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.

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.

Adamantiadis-Behçet's syndrome: central nervous system involvement

1993 ◽  
Vol 87 (4) ◽  
pp. 290-293 ◽  
Author(s):  
P. Hatzinikolaou ◽  
G. Vaiopoulos ◽  
S. Mavropoulos ◽  
D. Avdelidis ◽  
G. Stamatelos ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Involvement ◽  
Behçet’S Syndrome ◽  
System Involvement ◽  
Behçet's Syndrome ◽  
Behcet’S Syndrome ◽  
Behcet's Syndrome

Serum Levels of Interleukin-36 Alpha and Interleukin-36 Receptor Antagonist In Behcet’s Syndrome

2021 ◽  
Author(s):  
Pelin Ünsal ◽  
Pamir Çerçi ◽  
Şükrü Alper Açıkgöz ◽  
Göksal Keskin ◽  
Ümit Ölmez
Keyword(s):  
Central Nervous System Involvement ◽  
Serum Levels ◽  
Behçet’S Syndrome ◽  
Behçet's Syndrome ◽  
Oral Ulcers ◽  
Group A ◽  
Behcet’S Syndrome ◽  
The Mean ◽  
Clinically Significant ◽  
Behcet's Syndrome

Abstract Background Behcet’s syndrome (BS) is a systemic vasculitic disorder. This study aimed to investigate the levels of serum IL-36α and IL-36Ra in patients with BS. Material and Methods A total of 80 subjects (60 BS patients and 20 healthy controls [HC]) were included. Results The median IL-36α level was 0.11 ng/ml in the BS group and 0.09 ng/ml in the HC group (p=0.058). The mean IL-36Ra level was 13.62 pg/ml in the BS group and 13.26 pg/ml in the HC group (p=0.348). Serum IL-36Ra levels of the active group were significantly higher (p=0.037). Patients with oral ulcers and central nervous system involvement had higher serum IL36Ra levels. In the BS group, a positive correlation was found between serum IL-36Ra and CRP. In a multivariate analysis, the IL-36Ra level (OR=1.067; 95% CI=1.001–1.137; p=0.045) was independently associated with disease activity. Conclusion According to these findings, it is not clear whether such a slight difference is clinically significant, but they suggest that the IL-36 cytokine family may play a role in the course of the disease.

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