TABLE 2.
List of studies on the efficacy, safety, and treatment durability of rituximab in treating patients with epidermolysis bullosa acquisita
| STUDY | Dasdar et al11 | ||||||||
| DESIGN | Case series; n=15 | ||||||||
| GENDER | M | M | M | F | F | F | M | ||
| AGE AT DISEASE ONSET OR RTX INFUSION, Y | At onset, 72 | At onset, 46 | At onset, 43 | At onset, 20 | At onset, 47 | At onset, 53 | At onset, 88 | ||
| EBA VARIANT | non-MCB | non-MCB | non-MCB | non-MCB | MCB | non-MCB | MCB | ||
| SITE OF INVOLVEMENT | Mucosal | Mucosal | Mucosal | Mucosal | Mucosal | Mucosal | NM | ||
| EBA DIAGNOSIS | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | ||
| PREVIOUS TREATMENTS | PSL | PSL | PSL | AZT, dapsone | PSL | PSL, dapsone | MTX, Doxycycline | ||
| INDICATION OF RTX | Severe disease | Severe disease | Recalcitrant disease | Recalcitrant disease | Recalcitrant disease | Recalcitrant disease | Recalcitrant disease | ||
| EBA DURATION TO RTX INFUSION | 1m | 1m | 31m | 48m | 18m | 12m | 19m | ||
| RTX PROTOCOL | 4 x 500mg | 4 x 500mg | 4 x 500mg | 2 x 1000mg | 4 x 500mg | 4 x 500mg | 4 x 500mg | ||
| NUMBER OF CYCLES; DURATION OF TREATMENT | 1 cycle | 1 cycle | 1 cycle | 1 cycle | 1 cycle | 1 cycle | 1 cycle | ||
| ADJUVANT TREATMENTS | PSL, MTX | PSL | PSL | PSL, dapsone | PSL | PSL, dapsone | MTX | ||
| Disease control | Yes | Yes | Yes | Yes | Yes | Yes | Yes | ||
| Clinical remission | Yes | Yes | No | No | No | Yes | Yes | ||
| SIDE EFFECT OF RTX THERAPY | No | No | No | No | No | Sore throat | No | ||
| RELAPSE | No | No | No | Yes, 3m | Yes, 4m | Yes, 9m | Yes, 10m | ||
| FOLLOW-UP DURATION FROM RTX | 55m | 13m | 18m | 52m | 38m | 28 | 32m | ||
| OTHER ENDPOINTS | – | – | – | – | – | – | – | ||
| STUDY | Dasdar et al11 | ||||||||
| DESIGN | Case series; n=15 | ||||||||
| GENDER | F | M | M | F | F | F | M | F | |
| AGE AT DISEASE ONSET OR RTX INFUSION, Y | At onset, 45 | At onset, 54 | At onset, 54 | At onset, 29 | At onset, 58 | At onset, 57 | At onset, 50 | At onset, 63 | |
| EBA VARIANT | MCB | MCB | MCB | non-MCB | non-MCB | non-MCB | MCB | non-MCB | |
| SITE OF INVOLVEMENT | Mucosal | Mucosal | Mucosal | Mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Mucosal | Cutaneous and mucosal | |
| EBA DIAGNOSIS | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | |
| PREVIOUS TREATMENTS | PSL, AZT, MTX | PSL, cyclosporine, MMF, colchicine | PSL, MTX | PSL | MTX | PSL | PSL | PSL, MMF | |
| INDICATION OF RTX | Recalcitrant disease | Recalcitrant disease | Side effects of treatments | Severe disease | Recalcitrant disease | Severe disease | Recalcitrant disease | Recalcitrant disease | |
| EBA DURATION TO RTX INFUSION | 60m | 240m | 12m | 2m | 48m | 1m | 7m | 20m | |
| RTX PROTOCOL | 4 x 500mg | 4 x 500mg | 2 x 1000mg | 4 x 500mg | 2 x 1000mg | 4 x 500mg | 4 x 500mg | 4 x 500mg | |
| NUMBER OF CYCLES; DURATION OF TREATMENT | 1 cycle | 1 cycle | 2 cycles | 1 cycle | 1 cycle | 1 cycle | 1 cycle | 1 cycle | |
| ADJUVANT TREATMENTS | - | Dapsone | MMF, dapsone | PSL, MMF, dapsone | PSL, MTX | PSL | PSL, MTX | PSL, MMF, dapsone | |
| DISEASE CONTROL | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes | |
| CLINICAL REMISSION | Yes | No | Yes | Yes | No | Yes | No | Yes | |
| SIDE EFFECT OF RTX THERAPY | Fever, cough, COVID-19 | No | No | Urticaria, angioedema, arthralgia, edema | No | Nausea, mild headache | No | Blood pressure rise, dyspnea | |
| RELAPSE | Yes, 20m | No | No | No | No | No | Yes, 11 m | No | |
| FOLLOW-UP DURATION FROM RTX | 42m | 15m | 33m | 19m | 6m | 24m | 24m | 44m | |
| OTHER ENDPOINTS | – | – | – | – | – | – | – | – | |
| STUDY | Gordilho et al12 | Han et al13 | Koszegi et al14 | Szymański et al15 | Schauer et al16 | Mendes et al5 | |||
| DESIGN | Case series; n=7 | Case report; n=1 | Case report; n=1 | Case series; n=1 | Case series; n=2 | Case report; n=1 | |||
| GENDER | 4F, 3M | M | M | F | F | F | M | ||
| AGE AT DISEASE ONSET OR RTX INFUSION, Y | At onset, 51 (median) | At onset, 44 | At infusion, 58 | At onset, 66 | At onset, 28 | At onset, 67 | At onset, 69 | ||
| EBA VARIANT | 6MCB, 1 non-MCB | NM | NM | MCB | MCB | MCB | NM | ||
| SITE OF INVOLVEMENT | Cutaneous and mucosal | Cutaneous | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | ||
| EBA DIAGNOSIS | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | NM | Histopathologic examination, direct and indirect immunofluorescence | Direct and indirect immunofluorescence, ELISA | Direct and indirect immunofluorescence, ELISA, immunoblotting | Histopathologic examination, direct immunofluorescence, ELISA | ||
| PREVIOUS TREATMENTS | PSL (n=7), MMF (n=7), dapsone (n=4), cyclosporine (n=2), AZT (n=1) | PSL, MMF | PSL, cyclosporine, dapsone, tetracycline, AZT, plasmapheresis | Tetracycline, PSL, dapsone, Clobetasol propionate | Systemic CS, AZT, MMF, chloroquine | CS pulse therapy, AZT | Methylprednisolone, dapsone, AZT, topical therapy | ||
| INDICATION OF RTX | Severe disease | Recalcitrant disease | Recalcitrant disease | Side effects of treatments | Recalcitrant disease | Recalcitrant disease, side-effect of previous treatments | Recalcitrant disease, side-effect of previous treatments | ||
| EBA DURATION TO RTX INFUSION | 42m (median) | NM | NM | 6y | 10y | 6m | NM | ||
| RTX PROTOCOL | NM | 2 x 1000mg at two weeks interval | NM | 2 x 1000mg at two weeks interval | 2 x 1000mg at two weeks interval | 2 x 1000mg at two weeks interval | 4 x 375mg/m2 at one week interval | ||
| NUMBER OF CYCLES; DURATION OF TREATMENT | 3 cycles (1-9 cycles) | 1 cycle | 4 cycles | 1 cycle | 1 cycle | 1 cycle | 1 cycle | ||
| ADJUVANT TREATMENTS | NM | NM | NM | PSL | IVIg at one month interval, topical treatment | 2 x IVIg, topical treatment | NM | ||
| DISEASE CONTROL | Yes | Yes | Yes | Yes | Yes | No | Yes | ||
| CLINICAL REMISSION | Complete remission (n=3), partial remission (n=3) | Yes | Yes | Yes | No | No | No | ||
| SIDE EFFECT OF RTX THERAPY | NM | NM | NM | NM | NM | Poorly tolerated | NM | ||
| RELAPSE | NM | NM | NM | Yes | NM | NM | NM | ||
| FOLLOW-UP DURATION FROM RTX | NM | 36m | 27m | 15m | NM | NM | NM | ||
| OTHER ENDPOINTS | – | – | – | – | – | - | – | ||
| STUDY | Figueredo et al17 | Dubois et al18 | Yang et al19 | Bevans et al6 | Lamberts et al20 | ||||
| DESIGN | Case report; n=1 | Case report; n=1 | Case report; n=1 | Case series; n=3 | Case series; n=5 | ||||
| GENDER | F | M | M | M | M | F | F | F | F |
| AGE AT DISEASE ONSET OR RTX INFUSION, Y | At onset, 68 | At onset, 30 | At onset, 49 | At onset, 85 | At onset, 52 | At onset, 52 | At infusion, 59 | At infusion, 87 | At infusion, 56 |
| EBA VARIANT | non-MCB | NM | MCB | NM | NM | NM | non-MCB | non-MCB | non-MCB |
| SITE OF INVOLVEMENT | Cutaneous and mucosal (esophageal stricture) | Cutaneous and mucosal | Cutaneous and mucosal (significant involvement of the esophagus and alimentary tract) | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | NM | NM | NM |
| EBA DIAGNOSIS | Histopathologic examination, direct immunofluorescence | Direct immunofluorescence, ELISA | Skin biopsy, direct and indirect immunofluorescence, immunoblotting | Histopathologic examination, direct and indirect immunofluorescence, ELISA | Histopathologic examination, direct and indirect immunofluorescence | ||||
| PREVIOUS TREATMENTS | Systemic CS, dapsone | PSL, AZT, MMF, dapsone, IVIg | PSL, AZT, cyclosporine, dapsone, tetracycline, photophoresis, plasmapheresis | PSL, AZT, dapsone, IVIg | PSL, AZT, MMF, IVIg | P, dapsone, MMF | PSL, dapsone, AZT, CP, MMF, IVIg | PSL, AZT, intravenous CS | PSL, AZT, dapsone, doxycycline |
| INDICATION OF RTX | Recalcitrant disease | Recalcitrant disease, side-effect of previous treatments | Recalcitrant disease, side-effect of previous treatments | NM | NM | NM | Recalcitrant disease, side-effect of previous treatments | ||
| EBA DURATION TO RTX INFUSION | 3y | 2y | 18y | 20m | 6m | 4m | Mean, 29.1 (0.5-84m) | ||
| RTX PROTOCOL | NM | 2 x 1000mg at two weeks interval | 4 x 375mg/m2 at one week interval (first and second cycles) | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 2 x 1000mg at two weeks interval (first cycle) 4 x 375mg/m2 at one week interval (second, third, and fourth cycles) | 2 x 500mg at two weeks interval | 2 x 500mg at two weeks interval | 2 x 1000mg at two weeks interval + 2 x 500mg at months 6 and 12 |
| NUMBER OF CYCLES; DURATION OF TREATMENT | 1 cycle | 3 cycles;1 y | 2 x 1000mg at two weeks interval (third cycles) | 2 cycles | 2 cycles | 4 cycles | 1 cycle | 1 cycle | 1 cycle |
| ADJUVANT TREATMENTS | 5 x IVIg | NM | 3 cycles; 3 y | PSL, AZT, dapsone | PSL, MMF | P, D, MMF | Topical CS, PSL | PSL, AZT | Topical CS, PSL, AZT |
| DISEASE CONTROL | Yes | Yes | NM | Yes | Yes | Yes | No | No | Yes |
| CLINICAL REMISSION | Yes | Yes | Yes | Yes | Yes | Yes | No | No | Yes |
| SIDE EFFECT OF RTX THERAPY | NM | NM | Yes | NM | NM | NM | No | No | No |
| RELAPSE | No | No | Yes | Yes, 12m | No | Yes, 5m | NM | NM | No |
| FOLLOW-UP DURATION FROM RTX | 9m | 3y | NM | 20m | 60m | 22m | 79m | 2m | 22m |
| OTHER ENDPOINTS | – | – | – | – | – | – | – | – | – |
| STUDY | Lamberts et al20 | Oktem et al7 | |||||||
| DESIGN | Case series; n=5 | Case series; n=5 | |||||||
| GENDER | M | F | F | M | M | M | F | ||
| AGE AT DISEASE ONSET OR RTX INFUSION, Y | At infusion, 25 | At infusion, 43 | At onset, 61 | At onset, 56 | At onset, 31 | At onset, 55 | At onset, 50 | ||
| EBA VARIANT | non-MCB | non-MCB | NM | NM | NM | NM | NM | ||
| SITE OF INVOLVEMENT | NM | NM | Cutaneous and mucosal | Mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | ||
| EBA DIAGNOSIS | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence | |||||||
| PREVIOUS TREATMENTS | PSL, AZT, dapsone, MMF | PSL, dapsone | CS, AZT, dapsone, IVIg, colchicine, extracorporeal photochemotherapy | CS, AZT, dapsone, cyclosporine, colchicine, extracorporeal photochemotherapy | CS, cyclosporine, dapsone, IVIg, colchicine | CS, AZT, dapsone, extracorporeal photochemotherapy | CS, cyclosporine, MMF, colchicine | ||
| INDICATION OF RTX | Recalcitrant disease, side-effect of previous treatments | Recalcitrant disease | Recalcitrant disease | Recalcitrant disease | Recalcitrant disease | Recalcitrant disease | |||
| EBA DURATION TO RTX INFUSION | Mean, 29.1 (0.5- 84m) | 20y | 10y | 6y | 6y | 2y | |||
| RTX PROTOCOL | 2 x 1000mg at two weeks interval + 2 x 500mg at months 6 and 12 | 2 x 1000mg at two weeks interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 3 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | ||
| NUMBER OF CYCLES; DURATION OF TREATMENT | 1 cycle | 1 cycle | 1 cycle | 1 cycle | 1 cycle | 2 cycles; 2 m | 1 cycle | ||
| ADJUVANT TREATMENTS | Topical CS, PSL, AZT | Topical CS, PSL, dapsone | 24 x IVIg at one month interval, | 25 x IVIg at one month interval, colchicine | 26 x IVIg at one month interval, | 12 x IVIg at one month interval, colchicine | 10 x IVIg at one month interval, | ||
| DISEASE CONTROL | Yes | No | Yes | Yes | Yes | Yes | Yes | ||
| CLINICAL REMISSION | Yes | No | NM | NM | NM | NM | NM | ||
| SIDE EFFECT OF RTX THERAPY | No | No | No | Fever, shivering, and an urticaria-like | No | No | No | ||
| RELAPSE | No | NM | NM | NM | NM | NM | NM | ||
| FOLLOW-UP DURATION FROM RTX | 14m | 43m | 25m | 26m | 28m | 24m | 10m | ||
| OTHER ENDPOINTS | – | – | ABSIS score: skin: Baseline, 33.8 (18-49.5) After treatment,12.9 (1-23.5) Mucosa: Baseline, 1.2 (0-3); After treatment, 0 Oral: Baseline, 11.7 (0-23.5) After treatment, | ||||||
| STUDY | Brassat et al21 | Iranzo et al22 | Kolesnik et al23 | McKinley et al24 | Pickert et al25 | Li et al26 | |||
| DESIGN | Case report; n=1 | Case series; n=2 | Retrospective cohort; n=1 | Case report; n=1 | Case report; n=1 | Case report; n=1 | |||
| GENDER | M | M | M | F | M | M | F | ||
| AGE AT DISEASE ONSET OR RTX INFUSION, Y | At onset, 46 | At onset, 28 | At onset, 33 | At onset, 71 | At onset, 13 | At onset, 35 | At onset, 54 | ||
| EBA VARIANT | NM | non-MCB | Mixed-type | NM | NM | MCB | NM | ||
| SITE OF INVOLVEMENT | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | NM | Cutaneous and mucosal | Cutaneous | Cutaneous | ||
| EBA DIAGNOSIS | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence, immunoblotting | Histopathologic examination, direct immunofluorescence, ELISA | Histopathologic examination, direct and indirect immunofluorescence | Direct and indirect immunofluorescence, ELISA | Histopathologic examination, direct immunofluorescence | |||
| PREVIOUS TREATMENTS | PSL, dapsone, MMF | NM | NM | PSL, dapsone | Topical CS, PSL, dapsone, tetracycline | - | Topical clobetasol and tacrolimus, cyclosporine, MMF, PSL, etanercept, IVIg, CP | ||
| INDICATION OF RTX | Recalcitrant disease, contradiction of conventional immunosuppressive | NM | NM | Recalcitrant disease, side-effect of previous treatments | CS dependency | NM | Recalcitrant disease, side-effect of previous treatments | ||
| EBA DURATION TO RTX INFUSION | 2m | NM | NM | 8y | 15m | 2y | 2.5y | ||
| RTX PROTOCOL | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at days 4, 11, 18, and 25 | 2 x 1000mg at four months interval | RTX at one month interval | 4 x 375mg/m2 at one week interval | ||
| NUMBER OF CYCLES; DURATION OF TREATMENT | 1 cycle | 2 cycles | NM | 1 cycle | 1 cycle | 1 cycle | 1 cycle | ||
| ADJUVANT TREATMENTS | PSL | NM | NM | 11 x protein A immunoapheresis at days 1, 2, 3 and weekly for 8 weeks, PSL, dapsone, AZT | Dapsone | IVIg at one month interval | PSL | ||
| DISEASE CONTROL | Yes | Yes | Yes | Yes | Yes | Yes | Yes | ||
| CLINICAL REMISSION | NM | Yes | Yes | Yes | Yes | Yes | Yes | ||
| SIDE EFFECT OF RTX THERAPY | NM | NM | NM | No | NM | NM | No | ||
| RELAPSE | NM | NM | NM | No | No | NM | No | ||
| FOLLOW-UP DURATION FROM RTX | NM | 36m | NM | 37m | 2y | NM | 16m | ||
| OTHER ENDPOINTS | – | – | – | Bullous pemphigoid disease area index: Before, 20, After 4 w, 0 | – | – | – | ||
| STUDY | Le Roux-Villet et al27 | Kim et al28 | Kubisch et al29 | ||||||
| DESIGN | Prospective study; n=5 | Case report; n=1 | Case report; n=1 | ||||||
| GENDER | F | F | F | F | M | F | F | ||
| AGE AT DISEASE ONSET OR RTX INFUSION, Y | At onset, 56 | At onset, 17 | At onset, 30 | At onset, 79 | At onset, 70 | At onset, 64 | At onset, 71 | ||
| EBA VARIANT | NM | NM | NM | NM | NM | MCB | non-MCB | ||
| SITE OF INVOLVEMENT | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | ||
| EBA DIAGNOSIS | Histopathologic examination, direct and indirect immunofluorescence, immunoblotting | Histopathologic examination, direct and indirect immunofluorescence, ELISA | Histopathologic examination, direct and indirect immunofluorescence, immunoblotting | ||||||
| PREVIOUS TREATMENTS | CP, MTX | dapsone, cyclosporine, AZT, PSL, sulfasalazine | cyclosporine, PSL, sulfasalazine | dapsone, PSL, sulfasalazine | dapsone, CP, MMF, IVIg, PSL | PSL, AZT, dapsone, MTX, colchicine | PSL, dapsone, topical CS, colchicine | ||
| INDICATION OF RTX | Recalcitrant disease, contradiction of conventional immunosuppressive | Recalcitrant disease | Side-effect of previous treatments | ||||||
| EBA DURATION TO RTX INFUSION | 12m | 168m | 24m | 6m | 16m | 4y | NM | ||
| RTX PROTOCOL | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at days 9, 16, 23, and 30 | ||
| NUMBER OF CYCLES; DURATION OF TREATMENT | 1 cycle | 1 cycle | 1 cycle | 1 cycle | 1 cycle | 1 cycle | 1 cycle | ||
| ADJUVANT TREATMENTS | MTX | Dapsone, AZT, sulfasalazine | cyclosporine, PSL | Dapsone, sulfasalazine | Dapsone, PSL | Dapsone, colchicine, methylprednisolone | 7 x protein A immunoapheresis at days 1, 2, 3, 8, 15, 22, and 29, PSL, AZT | ||
| DISEASE CONTROL | Yes | Yes | Yes | Yes | Yes | Yes | Yes | ||
| CLINICAL REMISSION | Yes | Yes | Yes | Yes | Yes | Yes | Yes | ||
| SIDE EFFECT OF RTX THERAPY | No | No | Pneumocystis jirovecii pneumonia, death | No | No | No | NM | ||
| RELAPSE | Yes, 12m | Yes, 8m | Yes, 4m | Yes, 10m | No | No | NM | ||
| FOLLOW-UP DURATION FROM RTX | 18m | 26m | 4m | 10m | 12m | 10m | NM | ||
| OTHER ENDPOINTS | – | – | – | – | – | – | – | ||
| STUDY | Meissner et al30 | Saha et al31 | Cavailhes et al32 | Wallet-Faber et al33 | Sadler et al34 | Niedermeier et al35 | |||
| DESIGN | Case report; n=1 | Case report; n=1 | Case report; n=1 | Case report; n=1 | Case report; n=1 | Case series; n=2 | |||
| GENDER | M | F | M | F | F | M | M | ||
| AGE AT DISEASE ONSET OR RTX INFUSION, Y | At onset, 50 | At onset, 54 | At onset, 76 | At onset, 77 | At onset, 71 | At onset, 63 | At onset, 33 | ||
| EBA VARIANT | MCB | MCB | NM | non-MCB | MCB | MCB | MCB | ||
| SITE OF INVOLVEMENT | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | ||
| EBA DIAGNOSIS | Histopathologic examination, direct and indirect immunofluorescence, immunoblotting, ELISA | Histopathologic examination, indirect immunofluorescence, immunoblotting | Histopathologic examination, direct and indirect immunofluorescence, ELISA | Skin biopsy, direct and indirect immunofluorescence, immunoblotting | Histopathologic examination, direct and indirect immunofluorescence, immunoblotting | Histopathologic examination, indirect immunofluorescence, immunoblotting | |||
| PREVIOUS TREATMENTS | IVIg, MMF, colchicine | PSL, AZT, dapsone, cyclosporine, CP, MMF, IVIg, methylprednisolone | Topical CS, dapsone, topical tacrolimus, systemic CS, MMF, MTX, doxycycline | PSL, MMF, clobetasol, | Systemic CS, AZT, cyclosporine, MMF, colchicine, plasmapheresis, gold preparations, IVIg, daclizumab | AZT, MMF, cyclosporine, dapsone, CP, MTX, CS pulse therapy, photophoresis, leflunomide | PSL, AZT, dapsone, MMF, CP, cyclosporine, MTX, CS pulse therapy, IVIg, leflunomide | ||
| INDICATION OF RTX | Recalcitrant disease | Recalcitrant disease | Recalcitrant disease, CS dependency | Side-effect of previous treatments | Recalcitrant disease | Recalcitrant disease | Recalcitrant disease | ||
| EBA DURATION TO RTX INFUSION | 12y | 16y | 2y | NM | 6y | 4y | 9y | ||
| RTX PROTOCOL | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 5 x 144 mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | ||
| NUMBER OF CYCLES; DURATION OF TREATMENT | 1 cycle | 3 cycles; 1 y | 1 cycle | 1 cycle | 1 cycle | 1 cycle | 1 cycle | ||
| ADJUVANT TREATMENTS | Systemic CS, AZT | MMF | PSL | PSL | AZT | 2x immunoadsorpti on at one month interval, MMF | 2x immunoadsorpti on at one month interval, MMF | ||
| DISEASE CONTROL | Yes | Yes | Yes | Yes | Yes | Yes | Yes | ||
| CLINICAL REMISSION | Yes | No | Yes | NM | Yes | No | Yes | ||
| SIDE EFFECT OF RTX THERAPY | NM | Skin sepsis | NM | NM | No | NM | NM | ||
| RELAPSE | No | NM | No | Yes, 10m | Yes | NM | NM | ||
| FOLLOW-UP DURATION FROM RTX | 6m | NM | 1y | 10m | 2y | 6m | 9m | ||
| OTHER ENDPOINTS | – | – | – | – | - | ABSIS Score for Oral Mucosa: Before, 11 After, 11 Body Surface; Area Representing Disease Activity: Before, 9, After, 9 | ABSIS Score for Oral Mucosa: Before, 0, After, 0; Body Surface Area Representing Disease Activity: Before, 13, After, 2 | ||
| STUDY | Mercader et al36 | Crichlow et al37 | Schmidt et al38 | ||||||
| DESIGN | Case report; n=1 | Case report; n=1 | Case report; n=1 | ||||||
| GENDER | M | F | M | ||||||
| AGE AT DISEASE ONSET OR RTX INFUSION, Y | At onset, 72 | At onset, 58 | At onset, 46 | ||||||
| EBA VARIANT | NM | NM | NM | ||||||
| SITE OF INVOLVEMENT | Cutaneous and mucosal | Cutaneous and mucosal | Cutaneous and mucosal | ||||||
| EBA DIAGNOSIS | Histopathologic examination, direct and indirect immunofluorescence | Histopathologic examination, direct and indirect immunofluorescence, immunoblotting | Histopathologic examination, direct and indirect immunofluorescence, immunoblotting | ||||||
| PREVIOUS TREATMENTS | PSL, AZT, dapsone, MMF, IVIg, | PSL, AZT, MMF, cyclosporine, IVIg, Methylprednisolone | PSL, AZT, dapsone, colchicine, topical betamethasone, immunoadsorption | ||||||
| INDICATION OF RTX | Recalcitrant disease, side-effect of previous treatments | Recalcitrant disease, side-effect of previous treatments | Recalcitrant disease | ||||||
| EBA DURATION TO RTX INFUSION | 7m | 3y | 5w | ||||||
| RTX PROTOCOL | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | 4 x 375mg/m2 at one week interval | ||||||
| NUMBER OF CYCLES; DURATION OF TREATMENT | 1/4 cycle | 1 cycle | 1 cycle | ||||||
| ADJUVANT TREATMENTS | NM | PSL, MMF | PSL, AZT, colchicine | ||||||
| DISEASE CONTROL | No | Yes | Yes | ||||||
| CLINICAL REMISSION | No | Yes | Yes | ||||||
| SIDE EFFECT OF RTX THERAPY | Pneumonia, septic shock, death | No | Deep vein thrombosis | ||||||
| RELAPSE | NM | No | No | ||||||
| FOLLOW-UP DURATION FROM RTX | 10d | 1y | 1y | ||||||
| OTHER ENDPOINTS | - | - | - | ||||||
RTX, rituximab; EBA, epidermolysis bullosa acquisita; non-MB, non-mechanobullous; MB, mechanobullous; ELISA, enzyme-linked immunosorbent assay; PSL, prednisolone; AZT, azathioprine; MTX, methotrexate; MMF, mycophenolate mofetil; CS, corticosteroids; IVIg, intravenous immunoglobulin; CP, cyclophosphamide; y, year; m, months; w, week; NM, not mentioned.