Summary of rituximab treatment for refractory ANCA associated vasculitis
| Series | Patient number/diagnosis | Predominant disease manifestations | Median disease activity before RTX | RTX doses | Concomitant treatment | Remission details | Severe adverse reactions | Follow up | No of relapses (time to relapse) |
|---|---|---|---|---|---|---|---|---|---|
| AZA, azathioprine; BVAS, Birmingham vasculitis activity score28; BVAS/WG, Birmingham vasculitis activity score for Wegener’s granulomatosis29; CSS, Churg-Strauss angiitis; CYC, cyclophosphamide; E, episcleritis; EB, endobronchial disease; ENT, ear, nose, and throat G; gangrene J; joints; K, kidney; L, lung unspecified; LH, lung haemorrhage; LN, lung nodules; MMF, mycophenolate mofetil; MP, intravenous methylprednisolone; MPA, microscopic polyangiitis; MTX, methotrexate; OG, retro-orbital granulomata; ON, optic neuritis; P, prednisolone or prednisone; PE, pulmonary embolism; PEX, plasma exchange; PN, peripheral neuropathy; RTX, rituximab; SIR, sirolimus; WG, Wegener’s granulomatosis. | |||||||||
| Prospective pilot study (Smith K et al, unpublished data) | 11 Total (5 WG, 5 MPA, 1 CSS) | 2 EB, 1 LN, 1 PN (CSS), 1 OG, 5 J, 3 ENT, 2 E | BVAS = 10 (median) | 11×4×375 mg/m2 (+ 1×500 mg CYC) | 8 MMF, 1 AZA, 2 none, p 20 mg/day (median) | 9 Complete; BVAS = 0, 1 Partial; BVAS = 2 1 No remission p 7.5 mg/day (median) at 6 months | 1 PE + pneumonia, 1 subcutaneous abscess | 23 Months median | 6 (median 16.5 months) |
| Prospective pilot study23 | 10 Total (all WG) | 7 K, 1 LH, 3 LN, 1 ON, 1 OG+E | BVAS/WG = 6 | 10×4×375 mg/m2 | p 1 mg/kg +/− MP | 10 Complete; BVAS = 0 p 0 mg/day (median) at 6 months | 1 Infusion reaction | 12 Months median | 1 (9 months) |
| Prospective pilot study27 | 7 Total | 5 OG, LN, 2 EB | BVAS1 = 7.5 | 4×375 mg/m2 every 4 weeks | 5 CYC, 2 MTX, 1 MMF P17.5 mg/day (median) | 2 Complete 1 Partial 5 No remission (at 1 month after treatment) | None | median 18 months (range 6–28). Detailed information not given | None |
| Retrospective series22 | 11 Total (10 WG, 1 MPA) | 4 LH, 5 K | BVAS/WG = 3–11 (range) | 11×4×375 mg/m2 | 3 PEX, 8 None p 1 mg/kg/day +/−MP | 11 Complete; BVAS = 0 | 1 MRSA osteomyelitis | 14 Months median | 2 (7, 12 months) |
| Retrospective series24 | 9 Total (2 WG, 5 MPA) | 4 LN, 2 K, 1 G, 1 PN, 1 J (partial) | BVAS = 6 (median) | 6×4×500 mg 2×2×500 mg 1×4×375/m2 | 3 MMF, 1 MMF+SIR, 1 AZA, 2 CYC, 1 none p 17.5 mg/day (median) | 8 Complete; BVAS = 0 1 Partial; BVAS = 1 | None | 6–24 Months (range) | 2 (12, 13 months) |
| Retrospective series26 | 3 WG | 1 OG, 2 K, 2 L | Unknown | 2×4×375 mg/m2 1×1×375 mg/m2 | 1 MTX+P5 mg/day 1 MP+P10 mg/day 1 AZA+MP | ? 3 Complete | None | 8, 12, 21 Months | 3 (8, 12, 21 months) |
| Retrospective series25 | 2 WG | 2 ENT, 1 L, 1 pyoderma | Unknown | 1×3×375 mg/m2 1×15×375 mg/m2 | 1 MP 1 g×3 1 MTX+MMF | 1 Remission 1 No remission | Unknown | Unknown | Unknown |