sets. The influences of surgical treatment and immunosuppressive therapy (IST) on their prognosis were also explored. Results The diagnostic positive rate of the International Criteria for Behçet’s Disease was higher than that of the International Study Group criteria (kappa value 0.31, p < 0.05), indicating that the diagnostic consistency between the criteria sets was poor. There was no significant difference in survival between patients who had undergone ≤ 1 operation and those with ≥ 2 operations. Aortic valve replacement alone or in combination with aortic root replacement had no significant effect on the incidence of reoperation or death, but IST did significantly reduce this incidence (p < 0.05). However, there was no significant difference in the occurrence of reoperation or death between preoperative and postoperative IST versus postoperative IST only. Conclusion IST significantly improved the prognosis of BD patients with aortic regurgitation and/or aortic involvement.

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Long-Term Experience of Surgical Treatment for Aortic Regurgitation Attributable to Behçet's Disease

The Annals of Thoracic Surgery ◽

10.1016/j.athoracsur.2009.03.008 ◽

2009 ◽

Vol 87 (6) ◽

pp. 1775-1782 ◽

Cited By ~ 34

Author(s):

Dong Seop Jeong ◽

Kyung-Hwan Kim ◽

Jun Sung Kim ◽

Hyun Ahn

Keyword(s):

Surgical Treatment ◽

Aortic Regurgitation ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Behcet's Disease ◽

Long Term Experience

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Surgical treatment of behçet’s disease involving aortic regurgitation

The Annals of Thoracic Surgery ◽

10.1016/s0003-4975(99)00847-4 ◽

1999 ◽

Vol 68 (6) ◽

pp. 2136-2140 ◽

Cited By ~ 52

Author(s):

Motomi Ando ◽

Yoshio Kosakai ◽

Yutaka Okita ◽

Kiyoharu Nakano ◽

Soichiro Kitamura

Keyword(s):

Surgical Treatment ◽

Aortic Regurgitation ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Behcet's Disease

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Clinical Comparison of Behcet's Disease Between Japanese and Chinese Patients.

Nishi Nihon Hifuka ◽

10.2336/nishinihonhifu.59.745 ◽

1997 ◽

Vol 59 (5) ◽

pp. 745-748

Author(s):

Li-man WANG ◽

Satoru MINESHITA ◽

Jian-zhong WANG ◽

Su-xian DING ◽

Wei-yun LI ◽

...

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Chinese Patients ◽

Behcet's Disease ◽

Clinical Comparison

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

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.2272 ◽

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

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

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.1568 ◽

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

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Hyperhomocysteinaemia in Behçet's Disease

Biochemistry Research International ◽

10.1155/2010/361387 ◽

2010 ◽

Vol 2010 ◽

pp. 1-5 ◽

Cited By ~ 2

Author(s):

Amira Hamzaoui ◽

Olfa Harzallah ◽

Rim Klii ◽

Silvia Mahjoub

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Homocysteine Level ◽

Behçet's Disease ◽

Negative Control Group ◽

Negative Control ◽

Control Group ◽

Behcet's Disease ◽

Homocysteine Concentration ◽

Significant Difference

Objectives. The aim of this study was to investigate if hyperhomocysteinaemia is a contributive risk factor for the pathogenesis and the activity of Behçet's disease (BD).Design and Methods. Fifty four patients fullfiling the criteria of the International Study Group for BD were enrolled. Fifty healthy volunteers matched for age and sex with the BD group were included as a negative control group. Patients, with any condition that might affect plasma homocysteine concentration, were excluded.Results. Mean serum homocysteine concentration was significantly higher in patients with BD than in the healthy controls (), in patients with active disease (), and in masculine gender (). There was no significant difference between homocysteine level and clinical involvement.Conclusions. We demonstrated that plasma total homocysteine level (tHcy) is increased in BD and correlated with disease activity. No association was found between homocysteine levels and clinical involvement.

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Infliximab treatment in refractory vascular Behcet’s disease: A single-center experience

Vascular ◽

10.1177/1708538120927701 ◽

2020 ◽

Vol 28 (6) ◽

pp. 829-833

Author(s):

Demet Yalçın Kehribar ◽

Metin Ozgen

Keyword(s):

Behçet’S Disease ◽

Oral Anticoagulant ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Vascular Involvement ◽

Infliximab Treatment ◽

Single Center ◽

Vascular Events ◽

Behcet's Disease ◽

Significant Difference

Objective This study aims to investigate the efficacy and reliability of infliximab treatment in Behcet’s disease with vascular involvement. Methods This single-center retrospective study included a total of 18 patients diagnosed with Behcet’s disease with vascular involvement who were initiated infliximab treatment after exhibiting resistance to conventional immunosuppressive treatments. Results Seventeen patients achieved remission with infliximab treatment. While 18 patients were receiving a median of 50 (IQR: 20–61) mg/day equivalent of methylprednisolone before infliximab treatment, after infliximab treatment, only four patients were receiving 4 mg/day equivalent of methylprednisolone ( p < 0.001). Only 4 patients were receiving oral anticoagulant treatment during infliximab treatment, and compared to the patients who were not receiving oral anticoagulants, there was no significant difference between the two groups according to occurrence of new vascular events. Conclusion Infliximab seems to be an effective and reliable treatment in Behcet’s disease with vascular involvement and may also allow reduced dosage or even the discontinuation of corticosteroids. The results of our study suggest that oral anticoagulant use is unnecessary in Behcet’s disease with vascular involvement. However, further long-term randomized controlled studies are needed to investigate the length of infliximab regimen, whether or not it should be discontinued, and if so, whether or not immunosuppressants should be given as maintenance after discontinuation.

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