TABLE 1.
Summary of literature review regarding demographic data, efficacy, and treatment durability of rituximab in patients with epidermolysis bullosa acquisita
| DEMOGRAPHIC | MECHANOBULLOUS | NON-MECHANOBULLOUS | TOTAL |
|---|---|---|---|
| PATIENTS, N | 23 | 19 | 68 |
| Age at onset, y | |||
| MEAN | 54 | 52.7 | 52.9 |
| RANGE | 28-88 | 20-77 | 17-88 |
| NM | 6 | 6 | 13 |
| Gender, n (%) | |||
| FEMALE | 8 (47.1%) | 13 (72.2%) | 35 (51.5%) |
| MALE | 9 (52.9%) | 5 (27.8%) | 33 (48.5%) |
| NM | 6 | 1 | 0 |
| Previous treatments, n (%) | |||
| SYSTEMIC CORTICOSTEROIDS | 5 (31.2%) | 16 (94.1%) | 32 (60.4%) |
| DAPSONE | 3 (18.8%) | 6 (35.3%) | 16 (30.2%) |
| AZATHIOPRINE | 2 (12.5%) | 3 (17.6%) | 9 (16.9%) |
| MYCOPHENOLATE MOFETIL | 3 (18.8%) | 1 (5.9%) | 9 (16.9%) |
| IVIG | 1 (6.2%) | 0 (0%) | 7 (13.2%) |
| CYCLOSPORINE | 4 (25.0%) | 2 (11.8%) | 9 (16.9%) |
| COLCHICINE | 2 (12.5%) | 1 (5.9%) | 4 (7.5%) |
| OTHERS | 2 (12.5%) | 2 (11.8%) | 8 (15.1%) |
| NM | 7 | 2 | 15 |
| EBA duration until RTX infusion, months | |||
| MEAN | 82.7 | 23.0 | 51.3 |
| RANGE | 6-240 | 0.5-84 | 0.5-240 |
| NM | 6 | 4 | 14 |
| Adjuvant therapy, n (%) | |||
| SYSTEMIC CORTICOSTEROIDS | 5 (31.2%) | 16 (94.1%) | 32 (60.4%) |
| DAPSONE | 3 (18.8%) | 6 (35.3%) | 16 (30.2%) |
| AZATHIOPRINE | 2 (12.5%) | 3 (17.6%) | 9 (16.9%) |
| IVIG | 3 (18.8%) | 1 (5.9%) | 9 (16.9%) |
| COLCHICINE | 1 (6.2%) | 0 (0%) | 7 (13.2%) |
| MYCOPHENOLATE MOFETIL | 4 (25.0%) | 2 (11.8%) | 9 (16.9%) |
| IMMUNOADSORPTION | 2 (12.5%) | 1 (5.9%) | 4 (7.5%) |
| OTHER | 2 (12.5%) | 2 (11.8%) | 8 (15.1%) |
| NM | 7 | 2 | 15 |
| Disease control, n (%) | |||
| YES | 22 (95.6%) | 16 (84.2%) | 63 (92.7%) |
| NO | 1 (4.3%) | 3 (15.8%) | 5 (7.3%) |
| NM | 0 | 0 | 0 |
| Clinical remission, n (%) | |||
| YES | 10 (58.8%) | 11 (64.7%) | 45 (73.8%) |
| NO | 7 (41.2%) | 6 (35.3%) | 16 (26.2%) |
| NM | 6 | 2 | 7 |
| RTX-related side effects, n (%) | |||
| YES | 3 (30%) | 4 (28.6%) | 11 (28.2%) |
| NO | 7 (70%) | 10 (71.4%) | 28 (71.8%) |
| NM | 13 | 5 | 29 |
| Treatment durability | |||
| Relapse, n (%) | 6 (60%) | 3 (23.1%) | 15 (39.5%) |
| Follow-up, m | 23.9 | 24.1 | 23.0 |
| NM | 13 | 6 | 30 |
EBA, epidermolysis bullosa acquisita; RTX, rituximab, IVIg, intravenous immunoglobulin; NM, not mentioned