Table 2.
Novel Biologics in the treatment of BP.
| Novel biologics | Patients number/therapeutic targets | Dose regime | Concomitant therapies | Efficacy | Adverse reactions | References |
|---|---|---|---|---|---|---|
| Rituximab | 13 | 500 mg weekly for 4 weeks | Pred | >90% | Infections | (37) |
| 12 | 375 mg/m2 weekly for 4 weeks | IVIG | 100% | None | (38) | |
| 28 | 500 or 1,000 mg on day 1 and day 15 | None | 67.9% | None | (39) | |
| 62 | Initial dose of 375 mg/m2 every 1–4 weeks to 500 mg weekly for 2 weeks | None | 85% | Infections, anemia, neutropenia, syndrome of inappropriate antidiuretic hormone secretion (SIADH), drug fever, acute pruritus, peripheral arterial occlusive disease and tachycardia. |
(2) | |
| 20 | 375 mg/m2 weekly for 4 weeks or 1,000 mg on day 1 and day 15 | Pred, MMF, AZA, MTX | 75% | infections | (40) | |
| 38 | 375 mg/m2 weekly for 4 weeks or 1,000 mg on day 1 and day 15 | Pred | 76% | None | (41) | |
| Omalizumab | 1 | 300 mg every 2 weeks | NA | Disease control | NA | (42) |
| 1 | 300 mg every 2 and 4 weeks | NA | Disease control | NA | (43) | |
| 1 | 300 mg every 4 to 8 weeks | AZA, pred | Complete remission | None | (44) | |
| 6 | 375 mg every 2 weeks and 300 mg every 8 weeks | Pred, AZA | 2 complete remission, 3 symptom-free, 1 terminated due to intercurrent disease | COPD exacerbation due to termination of Pred, epigastric pain, mild elevation of liver enzymes | (45) | |
| 2 | 300 mg every 3 to 4 weeks; 300 mg every three weeks | Pred | Free of pruritus and few blisters | None | (3) | |
| 1 | 300mg every 4 weeks | Pred | Complete remission | Thrombocytopenia | (46) | |
| 11 | 300 mg every 2 to 4 weeks and 300 mg every 8 weeks | Pred, AZA, MP, TCS | 6 Complete remission, 1 partial remission, 4 not available | Elevation of liver enzymes, thrombocytopenia, two myocardial infarctions not directly due to omalizumab | (47) | |
| 1 | 300 mg monthly | TCS | Complete remission | None | (48) | |
| Complement system inhibitors | ||||||
| Nomacopan | 9/complement 5a and leukotriene B4 inhibitor | 90 mg day 1 and 30 mg daily until day 42 | None | 7 of 9 patients with a decreased BPDAI score | NA | (49) |
| Avdoralimab | 40/anti-C5aR1 antibody | 3 subcutaneous injections of avdoralimab every week for 12 weeks | TCS | ongoing | ongoing | NCT04563923 |
| Bertilimumab | 11/eotaxin-1 inhibitor | Intravenous (10 mg/kg), 3 courses biweekly | Pred | 81% reduction in disease severity | NA | NCT02226146 |
| IL-5 inhibitors | ||||||
| Mepolizumab | 30/IL-5 inhibitor | 750 mg every 4 weeks for 4 months | Pred | No superiority of mepolizumab versus placebo | None | (50) |
| Benralizumab | 120/IL-5R α-subunit inhibitor | Benralizumab subcutaneous loading dose followed by repeat dosing | Pred | ongoing | ongoing | NCT04612790 |
| IL-17 and IL-23 inhibitors | ||||||
| Ixekizumab | 4/IL-17A inhibitors | 160 mg subcutaneously at day 0, 80 mg at weeks 2, 4, 6, 8, 10, and 12 | None | Lack of benefit | NA | NCT03099538 |
| Ustekinumab | 18/IL-12/23 p40 subunit inhibitor | 90 mg subcutaneously at day 0 and week 4 and 16 | TCS | Ongoing | Ongoing | NCT04117932 |
| Tildrakinumab | 16/IL-23 inhibitor | 160 mg at week 0, 4, and 16 | None | NA | NA | NCT04465292 |
| Ligelizumab | 20/anti-IgE monoclonal antibody | 240 mg subcutaneously every 2 weeks | None | Predefined efficacy was not met | NA | NCT01688882 |
| AC-203 | 10/IL-1β modulator | Ointment applied twice daily | None | Terminated with partial enrollment completed | NA | NCT03286582 |
BP, bullous pemphigoid; IL, interleukin, Pred, prednisone; IVIG, intravenous immunoglobulin; TCS, topical corticosteroid; NA, not available; TwHF, Tripterygium wilfordii Hook F; MP, methylprednisolone; BPDAI, The Bullous Pemphigoid Disease Area Index; MMF, mycophenolate mofetil; AZA, azathioprine; MTX, methotrexine.