Recent Insights into the Management of Behçet Syndrome

Figures & data

Table 1 The Effect of Drugs According to the Types of Involvement in Behçet Syndrome

Drugs	Type of Study	Type of Organ Involvement
		Skin and Mucosa	Joint	Uveitis	Vascular Involvement	CNS Involvement	GI Involvement
Colchicine	RCT	✔	✔	✘
	OS
Apremilast	RCT	✔
	OS	✔
Azathioprine	RCT	✔	✔	✔
	OS			✔	✔	✔	✔
Cyclosporine-A	RCT			✔
	OS				✔	⚠
Cyclophosphamide	OS				✔
Interferon-alpha	RCT	✔	✘	✔
	OS	✔	✔	✔	✔
TNF-inhibitors	RCT	✔	✔
	OS	✔	✔	✔	✔	✔	✔
IL-1 inhibitors	RCT			✘
	OS	✔	✔	✔
IL-6 inhibitors	OS	⚠	⚠	✔	✔	✔
IL-17 inhibitors	RCT			✘
	OS		✔
IL-23 inhibitors	OS	✔
Thalidomide	RCT	✔	✘
	OS						✔
Mycophenolic acid	OS			✔	✔	✔
Tofacitinib	OS		✔	✔	✔

Notes: “✔”: Effective, “✘”: Not Effective, “”: Not Evaluated, “”: Controversial/Inconclusive, ⚠: Reported to cause relapses.

Abbreviations: RCT, randomized controlled trial; OS, observational study.

Table 2 Comparisons of Infliximab and Adalimumab in the Treatment of Behçet Uveitis in Retrospective Studies

	Atienza-Mateo B. et al, 2019 177 (94 M/83 F)		Fabiani C. et al, 2019 107 (61 M/46 F)
	IFX	ADA	IFX	ADA
n of patients	103 (55 M/48 F)	74 (39 M/ 35 F)	41	66
n of eyes	185	131	73	114
Mean age (SD)	40.4 (10.1)	38.7 (11.3)	42.2 (12.1)	39.5 (12.1)
Duration of uveitis^a	36 [12–72]	24 [12–60]	11.6 (8.6)	9.1 (7.4)
Pattern of uveitis (%)
Posterior	28 (27.2)	14 (18.9)	22 (52.4)	24 (36.4)
Panuveitis	64 (62.1)	45 (60.8)	19 (45.3)	37 (56.1)
Intermediate	0 (0)	1 (1.4)	1 (2.4)	5 (7.6)
Retinal vasculitis^b	114 (61.6)	78 (59.5)	25 (61.0)	46 (69.7)
Previous treatment (%)
Corticosteroid	95	88	100	100
Cyclosporine	75	78	23	27
Azathioprine	57^c	42^c	8	17
Methotraxate	44	42	12	20
Combination with cDMARD %	76.5	70.3	48.8	46.7
Cyclosporine	41.1	55.7	40	22.6
Azathioprine	21.8	19.2	15	29
Methotraxate	33.3	21.1	30	35.4
MMF	1.3	3.8	20	3.2
Mean follow-up TNFis (mo)	31.5±23.5	26.5±18.6	56.6 ± 56.0	26.5 ± 21.7
Treatment outcomes at mo 12
Improvement of ACI %	78.2	92.3	NA	NA
Improvement of vitritis %	79.0^d	93.3^d	NA	NA
Improvement of RV %	97	95	86.3	71.4
Macular thickness	264.9±59.7	250.6±36.9	NA^e	NA^e
BCVA	0.67±0.34^f	0.81±0.26^f	0.4±0.0	0.4±0.11
Decrease of uveitis attack%	NA	NA	84.2	66.7
Drug retention rate %	85.0^g	95.2^g	87.8	79.8
Severe AE/toxicity	8 (7.8)	4 (3.9)	NA	NA

Notes: ^cp=0.049, ^dp=0.04, ^fp=0.001, ^gp=0.042. ^aDisease duration given as median [IQR] in the first study and mean (SD) in the second study. ^bRetinal vasculitis was reported the number of involved eye in the first study and the number of patient in the second study. ^eNo quantitative measurements for macular thickness was given in the second study. However, central macular thickness was significantly lower in infliximab group compared to adalimumab group at the last follow-up.

Abbreviations: ADA: adalimumab, AE: adverse event, IFX: infliximab; ACI, anterior chamber inflammation; RV, retinal vasculitis; BCVA, Best corrected visual acuity; MMF, mycophenolate mofetil; NA, not available.