Table 3.
Sex | Age at onset | Antibody | Thymectomy | Histology | Time to refractory MG (mos.) | IS treatment at refractory timepoint | MGFA class at last FU | |
---|---|---|---|---|---|---|---|---|
1 | m | 73 | AChR | No | NA | 60 | CS 5 mg, MMF 1000 mg, CP (1g4w) | IIIa |
2 | m | 59 | AChR | No | NA | 56 | CS 75 mg, CP (1 g q4w), IA/PLEX | IIIb |
3 | m | 41 | MuSK | No | NA | 24 | CS 18.8 mg, TC 6 mg, IA/PLEX | IIb |
4 | m | 54 | AChR | Yes | Thymoma | 24 | CS 12.5 mg, AZA 150 mg, IA/PLEX | 0 (MM3) |
5 | f | 22 | SN | Yes | Hyperplasia | 197 | CS 10 mg, RTX (375 mg/m2 BSA) | IIa |
6 | f | 35 | AChR | Yes | Thymoma | 74 | CS 25 mg, MMF 2000 mg | IIa |
7 | f | 27 | AChR | Yes | Normal | 24 | CS 8.75 mg, AZA 150 mg, IA/PLEX | IIb |
8 | m | 13 | AChR | Yes | Hyperplasia | 158 | MMF 150, RTX (375 mg/m2 BSA), IA/PLEX | I |
9 | m | 49 | AChR | No | NA | 52 | CS 25 mg, AZA 200 mg, IVIG | I |
10 | m | 28 | SN | Yes | Hyperplasia | 49 | CS 25 mg, MMF 2000 mg, IVIG | I |
11 | f | 27 | AChR | Yes | Normal | 24 | CS 25 mg, AZA 100 mg, IVIG | IIb |
12 | m | 48 | AChR | Yes | Thymoma | 25 | CS 17.5 mg, MMF 2000 mg, RTX (375 mg/m2 BSA), IVIG | IIa |
13 | f | 31 | SN | Yes | Normal | 25 | CS 20 mg, AZA 100 mg, RTX (375 mg/m2 BSA); IA/PLEX | IIIa |
14 | f | 20 | AChR | Yes | Hyperplasia | 40 | CS 12.5 mg, AZA 100 mg | IIIb |
Characteristics of treatment-refractory patients. Treatment is shown for the timepoint patients met the definition of refractory MG
AChR denotes acetylcholine receptor, AZA azathioprine, BSA body surface area, CP Cyclophosphamide, CS corticosteroids, FU follow-up, IA/PLEX immunoadsorption or plasma exchange therapy (maintenance treatment), IS immunosuppressive, IVIG intravenous immunoglobulins (maintenance treatment), MG myasthenia gravis, MGFA Myasthenia Gravis Foundation of America, MM minimal manifestation, MMF mycophenolate mofetil, MuSK muscle-specific receptor tyrosine kinase, NA not applicable, SN seronegative, TC tacrolimus