Table 2.

Studies reporting on patients with Behçet's disease treated with tocilizumab and ustekinumab.

First author (reference, year)	N pts	Main BS clinical and laboratory features	HLA-B51	Previous biologic agents with causes of withdrawal	Dosage and eventual cotherapies	Followup	Outcome
Hirano (2012) [51]	1	Mucosal involvement, EN, and uveitis	NR	IFX: loss of efficacy	TCZ 8 mg/kg every 4 weeks	12 months	VA improvement and resolution of EN and genital aphtosis. Partial improvement of oral aphtosis
Shapiro (2012) [52]	1	Mucosal and neurologic involvement, bilateral uveitis, and cutaneous vasculitis	NR	IFX: concomitant onset of IgA nephropathy	TCZ 8 mg/kg every 4 weeks + PDN 30–60 mg/day	7 months	CR after the 2nd infusion; PDN was tapered off; complete resolution of ocular, neurological, and skin manifestations; oral ulcers recurred
Urbaniak (2012) [53]	1	Mucosal and neurologic involvement, EN, DVT, and thrombophlebitis	NR	IFX: worsening of the gait disturbance and relapse of myelitis	TCZ 8 mg/kg every 4 weeks + AZA 150 mg/day + PDN 1 mg/kg/day	8 months	Improvement of clinical signs and symptoms; after the 4th infusion TCZ was discontinued due to a scrotal abscess
Caso (2013) [54]	1	Fever, mucosal involvement, myalgia, bilateral uveitis, optic neuritis, EN, SPR, and increase of inflammatory markers overlapping with refractory pemphigus foliaceus	Positive	IFX and ADA: inefficacy; ANA: loss of efficacy	TCZ 480 mg every 4 weeks	14 months	CR with improvement of inflammatory markers within few days
Redondo-Pachón (2013) [55]	1	Mucosal involvement, EN, iridocyclitis, secondary amyloidosis, and increase of CRP	Positive	None	TCZ 8 mg/kg every 4 weeks + colchicine 1 mg/day	12 months	CR with decrease of proteinuria and CRP after the 2nd infusion.
Diamantopoulos (2013) [56]	2	Mucosal involvement, pseudofolliculitis, and cutaneous vasculitis	NR	IFX and ETN: short efficacy and ADR (not specified)	TCZ 8 mg/kg every 4 weeks + AZA 150 mg/day	Unknown	Inefficacy; worsening of mouth and genital ulcers
		Mucosal involvement, increase of inflammatory markers	NR	IFX and ADA: incomplete response and ADR (not specified)	TCZ 8 mg/kg every 4 weeks	3 months	Initial PR with loss of efficacy after the 3rd infusion; recurrence of genital ulcers
Baerveldt (2013) [57]	1	Mucosal involvement, anterior uveitis, arthritis, and pathergy reaction overlapping with psoriasis vulgaris and hidradenitis suppurativa	NR	None	Ustekinumab 45 mg at weeks 0 and 4 and every 12 weeks	36 months	CR within few months, clinical improvement of psoriasis

ADA: adalimumab; ADR: adverse reactions; ANA: anakinra; AZA: azathioprine; BD: Behçet's disease; CR: complete remission; CRP: C-reactive protein; DVT: deep venous thrombosis; EN: erythema nodosum; ETN: etanercept; HLA: human leukocyte antigen; IFX: infliximab; MRI: magnetic resonance imaging; MTX: methotrexate; N: number; NR: not reported; pts: patients; PDN: prednisone; PR: partial remission; TCZ: tocilizumab; VA: visual acuity.