Outcomes in 18 patients with CSS treated with a “standard” prednisone/cyclophosphamide treatment with (treatment group) or without (control group) intravenous immunoglobulin synchronised with plasmapheresis. The table shows the values at last assessment for SNVDI, BVAS and modified Rankin scores
| No/Sex/age (years) | Follow up (months) | Relapse | Outcome | Maintaining drug (mg/day) | SNVDI | BVAS | Modified Rankin score |
|---|---|---|---|---|---|---|---|
| BVAS, Birmingham Vasculitis Activity Score; SNVDI, Systemic Necrotising Vasculitis Damage Index; R, remission; SR, treatment free sustained remission for at least 18 months after discontinuation of treatment; MTX, methotrexate, 10 mg/week; PRD, prednisone; DFZ, deflazacort; Methyl-PDN, methyl-prednisolone. | |||||||
| Treatment group | |||||||
| 1/F/55 | 71 | No | SR | No | 5 | 2 | 2 |
| 2/M/69 | 37 | No | R | MTX | 3 | 2 | 2 |
| 3/M/44 | 68 | No | SR | No | 4 | 4 | 1 |
| 4/F/53 | 68 | Yes | SR | No | 4 | 2 | 1 |
| 5/F/57 | 52 | No | R | DFZ (6) | 5 | 4 | 1 |
| 6/F/56 | 44 | No | R | DFZ (6) | 3 | 2 | 1 |
| 7/F/69 | 36 | No | R | Methyl-PDN (4) | 5 | 2 | 1 |
| 8/F/43 | 33 | No | R | DFZ (6) | 3 | 2 | 0 |
| 9/F/33 | 32 | No | R | Methyl-PDN (4) | 2 | 1 | 1 |
| Control group | |||||||
| 10/F/47 | 108 | No | SR | No | 6 | 4 | 3 |
| 11/M/61 | 43 | Yes | R | Methyl-PDN (16) | 11 | 6 | 2 |
| 12/M/28 | 72 | Yes | R | DFZ (9) | 4 | 2 | 1 |
| 13/F/66 | 32 | No | R | PRD (10) | 5 | 4 | 2 |
| 14/M/29 | 36 | No | R | Methyl-PDN (8) | 5 | 3 | 1 |
| 15/F/59 | 42 | No | R | PRD (7.5) | 6 | 6 | 2 |
| 16/F/46 | 60 | No | R | PRD (7.5) | 5 | 6 | 2 |
| 17/M/48 | 66 | Yes | SR | No | 4 | 6 | 2 |
| 18/F/25 | 55 | Yes | R | Methyl-PDN (8) | 4 | 2 | 1 |