|
| First author (reference, year) | 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 |
|
