Table 1.
Clinical and immunological characteristics of the pemphigus vulgaris (PV) patients on adjuvant treatment with rituximab.
| Patients | Treatment(1) | Sex | Age | Clinical phenotype of PV(2) | Severity(3) | IgG autoantibody profile(4) | ||
|---|---|---|---|---|---|---|---|---|
| Mucosa | BSA (%) | Anti-Dsg1 | Anti-Dsg3 | |||||
| PV1 | Pred., MMF, Dapson | m | 53 | mucocutaneous | 2 | 3 | 103 | 549 |
| PV2 | Pred., MMF, MTX | m | 67 | mucocutaneous | 4 | 1 | 10 | 484 |
| PV3 | Pred., MMF, Azathioprin | f | 69 | mucosal | 7 | 0 | neg | 810 |
| PV4 | Pred. | f | 69 | no mucosal involvement | 0 | 4 | 163 | 2368 |
| PV5 | Pred. MMF, Azathioprin | f | 37 | mucocutaneous | 3 | 5 | 183 | 94 |
| PV6 | n.d. | m | 48 | n.d. | n.d. | n.d. | 979 | 906 |
| PV7 | Pred., MMF | m | 50 | mucocutaneous | 8 | 1 | 82 | 201 |
| PV8 | Pred., MMF, Dapson | f | 23 | mucocutaneous | 2 | 12 | 357 | 145 |
| PV10 | Pred., MMF | f | 57 | mucosal | 6 | 0 | 3 | 124 |
| PV11 | Pred., MMF, MTX | f | 28 | mucosal | 5 | 0 | 6 | 229 |
| PV12 | Pred., MMF | f | 54 | mucocutaneous | 7 | 5 | 199 | 175 |
| PV13 | n.d. | m | 62 | mucocutaneous | 7 | 10 | 78 | 220 |
| PV14 | Pred. | m | 79 | mucocutaneous | 6 | 10 | 170 | 200 |
| PV15 | Pred. | m | 36 | mucocutaneous | 4 | 60 | 155 | 201 |
| PV16 | n.d. | m | 64 | mucocutaneous | 6 | 20 | 163 | 58 |
| PV17 | Pred. | f | 51 | mucocutaneous | 5 | 10 | 181 | 209 |
| PV18 | Pred. | f | 70 | mucocutaneous | 4 | 10 | neg | 143 |
| PV19 | n.d. | m | 59 | mucocutaneous | 4 | 10 | 98 | 156 |
| PV20 | Pred. | m | 38 | mucocutaneous | 4 | 20 | 36 | 138 |
| PV21 | Pred. | f | 41 | mucocutaneous | 5 | 10 | 18 | 119 |
| PV22 | Pred. | m | 24 | mucocutaneous | 4 | 20 | 138 | 135 |
| PV23 | Pred. | f | 50 | Mucocutaneous | 5 | 10 | 176 | 163 |
(1)Systemic prednisolone (Pred) was administered throughout the observation period and was logarithmically tapered upon clinical reponse to treatment; azathioprine (AZA) or mycophenolate mofetil (MMF), respectively, was also administered throughout the observation period. n.d., not determined.
(2)Before treatment with rituximab.
(3)Determined by the extent of cutaneous involvement as body surface area (BSA) and the extent of mucosal involvement.
(4)Determined by enzyme-linked immunosorbent assay (ELISA) with recombinant desmoglein (Dsg) 1 and 3; optical densities are expressed as PIV (protein index value).
(5)PIV cutoff is 20.