Table 3.
Outcome of rituximab therapy in patients with mucous membrane pemphigoid.
| Characteristics | All MMPN = 109 | |
|---|---|---|
| Follow-up duration (months), median (range) | 51.4 | (1.2–132.8) |
| Total number of RTX cycles, median (IQR) | 4.0 | (4.0) |
| Total number of RTX injections, median (IQR) | 6.0 | (6.0) |
| Number of RTX cycles within 1st year after baseline, med (IQR) | 2 | (1–4) |
| Number of RTX injections within 1st year after baseline, med (IQR) | 4 | (1–7) |
| Patients with at least 2 cycles, N (%) | 97 | (89.0) |
| Time duration between the first two RTX cycles (months), median (IQR) | 6.1 | (2.9) |
| Patients with less than 4 months between the first two RTX cycles, N (%) | 24 | (22.0) |
| Disease control, N (%) | 97 | (89.0) |
| Time to DC (months), median survival (SD) | 7.1 | (0.5) |
| Number of RTX cycles to achieve DC, median (range) | 1 | (1–6) |
| Time to disease control according to the site | ||
| Ocular, median survival (SD) | 6.3 | (2.0) |
| Larynx, median survival (SD) | 8.2 | (0.7) |
| Skin, median survival (SD) | 3.7 | (1.1) |
| Buccal, median survival (SD) | 5.7 | (0.6) |
| Genital, median survival (SD) | 5.7 | (1.4) |
| Anal, median survival (SD) | 3.5 | (0.2) |
| CR, N (%) | 93 | (85.3) |
| Time to CR (months), median survival (SD) | 12.2 | (0.4) |
| Number of cycles to achieve CR, median (range) | 2 | (1–7) |
| Number of injections to achieve CR, median (range) | 4 | (2–14) |
| Patients in CR after one cycle, N (%) | 29 | (26.6) |
| Patients in CR after one or two cycles, N (%) | 67 | (61.5) |
| CR achieved within 1st year after baseline, N (%) | 49 | (45.0) |
| Time duration in CR (months), median (range) | 28.9 | (0.7–110.4) |
| CR off RTX, N (%) | 64 | (58.7) |
| Time duration in CR off RTX (months), median (range) | 24.9 | (0.5-101.3) |
| CR off RTX with minimal therapy, N (%) | 24 | (22.0) |
| Time duration in CR off RTX with minimal therapy (months), median (range) | 21.1 | (2.4–70-9) |
| CR off RTX with more than minimal therapy, N (%) | 40 | (36.7) |
| Time duration in CR off RTX with more than minimal therapy (months), median (range) | 21.7 | (0.5–101.3) |
| CR on RTX, N (%) | 29 | (26.6) |
| Time duration in CR on RTX (months), median (range) | 11.7 | (0.7–78.9) |
| Relapses after CR | ||
| First relapse¶, N (%) | 36 | (38.7) |
| MMPDAI at relapse†, median (range) | 2.2 | (0.0–15.0) |
| Number of sites involved†, median (range) | 1 | (1–3) |
| Ocular†, N (%) | 16 | (44.4) |
| Laryngeal†, N (%) | 7 | (19.4) |
| Buccal†, N (%) | 11 | (30.6) |
| Skin†, N (%) | 9 | (25.0) |
| Genital†, N (%) | 1 | (2.8) |
| CR after relapse treatment†, N (%) | 33 | (91.7) |
| Number of RTX cycles to achieve CR†, median (range) | 1 | 0-4 |
| Time to CR†, median (range) | 5.6 | (3.0–37.8) |
| Second relapse¶, N (%) | 9 | (9.7) |
| ≥2 relapses¶, N (%) | 1 | (1.1) |
| Partial response, N (%) | 11 | (10.1) |
| No response, N (%) | 5 | (4.6) |
| Concomitant therapies at last follow-up, N (%) | 106 | (97.2) |
| DDS and/or sulfasalazine, N (%) | 99 | (90.8) |
| Topical corticosteroids‡, N (%) | 1 | (0.9) |
| Systemic corticosteroids, N (%) | 1 | (0.9) |
MMP, mucous membrane pemphigoid; RTX, rituximab; IQR, interquartile range; DC, disease control; SD, standard deviation; CR, complete remission; MMPDAI, mucous membrane pemphigoid disease activity index; DDS, dapsone. *based on 93 cases who had CR; †based on 36 cases who had a relapse; ‡cutaneous application of more than 10g/d of high potent topical corticosteroids. ¶ corresponds to the definition of * based on 93 cases in CR.