FRI0194 IDENTIFYING PHENOTYPES OF PATIENTS WITH BEHÇET’S SYNDROME BY A CLUSTER ANALYSIS IN A LARGE COHORT
Authors: J. Zou, J. Guan
Journal: Annals of the Rheumatic Diseases (2020)

Background: Behçet’s syndrome (BS) is a clinical entity with heterogeneous clinical manifestations (1). The symptoms and major organ involvement of BS tend to vary among gender and age (2). Objectives: To investigate the gender associated clinical features and aggregate of patients with BS based on phenotypes and organ involvement in a well-defined large cohort of patients with BS. Methods: This was a single-center study of 1,596 consecutive BS patients in Shanghai, China from 2012 September to 2019 December. We compared clinical variables and conducted a hierarchical cluster analysis applied according to twenty-five clinical variables to determine subgroups of patients. Results: A total of 850 BS patients were included. Male gender was associated with ocular involvement (RR 2.33), vascular involvement (RR 2.05) and cardiac lesions (RR 5.52). Female gender associated with genital ulcers (RR 0.85). Four clusters (C1–C4) were yielded. Cluster C1 (n=165, 19.9%), merely oral and genital ulcer type: consisted predominantly of older females. Disease severity was relatively low, Krause score = 2.32. In C2 (n=365, 43.9%), mix type: was an equal gender distribution with older onset age. Most patients had erythematous nodosum (64.1%) and genital ulcers (82.2%). Cardiac and vascular involvement were observed in 9.3% and 12.1%, which were the highest frequencies among the four clusters. C3 (n=160, 19.3%), intestinal type: was an equal sex ratio with older onset age. All patients presented with intestinal ulcer, while fewer patients had erythematous nodosum and pseudofolliculitis, 20.0% and 25.0% respectively. In C4 (n=141, 17.0%), uveitis type: was predominantly male with younger onset age. Intestinal involvement was negatively associated with uveitis [0.24, (0.13, 0.46), P &lt; 0.0001]. No patients had cardiac involvement in C3 or C4. Regarding the sensitivity of classification criteria, Cheng and Zhang (China) remain the highest in all clusters compared with Japan revised criteria, ICBD, ISG. Conclusion: From our large cohort, the gender-phenotypes analyses suggest that male is a risk factor for ocular and cardiovascular in BS patients. We identify four unique subphenotypes representing different clinical features, intestinal and ocular phenotype clusters represent the two most common manifestations of major organ involvement in BS. References: [1]Bettiol A, Hatemi G, Vannozzi L, et al. Treating the Different Phenotypes of Behcet’s Syndrome. Front Immunol 2019;10:2830. [2]Ishido T, Horita N, Takeuchi M, et al. Clinical manifestations of Behcet’s disease depending on sex and age: results from Japanese nationwide registration. Rheumatology (Oxford). 2017;56:1918-27. TABLE 1. Characteristics of patients with Behçet’s syndrome after clustering on clinical manifestations Characteristics C1 (n=165) C2 (n=356) C3 (n=160) C4 (n=141) Age at onset (IQR), years 29 (20-40) 28 (20-35) 27 (20-38) 25 (19-32 ) Disease duration (IQR), years 5 (3-10) 8 (3-10) 6 (3-10) 7 (4-10) Male gender (%) 38.9 52.3 51.2 75.2 Classification criteria JPN (%) 0 69.6 25.0 83.7 CHN (%) 98.8 96.7 97.5 99.3 ICBD (%) 98.8 89.9 65.6 99.3 ISG (%) 0.6 72.1 25.0 81.6 Oral ulceration (%) 100 100 98.1 99.3 Genital ulceration (%) 98.2 82.2 63.1 58.9 Erythema nodosum (%) 0 64.1 20.0 48.9 Pseudofolliculitis (%) 0 34.5 25.0 27.0 Arthritis/Arthralgia (%) 0 36.2 11.6 14.2 Uveitis (%) 0.6 0.8 1.9 100 Gastrointestinal involvement (%) 0 1.1 100 2.7 Vascular involvement (%) 2.4 12.1 1.2 4.3 Cardiac lesion 3.6 9.3 0 0 Central nerve system involvement (%) 4.2 3.8 2.5 3.5 JPN: Japan revised criteria; CHN: Cheng and Zhang (China) criteria; ICBD: International Criteria for Behçet’s disease; ISG: International Study Group for Behçet’s disease. Disclosure of Interests: None declared