Table 1.
References | Study design | Age at first dose (years), mean (SD) | Total (female) | Antibody subtype | MG subtype | Disease duration (months), mean (SD) | Follow-up time (months), mean (SD), minimum | RTX regimen |
---|---|---|---|---|---|---|---|---|
Lu et al. (27) | Prospective | 30.6(29.6)* | 12(10) | AchR+ | Refractory generalized | 59.6(37.7) | 18(0), 18 | Induction treatment: 600 mg Maintenance treatment:600 mg every 6 months |
Brauner et al. (20) | Retrospective | 60(18) | 72(31) | AchR+ | Generalized | 67(95) | 40(19), NA | Induction treatment: 1,000 mg in 3 patients, 500 mg in 57 patients, 100 mg for 12 patients. Maintenance treatment: 500 mg in 69 patients, 100 mg every 6 months in 3 patients |
Piehl et al. (19) | Randomized clinical trial | 67.4(13.4) | 25(7) | AchR+ | New onset generalized | 4.47(3.05) | 6(0), 6 | A single dose of 500 mg RTX |
Zhou et al. (28) | Retrospective | 40(13.85) | 12(11) | Musk+ | Generalized | 21.42(22.11) | 6(0), 6 | 100 mg (day1) and 500 mg (day2) |
Zhao et al. (29) | Retrospective | NA | 8(6) | Musk+ | Generalized | 41.38(63.19) | 6(0), 6 | Induction treatment: 100 mg weekly for 3 weeks. Maintenance treatment: 100 mg if CD19+ B cell > 1% or CD19+CD27+ memory B cells > 0.05% of PBMCS |
Meng et al. (30) | Retrospective | 44.9(9.7)* | 8(8) | Musk+ | Generalized | 10.75(12.69) | 22(8.55), 8 | 375 mg/m2 for 1 or 2 infusions |
Yang et al. (21) | Retrospective | 50.56(13.51) | 9(8) | Musk+ | Generalized | 24.22(14.76) | 13(6.4), 3 | Induction treatment: 500 mg Maintenance treatment: 500 mg every 6 months or 1 year |
Blum et al. (31) | Retrospective | 50.08(18.72) | 13(9) | Musk+ | Generalized | 10.08(13) | 11.85(6.56), 4 | 500 mg weekly for 2 weeks in 12 patients, 500 mg in 1 patient |
Choi et al. (32) | Retrospective | 50.53(15.56) | 17(11) | 9AchR+, 6Musk+, 2Neg | Refractory generalized | 122.2(88.3) | 24.47 (11.29), 7 | Induction treatment: 375 mg/m2 twice with a 2-week interval Maintenance treatment:375 mg/m2 once if B cell repopulates or relapse |
Heckmann (33) | Retrospective | 36.38(15.18) | 17(16) | 5musk, 10AchR+, 2Neg | Refractory generalized | 134.2(108.5) | 19.12(12.81), 6 | A single dose of 375 mg/m2 |
Zhong et al. (34) | Prospective | 30.8(13.5) | 12(10) | AchR+ | Refractory generalized | 54.83(40.83) | 6(0), 6 | A single dose of 600 mg |
Li et al. (35) | Retrospective | NA | 19(12) | AchR+ | New onset generalized | NA | 51.55(11.32), 30.3 | 100 mg for 1 day, 2 days, 3 days, or 4 days if CD19+Bcells were (0.5%-1.5%), (1.5%-5%), (5%-15%), or (>15%) of PBMCS |
Castiglione et al. (36) | Retrospective | NA | 9(NA) | 5AchR+, 4Musk+ | Generalized | NA | 24(0), 24 | 375 mg/m2 twice with a 2-week interval in 7 patients, 500 mg weekly for 3 weeks in 2 patients |
Du et al. (37) | Retrospective | 58.92(10.01) | 13(7) | AchR+ | New onset generalized | 2.85(2.07) | 25(11.21), 12 | Induction treatment:100 mg no more than 3 weeks, Maintenance treatment:100 mg based on circulating B-cell repopulation every 3 months |
Jing et al. (18) | Prospective | 34.4(13.1) | 15(14) | 13AchR+, 1Musk+, 1 Neg | Refractory generalized | 57.3(32.8) | 6(0), 6 | 100 mg (day 1) and 500 mg (day2) |
Ren et al. (38) | Retrospective | 48.45(16.29) | 22(18) | 9AchR+, 4Musk+, 1LRP4, 8Neg | Refractory generalized | 66 range(45-144) | Median 48.5 range(23-84) | Induction treatment: 100 mg in 12 patients, 200 mg in 2 patients, 500 mg in 7 patients, 100 mg (day1)and 500 mg (day 2) in 1 patient. Maintenance treatment: 500 mg in 17 patients, 100-200 mg in 5 patients if CD20+B-cell %>1% of PBMCS |
Brauner et al. (20) | Prospective | 53.44(12.77) | 9(5) | AchR+ | Generalized | NA | 6(0), 6 | 100 mg weekly for 4 weeks in 6 patients, 100 mg in 3 patients |
NA, not available.
Age at onset.