Analysis of vascular involvement in 460 patients with Behçet's syndrome: Clinical characteristics and associated factors

Abstract Background: Intestinal Behçet’s syndrome (BS) has high morbidity and mortality rates with serious complications. This study aimed to investigate the clinical characteristics and laboratory indicators of intestinal BS compared with mucocutaneous BS patients in China and analyzed the risk factors of intestinal complications in BS patients.Methods: A retrospective analysis was used to collect the demographic data and laboratory results from 97 patients newly diagnosed with intestinal BS and 154 patients newly diagnosed with mucocutaneous BS. Univariate and multivariate logistic regression analyses were used to analyze the demographic data and laboratory indexes whether a risk factor of intestinal involvement in BS patients.Results: The most common clinical manifestations of first onset in intestinal BS patients were oral ulceration (100.00%), followed by genital ulcers (62.89%) and erythema nodule (28.87%), gastrointestinal lesions (28.87%), pseudofolliculitis (25.77%), fever (17.53%), arthritis (16.49%), ocular involvement (5.15%), while the least common were vascular involvement (2.06%) and blood system involvement (2.06%). The most common intestinal segment involved in intestinal BS patients was terminal ileum (30.9%), followed by ileocecal (18.6%), colon (15.5%). Most intestinal BS patients (89.7%) used ≥ 3 immunosuppressants to control disease while most mucocutaneous BS patients (92.9%) used 1 or 2 immunosuppressant. By univariate logistic regression analysis, we found gender, age at hospitalization, age of disease onset, BDCAF, T-SPOT, fever, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), white blood cell (WBC), red blood cells (RBC), hemoglobin (HGB), neutrophil-to-lymphocyte ratio (NLR), serum amyloid A (SAA), complement 3 (C3), albumin, total cholesterol(TCH), high-density lipoprotein(HDL) and interleukin 6 (IL-6) were all risk factors of intestinal involvement in BS patients (P<0.05 or P=0.00). Additionally, gender (male), BDCAF (≥2), ESR (≥15mm/H), CRP (>10mg/L), HGB (<130g/L) and IL-6 (>7pg/ml) were the independent risk factors of intestinal involvement in BS patients (all P<0.05).Conclusions: More attention should be paid to gender, BDCAF, ESR, CRP, HGB and IL-6 of intestinal involvement in BS patients and therapeutic regiment should be adjusted timely to prevent the occurrence of serious clinical complication in BS patients.

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Distinct clinical characteristics of pediatric Behçet’s syndrome: A study from a referral center in China

Modern Rheumatology ◽

10.1080/14397595.2021.1891670 ◽

2021 ◽

pp. 1-16

Author(s):

Jun Zou ◽

Jian-feng Luo ◽

Yan Shen ◽

Jian-long Guan

Keyword(s):

Clinical Characteristics ◽

Behçet’S Syndrome ◽

Referral Center ◽

Behçet's Syndrome ◽

Behcet’S Syndrome ◽

Behcet's Syndrome

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

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.5004 ◽

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

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

Arthritis Research & Therapy ◽

10.1186/s13075-021-02429-7 ◽

2021 ◽

Vol 23 (1) ◽

Cited By ~ 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.

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The incidence, clinical characteristics, and outcome of COVID-19 in a prospectively followed cohort of patients with Behçet’s syndrome

Rheumatology International ◽

10.1007/s00296-021-05056-2 ◽

2021 ◽

Author(s):

Guzin Ozcifci ◽

Tahacan Aydin ◽

Zeynep Atli ◽

Ilker Inanc Balkan ◽

Fehmi Tabak ◽

...

Keyword(s):

Clinical Characteristics ◽

Behçet’S Syndrome ◽

Behçet's Syndrome ◽

Behcet’S Syndrome ◽

Behcet's Syndrome

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

Vascular ◽

10.2310/6670.2007.00010 ◽

2007 ◽

Vol 15 (1) ◽

pp. 46-48 ◽

Cited By ~ 10

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.

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

Journal of Medical Case Reports ◽

10.1186/s13256-021-02931-1 ◽

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.

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Clinical characteristics and risk factors of intestinal involvement in Behçet’s syndrome patients: a cross-sectional study from a single center

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-021-01772-x ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Cheng-cheng Hou ◽

Jing-fen Ye ◽

Hai-fen Ma ◽

Jian-long Guan

Keyword(s):

Risk Factors ◽

Logistic Regression ◽

Clinical Characteristics ◽

Behçet’S Syndrome ◽

Laboratory Parameters ◽

Behçet's Syndrome ◽

Amyloid A ◽

Intestinal Involvement ◽

Behcet’S Syndrome ◽

Behcet's Syndrome

Abstract Background Intestinal Behçet’s syndrome (BS) has high morbidity and mortality rates with serious complications. The purpose of this study was to investigate the clinical characteristics and laboratory parameters of intestinal and mucocutaneous BS patients and analyze the risk factors of intestinal involvement in BS patients. Methods A retrospective analysis was used to collect the demographic data and laboratory parameters from 97 intestinal and 154 mucocutaneous BS patients. Univariate and multivariate logistic regression analyses were used to investigate the risk factors of intestinal involvement in BS patients. Results The most common clinical manifestations of first onset in intestinal BS patients were oral ulceration (100.00%), followed by genital ulcers (62.89%) and erythema nodule (28.87%), gastrointestinal lesions (28.87%), pseudofolliculitis (25.77%), fever (17.53%), arthritis (16.49%), ocular involvement (5.15%), while the least common were vascular involvement (2.06%) and hematologic involvement involvement (2.06%). The most common intestinal segment involved in intestinal BS patients was terminal ileum (30.9%), followed by ileocecal (18.6%), colon (15.5%). By univariate logistic regression analysis, gender, age at hospitalization, age of disease onset, BDCAF, T-SPOT, fever, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), leukocyte, erythrocyte, hemoglobin (HGB), neutrophil-to-lymphocyte ratio, serum amyloid A, complement 3, albumin, total cholesterol, high-density lipoprotein and interleukin 6 (IL-6) were found all risk factors of intestinal involvement in BS patients (P < 0.05 or P = 0.00). Moreover, gender (male), BDCAF (≥ 2), ESR (≥ 15 mm/H), CRP (> 10 mg/L), HGB (< 130 g/L) and IL-6 (> 7 pg/ml) were found the independent risk factors of intestinal involvement in BS patients (all P < 0.05). Conclusions More attention shall be paid to gender, BDCAF, ESR, CRP, HGB and IL-6 in BS patients. When gender (male), BDCAF (≥ 2), ESR (≥ 15 mm/H), CRP (> 10 mg/L), HGB (< 130 g/L) and IL-6 (> 7 pg/ml) being observed, it may reminds that the presence of intestinal involvement in BS patients.

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