scholarly journals Vascular involvement in Behçet’s syndrome: a retrospective analysis of associations and the time course

Rheumatology ◽  
2014 ◽  
Vol 53 (11) ◽  
pp. 2018-2022 ◽  
Author(s):  
Koray Tascilar ◽  
Melike Melikoglu ◽  
Serdal Ugurlu ◽  
Necdet Sut ◽  
Erkan Caglar ◽  
...  
Keyword(s):  
Retrospective Analysis ◽  
Time Course ◽  
Vascular Involvement ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

scholarly journals AB0532 CORRELATION WITH THE FREQUENCY OF DISEASE RELAPSES DURING THE FIRST 3 YEARS FROM THE DIAGNOSIS AND DISEASE OUTCOMES IN BEHCET’S SYNDROME

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1563.2-1563
Author(s):  
R. Talarico ◽  
A. Figliomeni ◽  
L. Mione ◽  
A. Parma ◽  
E. Cioffi ◽  
...  
Keyword(s):  
Disease Activity ◽  
Disease Onset ◽  
Disease Outcome ◽  
Vascular Involvement ◽  
Joint Involvement ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Disease Outcomes ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Background:Beside the organ involvement, a number of demographic factors could considerably influence the long-term and short-term outcomes of Behçet’s syndrome (BS): age at disease onset, duration of disease, gender and sex. Younger men patients are more suitable to have a more severe disease, due to an increased frequency both of morbidity and mortality, related to ocular,vascular and neurological involvementObjectives:The primary aims of the study were to evaluate disease activity in a cohort of BS patients consecutively followed in a BS clinic of a tertiary centre and to explore whether there is a correlation between frequency of relapses in the first 3 years of diseases and disease outcomes.Methods:One-hundred and sixty-five patients (91 males and 74 females; mean age 39±9 years, mean disease duration 9±5) with a diagnosis of BS according to the ISG criteria were studied. Disease activity has been evaluated by BDCAF and patients were also categorized in major or minor involvement of BS according or not to the presence of ocular, neurological and vascular involvement in the course of disease The numbers of relapses in the first 3 years from diagnosis were correlated with disease outcome and damage.Results:At time of the evaluation, 47% of BS patients presented an active disease; 69 patients presented muco-cutaneous involvement, 39 ocular disease, 21 joint involvement, 12 neurological impairment and 9 gastro-enteric involvement. Seventy-nine percent of patients presented in the course of the disease a severe BS involvement and the majority was represented by patients characterised by a more frequent relapse in the first 3 years of disease (M/F: 65/48, mean age 43±3 years). Those patients who experienced a more higher number of relapse in the first 3 years compared to the others were also characterised by poor disease outcomes and worse prognosis over time and this correlation was independent by the therapies taken.Conclusion:The high frequency of relapses during the first three years from diagnosis may be considered an important prognostic factor for disease outcome in BS patients, therefore could be taken into account as a useful element to tailor the management, not only according to the type and severity of symptoms and epidemiological factors.Acknowledgments:noneDisclosure of Interests:None declared

scholarly journals Cluster analysis of phenotypes of patients with Behçet’s syndrome: a large cohort study from a referral center in China

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Jun Zou ◽  
Jian-feng Luo ◽  
Yan Shen ◽  
Jian-fei Cai ◽  
Jian-long Guan
Keyword(s):  
Central Nervous System ◽  
Cluster Analysis ◽  
Nervous System ◽  
Central Nervous System Involvement ◽  
Vascular Involvement ◽  
Behçet’S Syndrome ◽  
System Involvement ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Introduction Behcet’s syndrome (BS) is a complex, heterogeneous disorder. However, classification of its subgroups is still debated. The purpose of this study was to investigate the clinical features and aggregation of patients with BS in China, based on manifestations and organ involvements. Methods This was a cross-sectional study of BS patients in Huadong Hospital of Fudan University between September 2012 and January 2020. We calculated relative risks (RRs) of clinical variables according to sex. Moreover, we conducted a hierarchical cluster analysis applied according to eighteen variables to determine subgroups of patients. Results A total of 860 BS patients were included. Male sex was associated with ocular involvement (RR 2.32, 95% CI 1.67, 3.22, P < 0.0001), vascular involvement (RR 2.00, 95% CI 1.23, 3.23, P = 0.004), cardiac lesion (RR 5.46, 95% CI 2.33, 12.77, P < 0.0001), and central nervous system involvement (RR 2.95, 95% CI 1.07, 6.78, P = 0.007) and was negatively associated with genital ulcers (RR 0.84, 95% CI 0.79, 0.91, P < 0.0001). Five clusters (C1–C5) were observed. C1 (n = 307) showed the skin and mucosa type. In C2 (n = 124), all had articular involvement, barely having major organ involvement except for 18 cases with intestinal lesions. In C3 (n = 156), the gastrointestinal type, 144 patients presented with intestinal involvement, and 36 patients with esophageal ulcers. In C4 (n = 142), all subjects presented with uveitis. C5 (n = 131) consisted of 44 patients with cardiac lesions, 58 with vascular involvement, and 26 cases having central nervous system involvement. Conclusion Our analysis confirmed sex differences in phenotypes of BS. Cluster analysis identified gastrointestinal, uveitis, and cardiovascular involvement cluster separately in different subsets, which represents the most commonly involved organs. Further research is required to replicate and clarify the patterns of phenotype in BS.

Huge Popliteal Arterial Aneurysms in Behçet's Syndrome: Is Ligation an Alternative Treatment?

Vascular ◽  
2007 ◽  
Vol 15 (1) ◽  
pp. 46-48 ◽  
Author(s):  
Mustafa Goz ◽  
Omer Cakir ◽  
M.Nesimi Eren
Keyword(s):  
Popliteal Artery ◽  
Arterial Occlusion ◽  
Artery Aneurysm ◽  
Vascular Involvement ◽  
Behçet’S Syndrome ◽  
Aneurysmal Dilatation ◽  
Behçet's Syndrome ◽  
Tibial Arteries ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Behçet's syndrome is a multisystemic disease characterized by relapsing uveitis, oral and genital ulcerations, and vascular system involvement. The vascular involvement is seen as venous occlusion, arterial occlusion, and aneurysm formation in this disease, and the surgical treatment of a Behçet's aneurysm has technical difficulties. In this report, we suggest that the huge popliteal artery aneurysm in Behçet's syndrome can be treated by ligation of the popliteal artery. A 58-year-old male patient was admitted to our clinic because of an infrapopliteal great mass at the left leg. Color Doppler ultrasonography and arteriography revealed a 71 × 54 mm aneurysmal dilatation at the distal popliteal artery. Surgery did not reveal any suitable arterial formation for bypass to the distal area of the popliteal artery and tibial arteries. For this reason, we applied ligation of aneurysmal dilatation at the distal popliteal artery. The patient tolerated the operation well and had no signs of ischemia during the postoperative period. In conclusion, especially in aneurysm of arteries such as the popliteal artery, which has critical importance for maintaining distal perfusion, ligation may be a treatment method if there are no other alternatives.

scholarly journals Pulmonary artery thrombosis as the first presentation of Behçet’s syndrome: a case report and review of the literature

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Ziyad Alakkas ◽  
Waad Kazi ◽  
Mohamed Mattar ◽  
Eman Abdul Wahhab Salem ◽  
Naglaa Fawzy Seleem
Keyword(s):  
Pulmonary Artery ◽  
Morbidity And Mortality ◽  
Vascular Involvement ◽  
Unknown Etiology ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Artery Thrombosis ◽  
Behcet’S Syndrome ◽  
Genital Ulcers ◽  
Behcet's Syndrome

Abstract Background Behçet’s syndrome is a type of systemic chronic vasculitis of unknown etiology, frequently characterized by recurrent oral and genital ulcers and uveitis. It is less commonly characterized by arthritis and skin, vascular, and gastrointestinal involvements. Behçet’s syndrome affects various sizes of vessels by perivascular infiltration and vasculitis. Unlike other classic types of vasculitis, Behçet’s syndrome patients can present with both arterial and venous involvement. Although vascular Behçet’s syndrome is found in only around 15% of Behçet’s syndrome patients, it is the major cause of morbidity and mortality among them. Furthermore, although deep venous thrombosis has high incidence in Behçet’s syndrome patients, pulmonary artery thrombosis is an uncommon complication. Combining the findings of this and previous case reports of pulmonary artery thrombosis in Behçet’s syndrome patients, we sought to determine the best treatment options for pulmonary artery thrombosis in Behçet’s syndrome patients. Case presentation We present the case of a 22-year-old Arabian male who was admitted to an emergency department with acute chest pain, dyspnea, and hemoptysis for 2 weeks. He gave a long history of recurrent oral and genital ulcers for the last 4 months but without seeking medical advice. Spiral computed tomography showed arterial filling defects with a pulmonary nodule for which the presence of a pulmonary artery aneurysm ruled out. The lung perfusion scan showed multiple pulmonary perfusion defects. After excluding common infectious diseases such as tuberculosis and brucellosis, a diagnosis of Behçet’s syndrome with pulmonary artery thrombosis was made. Steroids with enoxaparin were initiated. The patient was discharged later on prednisolone (tapering dose) with adalimumab and apixaban. He was on regular follow-up for the next 9 months. Conclusions Vascular involvement in Behçet’s syndrome is a major contributor to morbidity and mortality of Behçet’s syndrome patients. Consequently, early detection of vascular involvement has a major impact on the prognosis of patients with Behçet’s syndrome.

scholarly journals A Behçet’s Syndrome Case with an Unusual Vascular Involvement

2014 ◽  
Vol 16 (2) ◽  
pp. 118-120
Author(s):  
Nese Dursunoglu ◽  
Ali Ihsan Yildiz ◽  
Ali Kocyigit ◽  
Bedriye Atay Yayla ◽  
Zahide Alaçam ◽  
...  
Keyword(s):  
Vascular Involvement ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

AB0602 Anti-TNF Treatment for Refractory Vascular Involvement of BehÇEt's Syndrome: A Report of 16 Patients

2014 ◽  
Vol 73 (Suppl 2) ◽  
pp. 1005.1-1005 ◽  
Author(s):  
V. Hamuryudan ◽  
E. Seyahi ◽  
M. Melikoglu ◽  
S. Ugurlu ◽  
G. Hatemi ◽  
...  
Keyword(s):  
Vascular Involvement ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Behçet’s syndrome in Italy: a detailed retrospective analysis of 396 cases seen in 3 tertiary referral clinics

2020 ◽  
Vol 15 (6) ◽  
pp. 1031-1039 ◽  
Author(s):  
Jurgen Sota ◽  
Donato Rigante ◽  
Giacomo Emmi ◽  
Giuseppe Lopalco ◽  
Ida Orlando ◽  
...  
Keyword(s):  
Retrospective Analysis ◽  
Behçet’S Syndrome ◽  
Tertiary Referral ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

scholarly journals Cluster Analysis of Phenotypes of Patients with Behçet's Syndrome: A Large Cohort Study from a Referral Center in China

2020 ◽  
Author(s):  
Jun Zou ◽  
Jian-feng Luo ◽  
Yan Shen ◽  
Jian-fei Cai ◽  
Jian-long Guan
Keyword(s):  
Central Nervous System ◽  
Cluster Analysis ◽  
Nervous System ◽  
Central Nervous System Involvement ◽  
Vascular Involvement ◽  
Behçet’S Syndrome ◽  
System Involvement ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Abstract Introduction: Behcet’s syndrome (BS) is a complex, heterogeneous disorder. However, classification of its subgroups is still debated. The purpose of this study was to investigate the clinical features and aggregation of patients with BS in China, based on manifestations and organ involvements.Methods: This was a cross-sectional study of BS patients in Huadong Hospital of Fudan University between September 2012 and January 2020. We calculated relative risks (RRs) of clinical variables according to sex. Moreover, we conducted a hierarchical cluster analysis applied according to eighteen variables to determine subgroups of patients.Results: A total of 860 BS patients were included. Male sex was associated with ocular involvement (RR 2.32, 95% CI: 1.67, 3.22, P < 0.0001), vascular involvement (RR 2.00, 95% CI: 1.23, 3.23, P = 0.004), cardiac lesion (RR 5.46, 95% CI: 2.33, 12.77, P < 0.0001) and central nervous system involvement (RR 2.73, 95% CI: 1.10, 6.76, P = 0.025) and was negatively associated with genital ulcers (RR 0.84, 95% CI: 0.79, 0.91, P < 0.0001). Five clusters (C1–C5) were observed. C1 (n=307) showed the skin and mucosa type. In C2 (n=124), all had articular involvement, barely having major organ involvement except for 18 cases with intestinal lesions. In C3 (n=156), the gastrointestinal type, 144 patients presented with intestinal involvement, and 36 patients with esophageal ulcers. In C4 (n=142), all subjects presented with uveitis. C5 (n=131) consisted of 44 patients with cardiac lesions, 58 with vascular involvement and 26 cases having central nervous system involvement.Conclusion: Our analysis confirmed sex differences in phenotypes of BS. Cluster analysis identified gastrointestinal, uveitis and cardiovascular involvement cluster separately in different subsets,which represents the most commonly involved organs. Further research is required to replicate and clarify the patterns of phenotype in BS.

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