Clinical data of 21 patients with myasthenia gravis
Sample no | Subgroup | Sex/age at onset, years | Disease duration, years* | Diagnostic testing | PIS/MGFA classification* | Therapies given* (thymus histology) | AChR RIA, nmol/L | AChR CBA | MuSK CBA | CN21 without rapsyn current inhibition, % (no of cells) | CN21 with rapsyn current inhibition, % (no of cells) |
1 | AChR | F/14 | 17 | Decr + Tens + | IIIa | APR 5 mg | 38.7 | ND | ND | 30.3±3.3† (3) | 22.9±5.3† (4) |
2 | AChR | F/59 | 8 | Decr + Tens + | IIIb | APR, AZA, THX (normal) | 7.6 | ND | ND | 16.9±9.1 (3) | 9.9±3.1 (3) |
3 | AChR | F/30 | 2 | Decr + Tens + | I | APR, THX (hyperplasia) | >300 | ND | ND | 11.3±1.7 (3) | 14.8±2.9 (3) |
4 | AChR | F/35 | 9 | Decr – Tens + | MM | MMF, THX (thymoma) | 15.6 | ND | ND | 10.0±3.3 (3) | 22.4±8.8† (3) |
5 | AChR | M/58 | 2 | Decr – Tens + | MM | APR, AZA | 14.8 | ND | ND | 10.4±4.9 (3) | 10.8±3.4 (3) |
6 | AChR | F/35 | 8 | Decr + Tens + | I | APR, THX (hyperplasia) | 11.8 | ND | ND | 16.7±5.8 (3) | 6.4±4.0 (3) |
7 | AChR | M/72 | 1 | Decr + Tens + | IIb | Naive | 5.8 | ND | ND | 28.5±15.3† (3) | 39.5±12.1† (4) |
8 | AChR | M/54 | 3 | Decr + Tens + | PR | APR, AZA, THX (normal) | 7.4 | ND | ND | 11.1±3.1 (3) | 2.3±4.2 (3) |
9 | AChR | M/65 | 3 | Decr – Tens + | PR | APR, THX (normal) | 5.4 | ND | ND | 10.1±4.1 (3) | 29.1±8.4† (3) |
10 | Clustered AChR | M/42 | 0.5 | Decr + Tens + | PR | APR, AZA, PLEX | <0.25 | Pos | Neg | 14.5±7.5 (7) | 24.0±1.2† (4) |
11 | Clustered AChR | M/48 | 20 | Decr + Tens + | PR | AChEI, THX (normal) | <0.25 | Pos | Neg | 9.4±3.3 (3) | 27.9±0.2† (3) |
12 | MuSK | F/22 | 6 | Decr – Tens + | PR | AZA, THX (hyperplasia) | Neg | Pos | 13.4±4.3 (3) | 11.0±2.4 (3) | |
13 | MuSK | F/38 | 4 | Decr + Tens – | IIIb | APR, AZA, PLEX | Neg | Pos | 9.1±2.1 (3) | 8.7±1.4 (3) | |
14 | MuSK | F/28 | 12 | Decr + Tens – | IIIb | APR, AZA | Neg | Pos | 18.2±3.4 (3) | 10.6±3.1 (3) | |
15 | MuSK | F/23 | 19 | Decr – Tens – | IIb | APR, RTX, THX (normal) | Neg | Pos | 13.1±1.2 (3) | 7.4±1.0 (3) | |
16 | MuSK | F/41 | 16 | Decr + Tens + | PR | AZA 100, THX (normal) | Neg | Pos | 0.4±4.7 (3) | 11.3±2.6 (3) | |
17 | SNMG | M/14 | 11 | Decr + Tens + | PR | APR | Neg | Neg | 10.9±1.4 (3) | 11.8±2.4 (3) | |
18 | SNMG | M/55 | 1 | Decr – Tens + | IIb | Naive | Neg | Neg | 19.1±3.7 (3) | 1.8±11.9 (3) | |
19 | SNMG | M/28 | 5 | Decr + Tens – | IIb | APR, MMF, THX (hyperplasia) | Neg | Neg | 16.6±3.1 (3) | 14.6±1.9 (4) | |
20 | SNMG | F/25 | 22 | Decr + Tens – | IIIb | TAC, THX (hyperplasia) | Neg | Neg | 14.4±2.8 (3) | 10.1±1.1 (3) | |
21 | SNMG | F/25 | 6 | Decr – Tens + | IIa | AZA, THX (thymitis) | Neg | Neg | 25.8±2.7† (3) | 8.9±3.0 (3) |
*At the time of blood sampling.
†Significant current inhibition higher than 3 SD from mean value of five HC sera.
AChEI, acetylcholinesterase inhibitor; AChR, acetylcholine receptor; APR, prednisolone; AZA, azathioprine; BSA, bovine serum albumin; CBA, cell-based assay; CSR, complete stable remission; Decr, decrement at repetitive stimulation; HC, Healthy control; MGFA, Myasthenia Gravis Foundation of America; MM, minimal manifestation; MMF, mycophenolate mofetil; MuSK, muscle-specific kinase; ND, not determined; ns, Not significant; PBS, Phosphate buffered saline; PIS, postintervention status; PR, pharmacological remission; RIA, radioimmunoprecipitation assay; RTX, rituximab; SNMG, seronegative myasthenia gravis; TAC, tacrolimus; Tens, Tensilon test; THX, thymectomy.