Table 2: Studies reporting on patients with Behçet’s disease treated with tocilizumab and ustekinumab.

First author (reference, year) ptsMain BS clinical and laboratory featuresHLA-B51Previous biologic agents with causes of withdrawalDosage and eventual cotherapies FollowupOutcome

Hirano (2012) [51]1Mucosal involvement, EN, and uveitisNRIFX: loss of efficacyTCZ 8 mg/kg every 4 weeks12 monthsVA improvement and resolution of EN and genital aphtosis. Partial improvement of oral aphtosis

Shapiro (2012) [52]1Mucosal and neurologic involvement, bilateral uveitis, and cutaneous vasculitisNRIFX: concomitant onset of IgA nephropathyTCZ 8 mg/kg every 4 weeks
+ PDN 30–60 mg/day
7 monthsCR after the 2nd infusion; PDN was tapered off; complete resolution of ocular, neurological, and skin manifestations; oral ulcers recurred

Urbaniak (2012) [53]1Mucosal and neurologic involvement, EN, DVT, and thrombophlebitisNRIFX: worsening of the gait disturbance and relapse of myelitisTCZ 8 mg/kg every 4 weeks
+ AZA 150 mg/day
+ PDN 1 mg/kg/day
8 monthsImprovement of clinical signs and symptoms; after the 4th infusion TCZ was discontinued due to a scrotal abscess

Caso (2013) [54]1Fever, mucosal involvement, myalgia, bilateral uveitis, optic neuritis, EN, SPR, and increase of inflammatory markers overlapping with refractory pemphigus foliaceusPositiveIFX and ADA: inefficacy;
ANA: loss of efficacy
TCZ 480 mg every 4 weeks14 monthsCR with improvement of inflammatory markers within few days

Redondo-Pachón (2013) [55]1Mucosal involvement, EN, iridocyclitis, secondary amyloidosis, and increase of CRPPositiveNoneTCZ 8 mg/kg every 4 weeks
+ colchicine 1 mg/day
12 monthsCR with decrease of proteinuria and CRP after the 2nd infusion.

Diamantopoulos (2013) [56]2Mucosal involvement, pseudofolliculitis, and cutaneous vasculitisNRIFX and ETN: short efficacy and ADR (not specified)TCZ 8 mg/kg every 4 weeks
+ AZA 150 mg/day
UnknownInefficacy; worsening of mouth and genital ulcers
Mucosal involvement, increase of inflammatory markersNRIFX and ADA: incomplete response and ADR (not specified)TCZ 8 mg/kg every 4 weeks3 monthsInitial PR with loss of efficacy after the 3rd infusion; recurrence of genital ulcers

Baerveldt (2013) [57]1Mucosal involvement, anterior uveitis, arthritis, and pathergy reaction overlapping with psoriasis vulgaris and hidradenitis suppurativaNRNoneUstekinumab 45 mg at weeks 0 and 4 and every 12 weeks36 monthsCR 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; : number; NR: not reported; pts: patients; PDN: prednisone; PR: partial remission; TCZ: tocilizumab; VA: visual acuity.