Table 3 Detailed information associated with disease severity and treatment responses of the 18 enrolled MuSK-MG patients
Patient No. | Immunotherapy history | QMGs before therapy | MGFA before therapy | Maximum QMGs | Maximum severity MGFA | QMGs at last follow-up | MGFA-PIS at last follow-up | Immunotherapy at last follow-up | MuSK-ab at last follow-up (nmol/L) | No. of myasthenic crisis | Time from onset to crisis (m) |
|---|---|---|---|---|---|---|---|---|---|---|---|
1 | TAC, Pred | 22 | IIIb | 28 | V | 0 | PR | TAC (3 mg/d) | 0.001 | 1 | 34 |
2 | TAC, Pred | 15 | IIIb | 15 | IIIb | 0 | PR | TAC (3 mg/d) | 0.001 | 0 | NA |
3 | TAC, Pred | 16 | IIIb | 16 | IIIb | 0 | MM | TAC (3 mg/d) Pred (20 mg/d) | 0.20 | 0 | NA |
4 | TAC, Pred | 10 | IIb | 13 | IIb | 0 | PR | TAC (3 mg/d) | 0.46 | 0 | NA |
5 | RTX, Pred | 12 | IIb | 12 | IIb | 0 | PR | RTX (100 mg/6 m) | 0.22 | 0 | NA |
6 | RTX, Pred | 6 | I | 6 | I | 0 | MM | RTX (100 mg/5 m) Pred (5 mg/d) | 0.26 | 0 | NA |
7 | RTX, Pred | 11 | IIb | 11 | IIb | 0 | PR | RTX (100 mg/6 m) | 0.001 | 0 | NA |
8 | RTX, Pred | 20 | V | 20 | V | 0 | MM | RTX (100 mg/6 m) Pred (5 mg/d) | 0.20 | 1 | 3 |
9 | AZA, Pred, RTX | 7 | V | 26 | V | 0 | PR | RTX (100 mg/6 m) | 0.47 | 3 | 16a |
10 | RTX, Pred | 19 | V | 19 | V | 0 | MM | RTX (100 mg/6 m) Pred (15 mg/d) | 0.25 | 1 | 2 |
11 | AZA, Pred | 15 | IIb | 22 | V | 0 | PR | Pred (5 mg/d) | 0.45 | 1 | 15 |
12 | AZA, Pred | 6 | IIb | 6 | IIb | 2 | I | Pred (10 mg/d) | 0.51 | 0 | NA |
13 | AZA, Pred, RTX | 8 | IIb | 14 | IIIb | 0 | CSR | None | 0.37 | 0 | NA |
14 | Pred, RTX | 12 | IIb | 12 | IIb | 0 | PR | RTX (100 mg/6 m) | ND | 0 | NA |
15 | RTX, Pred | 20 | IIIb | 20 | IIIb | 0 | PR | RTX (100 mg/6 m) Pred (20 mg/d) | ND | 0 | NA |
16 | AZA, Pred, TAC | 18 | IIb | 18 | IIb | 1 | MM | TAC (3 mg/d) Pred (25 mg/d) | ND | 0 | NA |
17 | Pred | 11 | IIb | 16 | IIIb | 0 | PR | Pred (10 mg/d) | ND | 0 | NA |
18 | Pred | 3 | I | 3 | I | 0 | MM | Pred (25 mg/d) | ND | 0 | NA |