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. 2021 Jan 30;23:45. doi: 10.1186/s13075-021-02429-7

Table 2.

Characteristics of patients with Behçet’s syndrome after clustering on clinical manifestations

Characteristics C1 (n = 307) C2 (n = 124) C3 (n = 156) C4 (n = 142) C5 (n = 131)
Demographics
 Age (IQR), years 36 (27–47) 39 (29–48) 37 (28–48) 33 (27–42) 37 (29–44)
 Age at onset (IQR), years 28 (20–38) 28 (19–37) 28 (19–38) 25 (20–32) 27 (21–35)
 Disease duration (IQR), years 6 (3–10) 9 (4–10) 6 (3–10) 7 (4–10) 6 (3–10)
 Krause score 3 (2–3) 4 (3.3–5) 4 (4–5) 6 (5–6) 6 (5–7)
 Sex ratio (M/F) 0.64:1 0.97:1 1.08:1 2.94:1 2.54:1
Classification criteria
 JPN (%) 52.4 41.9 22.4 83.8 43.5
 CHN (%) 100 99.2 94.2 99.3 91.6
 ICBD (%) 100 79.0 62.8 99.3 87.8
 ISG (%) 52.8 45.2 23.1 81.7 43.5
Clinical manifestation
 Oral ulceration (%) 100 100 98.0 99.2 100
 Genital ulceration (%) 100 75.8 61.5 58.5 63.4
 Erythema nodosum (%) 40.7 44.4 19.2 48.6 49.6
 Folliculitis (%) 20.8 28.2 25.6 28.2 22.1
 Arthritis/arthralgia (%) 0 100 1.3 16.9 23.7
Major organ involvement
 Uveitis (%) 0 0 0 100 9.9
 Intestinal involvement (%) 0 14.5 92.3 5.6 6.1
 Esophageal lesions (%) 0 1.6 20.5 0.7 0.8
 Vascular involvement (%) 0 0 0 0 44.3
 Cardiac lesion (%) 0 0 0 0 33.6
 Myelodysplastic syndrome (%) 0.3 1.6 1.9 0 0
 Central nervous system involvement (%) 0 0.8 1.9 0.7 19.8

Values presented as n (%) unless otherwise stated. JPN Diagnostic criteria of the Behçet’s disease research committee of Japan (1987 revision), CHN the Chinese version of Behçet’s disease criteria created by Cheng and Zhang, ICBD International Criteria for Behçet’s disease, ISG International Study Group for Behçet’s disease