scholarly journals AB0482 INFLUENCES OF TIME OF INTRODUCTION OF INFLIXIMAB ON THE FUNCTIONAL DISABILITY AND JOB STATUS OF PATIENTS WITH CHRONIC PROGRESSIVE NEURO-BEHCET’S DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1539.1-1539
Author(s):  
S. Hirohata ◽  
H. Kikuchi ◽  
T. Sawada ◽  
M. Kuwana ◽  
Y. Kirino ◽  
...  

Background:Chronic progressive neuro-Behcet’s disease (CPNBD) is characterized by progressive neurobehaviour changes leading to disability and death. It has been appreciated that methotrexate is effective for CPNBD. Notably, recent studies have demonstrated that infliximab is effective for patients with CPNBD who had inadequate responses to methotrexate. However, the appropriate timing for introduction of infliximab remains unclear.Objectives:The current studies examined the effects of intervals before introduction of infliximab on the functional disability and job status of patients with CPNBD.Methods:Eleven patients (8 males, 3 females, ages 35.2±9.3 [mean±SD]), who met the international classification criteria for BD with CPNBD and received infliximab, were retrospectively followed up. The functional disability of the patients was evaluated by Steinbrocker functional classification as is used in rheumatoid arthritis. Correlation between the patients’ functional outcome and the intervals before the introduction of infliximab was analyzed by Spearman’s rank correlation test.Results:All the 11 patients had received methotrexate prior to infliximab. The intervals from the onset to the introduction of infliximab and the follow-up periods were 26.6±35.1 months and 65.2±43.6 months [mean±SD], respectively. Among the 11 patients, 9 patients did not show progression after the introduction of infliximab, whereas 2 patients still progressed and lost job. In the latter 2 patients, infliximab had been discontinued before the final follow-up. No patients improved from the functional disability or gained job even after infliximab treatment. The functional disability grades of the patients after the introduction of infliximab were significantly correlated with the intervals from the onset of CPNBD to the introduction of infliximab (r=0.6177, p=0.0476).Conclusion:The results indicate that the delay of the introduction of infliximab leads to the irreversible functional disability and job loss of the patients with CPNBD. Thus, it is recommended that infliximab should be administered as soon as possible for the patients with CPNBD with inadequate response to methotrexate.References:[1]Kikuchi H, Aramaki K, Hirohata S. Effect of infliximab in progressive Neuro-Behcet’s syndrome.J Neurol Sci2008; 272: 99-105Disclosure of Interests: :Shunsei Hirohata Speakers bureau: Tanabe Mitsubishi, Hirotoshi Kikuchi Speakers bureau: Tanabe Mitsubishi, Tetsuji Sawada: None declared, Masataka Kuwana Grant/research support from: Acetelion, Consultant of: Acetelion, Bayer, Chugai, Corbus Pharmaceuticals, CSL Behring and Reata Pharmaceuticals. He was a member of the SENSCIS trial Steering Committee (Boehringer Ingelheim), Yohei Kirino: None declared, Mitsuhiro Takeno Speakers bureau: Esai, Tanabe-Mitsubishi – speaker; Celgene Corporation – advisory board, Yoshiaki Ishigatsubo: None declared

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1562.1-1563
Author(s):  
L. Sun ◽  
J. Liu ◽  
W. Zheng

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


Vascular ◽  
2020 ◽  
Vol 28 (6) ◽  
pp. 829-833
Author(s):  
Demet Yalçın Kehribar ◽  
Metin Ozgen

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.


2020 ◽  
Vol 30 (6) ◽  
pp. 943-944
Author(s):  
Xiaobing Li ◽  
Xian Fan ◽  
Li Shen ◽  
Rufang Zhang

Abstract Pulmonary artery (PA) aneurysm is a very rare complication of Behcet’s disease. We report on a 14-year-old boy with a giant left distal PA aneurysm caused by Behcet’s disease. A left thoracotomy was first performed to separate the aneurysm, but it was interrupted due to continuous and massive tracheorrhagia. We immediately converted to a median sternotomy and established cardiopulmonary bypass (CPB). The patient’s condition was stable; aneurysmectomy and left-down lobectomy were successfully performed. Results of the 2-year follow-up were favourable. Based on our experience, we recommend selecting CPB when performing surgery on patients with PAA, especially those with Behcet’s disease.


2013 ◽  
Vol 35 (2) ◽  
pp. 213.e9-213.e11 ◽  
Author(s):  
Domenico De Berardis ◽  
Nicola Serroni ◽  
Daniela Campanella ◽  
Luigi Olivieri ◽  
Stefano Marini ◽  
...  

2005 ◽  
Vol 32 (5) ◽  
pp. 365-370 ◽  
Author(s):  
Aydın Yücel ◽  
Selma Sönmezoğlu Maraklı ◽  
Varol Lütfü Aksungur ◽  
Soner Uzun ◽  
Yaşar Sertdemir ◽  
...  

2013 ◽  
Vol 107 (3) ◽  
pp. 466-471 ◽  
Author(s):  
Gulden Bilgin ◽  
Gulten Sungur ◽  
Vildan Kucukterzi

Vascular ◽  
2016 ◽  
Vol 24 (5) ◽  
pp. 454-460
Author(s):  
Mohamed A Elsharawy ◽  
Aymen Elsaid ◽  
Bander Al-Dhafery ◽  
Ibrahim Alghnimi ◽  
Fatimah Almabyouq

Objective Aneurysms in Behcet’s disease are rare, serious, and recurrent. To achieve durable treatment, patients should receive immunosuppressive therapy before intervention to induce remission. We present early and long-term results of emergency cases of active Behcet’s disease, which did not permit waiting for suppressive treatment. Patients and methods The study was undertaken on all cases admitted to the vascular unit, King Fahd Hospital of University for aneurysm treatment in patients with active Behcet’s disease over about 10 years. All patients had exclusion of the aneurysm either by open surgery or endovascular intervention. Morbidities and mortality were recorded within the hospital admission and on the follow-up. Results During the study period, three cases were included. All interventions were successful and lifesaving. However, two cases, treated with surgical interposition grafts, were blocked in the intermediate term follow-up (2–12 months) and one case, treated with endovascular treatment, complicated with pseudoaneurysm at femoral puncture site after six months. Conclusion Although early results were good, intermediate ones were not satisfactory because of progressive graft thrombosis and formation of new aneurysms. Awareness of these rare cases help for early identification and proper immunosuppressive before emergency vascular intervention is warranted.


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