Table 1.
B-cell targeted mAb therapies in use or under investigation for the treatment of AE.
| Drug | Specific target | Evidence | Studied group; n | administration | Efficacy | Relapse, n (%) | Adverse, n (%) | |
|---|---|---|---|---|---|---|---|---|
| mRS score | CASE score | |||||||
| Rituximab | CD20 | Meta-analysis (17) | NMDAR AE:277 | 375 mg/m2 weekly for 4 weeks (most commonly used regimen) | mRS score ≤ 2; n (%) 206 (72) mean mRS score decreased by 2.67 |
NA | 21 (14.2) | Infusion-related reactions: 29 (16); Pneumonia:11 (6); Severe sepsis:2 (1) |
| Observational cohort (18) | NMDAR AE: 81 LGI1 AE: 26 CASPR2 AE: 11 GAD65 AE: 31 |
Infusion dose,g; Median (IQR) NMDAR:1.0(0.3) LGI1:1.0(0.3) CASPR2:1.0(0.02) GAD65:1.0 (0) |
mRS score ≤2; n (%) NMDAR: 48 (94) LGI1: 20 (83) CASPR2: 4 (80) GAD65: 14 (52) |
NA | NMDAR: 13 (19) LGI1: 5 (20) CASPR2: 1 (11) GAD65: 0 |
Infusion-related reactions: 2 Lymphopenia: 1 frequent infections: 1 unknown side effect: 1 |
||
| Ocrelizumab | CD20 | Terminated (NCT03835728) (19) |
LGI1 AE: 1 (Patient 1) NMDAR AE: 2 (Patient 2-3) |
Two 300-mg infusions 2 weeks apart, 600-mg infusion 24 weeks later; If clinical worsening endpoint is reached in the first 6 months, receive a single dose of 600 mg | Change of mRS: Patient 1: 3 to 3; Patient 2: 2 to 1; Patient 3: 4 to 1 |
NA | No | No |
| Ofatumumab | CD20 | Case Series (20) | LGI1 AE: 2 (Patient 1-2) NMDAR AE: 1 (Patient 3) |
Patient 1 and 2: 20 mg/week, 3 weeks Patient 3: 20 mg infusions 2 weeks apart |
Change of mRS: Patient 1: 3 to 1; Patient 2: 2 to 1; Patient 3: 4 to 1 |
NA | No | short-term low-grade fever: 3 (100) |
| Inebilizumab | CD19 | Ongoing (NCT04372615) | NMDAR AE | NA | NA | NA | NA | NA |
| Daratumumab | CD38 | Case Report (21–23) | NMDAR AE: 2 (Patient 1-2) CASPR2 AE: 3 (3–5) SCNAE: 2 (6–7) |
16 mg/kg weekly for the first 8 cycles, biweekly or monthly for the remaining cycles | Change of mRS Patient 1: 5 to 1 Patient 2: 5 to 3 Patient 3-5: 5 to 6 Patient 6: 5 to 5 Patient 7: 5 to 4 |
Change of CASE Patient 1: 27 to 1 Patient 6: 22 to 8 Patient 7: 21 to 3 |
No | Death after septic shock: 2 (67) Blood stream infections: 3 (100) urinary tract infections: 3 (100) Tracheobronchitis: 3 (100) Fever: 2 (67) Dyspnea: 1 (33) Tachycardia: 1 (33) |
| Bortezomib | Proteaso-me | Systematic review (24) | NMDAR AE: 29 | Usually used 1.3 mg/m2 administered subcutaneously per cycle | mRS score ≤ 2; n (%) 16 (55) |
NA | NA | Hematological side effects: 8 (28) infectious side effects: 3 (10) Gastrointestinal: 3 (10) |
| Ongoing (NCT03993262) | No antibody specified AE | NA | NA | NA | NA | NA | ||
| Tocilizumab | IL-6R | Case report (25) | CASPR2 AE: 1 | 8 mg/kg every 4 weeks | Full remission | NA | No | No |
| Retrospective cohort (26) | No antibody specified AE: Tocilizumab group:30 Additional rituximab group:31 |
Tocilizumab group: 8mg/kg monthly for >= 2 cycles Additional rituximab group: Tocilizumab 8mg/kg + rituximab 375 mg/m2 monthly |
mRS score ≤ 2; n (%) Tocilizumab group: 18 (60) Additional rituximab group: 7 (23) |
NA | NA | Tocilizumab group: decrement of absolute neutrophil count: 3 (10) |
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| Prospective cohort (27) | NMDAR AE: 52 | Teratoma removal, Steroid, IVIG, Rituximab, and Tocilizumab (Tocilizumab: 8 mg/kg monthly) |
In the subgroup analysis with baseline mRS score of 5 (n=15), 1-year Δ mRS scores: 4 | In the subgroup analysis with baseline of 5 (n=15), 1-year Δ CASE scores: 21 | NA | Pneumonia: 6 Neutropenia: 11 Lymphopenia: 5 Urinary tract infection: 7 Any serious adverse event: 1 |
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| Satralizumab | IL-6R | Ongoing (NCT05503264) | NMDAR/LGI1 AE | NA | NA | NA | NA | NA |
AE, Autoimmune encephalitis; CAAR-T, chimeric autoantibody receptor T cell; IL, interleukin; NMDAR, N-methyl-D-aspartate receptor; LGI1, leucine-rich glioma-inactivated protein 1; CASPR2, contactin-associated protein-like 2; GAD, glutamic acid decarboxylase; SCNAE, serum and cerebrospinal fluid negative AE; NA, not available; CASE, Clinical Assessment Scale for Autoimmune Encephalitis; mRS, Modified Rankin Scale.