First author (reference, year)
ptsClinical and laboratory features HLA-B51 Previous biologic agents and causes of withdrawal Dosage and eventual cotherapies Followup Outcome Botsios (2008) [43 ] 1 Fever, mucosal involvement, colon ischemic perforation, necrotizing lymphocytic venulitis, thrombosis, serositis, increase of inflammatory markers, and SPR Negative IFX: mucosal abdominal abscesses ANA 100 mg/day + PDN 5 mg/day 20 months CR and improvement of inflammatory markers in 7–10 days. Disease-free at 20-month followup Bilginer (2010) [44 ] 1 Fever, mucosal involvement, EN, arthritis, secondary amyloidosis, increase of inflammatory markers, and SPR overlapping with FMF NR None ANA 1 mg/kg/day 18 months CR and improvement of inflammatory markers; patient free of clinical symptoms at 18-month followup; however proteinuria gradually increased (from 1,8 to 2,4 g/day) Gül (2012) [45 ] 7 Acute posterior or panuveitis and/or retinal vasculitis NR None GEV: 0.3 mg/kg (single infusion) Up to disease relapse Improvement in visual acuity from day 1 in 5 out of 7 patients. Complete resolution of retinal findings achieved in 4–21 days (median 14 days). No detailed assessments of extraocular manifestations were performed. Recurrence of folliculitis and oral aphtosis. Median duration of response: 49 days (range: 21–97 days) Ugurlu (2012) [46 ] 1 Mucosal involvement, EN, bilateral panuveitis, retinal vasculitis, and SPR NR INF-γ : fever; CAN 150 mg (single dose) 8 weeks CR for 8 weeks; resolution of ocular inflammation and rapid VA improvement. Emmi (2013) [47 ] 1 Mucosal and gastrointestinal involvement, arthritis, pseudofolliculitis, and bilateral retinal vasculitis NR IFX: ADR (diffuse urticaria with angioedema); ANA 100 mg/day 12 months CR after 12 months of followup; rapid and persistent disappearance of joint pain, mucocutaneous and bowel manifestations; VA improvement, clearing of the vitreous opacity and no active retinal inflammation Cantarini (2013) [12 ] 9 Mucosal involvement, EN, headache, retinal vasculitis, low-back pain, and increase of inflammatory markers Positive ETN and IFX: loss of efficacy ANA 150 mg/day + 9 months PR; PDN was reduced to 7.5 mg/day Fever, mucosal involvement, skin lesions, headache, arthritis, abdominal pain, and increase of inflammatory markers Positive None ANA 100 mg/day 19 months CR at 12-month followup; Fever, mucosal involvement, skin lesions, headache, and increase of SAA Positive ANA (100 mg/day): inefficacy ANA 150 mg/day + 19 months DVT not resolved with heparin at 6 months; CYC (5 mg/kg/day) was added; Mucosal involvement, bilateral panuveitis, retrobulbar optic neuritis, papillophlebitis, headache, arthralgia, DVT, and increase of SAA Positive ETN: loss of efficacy ANA 100 mg/day + 6 months Flare of panuveitis after 3 months; ANA was withdrawn; CR with ADA (40 mg twice monthly) + MTX (10 mg/weekly) + PDN (25 mg/day) Mucosal involvement, bilateral panuveitis, arthralgia, and increase of inflammatory markers Positive None ANA 100 mg/day + AZA 50 mg/day + PDN 7.5 mg/day 8 months CR after 12 months Fever, mucosal involvement, venous thrombosis, arthritis, panuveitis, headache, pseudofolliculitis, and increase of ESR and SAA Positive None ANA 100 mg/day 12 months Flare of uveitis after 8 months; ANA was increased to 150 mg/day + MTX (15 mg/weekly) + colchicine (1 mg/day); PR at 17 months of followup Mucosal involvement, skin lesions, abdominal pain, photophobia, and increase of SAA Positive ADA: inefficacy ANA 2 mg/kg/day + PDN 15 mg/day 9 months CR at first; relapse after 4 months requiring an increased dosage of ANA (2.5 mg/kg/day); PR after 7 months Fever, mucosal involvement, EN, arthritis, anterior uveitis, pseudofolliculitis, and increase of CRP Positive None ANA 100 mg/day + 6 months PR; CYC (5 mg/kg/day) was added after 8 months of followup Fever, mucosal and gastrointestinal involvement, headache, anterior uveitis, and arthralgia Positive ETN and ADA: loss of efficacy ANA 100 mg/day 9 months Inefficacy after 8 weeks; Caso (2014) [48 ] 1 Mucosal and ocular involvement, pseudofolliculitis, sacroiliitis, and increase of inflammatory markers Positive IFX: loss of efficacy ANA 100 mg/day + 12 months CR in few days; PDN was tapered to 5 mg/day Cantarini (2012) [49 ]50 ] 3 Fever, mucosal involvement, skin lesions, arthritis, abdominal pain, headache, and increase of inflammatory markers and SAA overlapping with granuloma annulare Positive SSZ, MTX, CYC, AZA and LFN: inefficacy; CAN 150 mg every 8 weeks 16 months CR in few months; Fever, mucosal and gastrointestinal involvement, headache, anterior uveitis, and arthralgia Positive ETN and ADA: loss of efficacy; ANA: inefficacy CAN 150 mg every 6 weeks 12 months CR after few months Fever, mucosal involvement, DVT, panuveitis, headache, arthritis, pseudofolliculitis, and increase of ESR and SAA Positive ANA: ADR (urticarial skin lesions) CAN 150 mg every 6 weeks 6 months CR within few days
