Baseline characteristics in 18 patients with CSS treated with a “standard” prednisone/cyclophosphamide treatment with (treatment group) or without (control group) intravenous immunoglobulin synchronised with plasmapheresis. Five and four patients in each group (numbers 1–5 and 10–13), whose FFS values were ⩾1, received oral cyclophosphamide for 6 months (see Methods for details)
| Sex/age | Clinical features at the onset of involvement | Kind of PNS or CNS | FFS | BVAS | Modified Rankin score | MPO-ANCA |
|---|---|---|---|---|---|---|
| *Patient 18 had no neuropathy. FFS, five factor score; BVAS, Birmingham Vasculitis Activity Score; MM, mononeuritis multiplex, MN, mononeuritis; AP, asymmetrical polyneuropathy; ILD, interstitial lung disease; TT, tibiotarsic joints. Renal impairment: serum creatinine levels of 125–249 μmol/l and/or proteinuria of 0.2–1 g/24 h. | ||||||
| Treatment group | ||||||
| 1/F/55 | Systemic symptoms, skin ulcers, sinusitis, alveolar opacities, ILD, myocardial infarct | AP | 1 | 30 | 4 | + |
| 2/M/69 | Systemic symptoms, skin vasculitis, sinusitis, alveolar opacities, ILD, proteinuria, TT arthritis | AP | 1 | 28 | 4 | + |
| 3/M/44 | Systemic symptoms, palpable purpura, nasal polyposis, pleurisy, myocarditis | MM | 1 | 24 | 3 | + |
| 4/F/53 | Skin ulcers, retinal haemorrhage, sinusitis, alveolar opacities, proteinuria | AP | 1 | 29 | 2 | – |
| 5/F/57 | Systemic symptoms, alveolar opacities, pleurisy, pericarditis, myocarditis | AP | 1 | 26 | 3 | – |
| 6/F/56 | Sinusitis, alveolar opacities | AP | 0 | 15 | 3 | – |
| 7/F/69 | Sinusitis, alveolar opacities, TT arthritis | MM, diplopia | 0 | 16 | 3 | – |
| 8/F/43 | Systemic symptoms, nasal polyposis, alveolar opacities, arthritis | MN | 0 | 15 | 2 | + |
| 9/F/33 | Skin ulcers, sinusitis, alveolar opacities | MM | 0 | 19 | 1 | – |
| Control group | ||||||
| 10/F/47 | Systemic symptoms, sinusitis, myocardial infarct, renal impairment | MM, stroke | 2 | 31 | 4 | – |
| 11/M/61 | Skin ulcers, sinusitis, alveolar opacities, proteinuria | MM | 1 | 23 | 4 | – |
| 12/M/28 | Sinusitis, alveolar opacities, intestinal vasculitis | MN | 1 | 15 | 3 | + |
| 13/F/66 | Systemic symptoms, sinusitis, pleurisy, pericarditis, intestinal vasculitis | MM | 1 | 25 | 2 | – |
| 14/M/29 | Systemic symptoms, nasal polyposis | MM, diplopia | 0 | 13 | 2 | + |
| 15/F/59 | Sinusitis, alveolar opacities | MM | 0 | 15 | 3 | + |
| 16/F/46 | Systemic symptoms, sinusitis, pleurisy, pericarditis | MM | 0 | 20 | 3 | + |
| 17/M/48 | Sinusitis, alveolar opacities | AP | 0 | 15 | 1 | – |
| 18/F/25 | Systemic symptoms, skin ulcers, alveolar opacities, pleurisy, pericarditis | * | 0 | 18 | 2 | + |