Table 1.
Baseline clinical and demographic features of the patients
| Variable | Value at study entry |
|---|---|
| Age (range) | 45 years (17–73 years) |
| Female/male | 9/11 |
| cANCA/anti-PR3, n (%) | 16 (80%) |
| pANCA/anti-MPO, n (%) | 2 (10%) |
| Biopsy performed for diagnosis | 17 (85%) |
| GPA disease duration, median (range) | 100 months (5–326 months) |
| BVAS/WG, median (range) | 3.0 (1–6) |
| VDI, median (range) | 3.0 (0–7) |
| Organ involvement, n (%) | Before study entry (ever) (%) | Active disease at study entry (%) |
|---|---|---|
| Constitutional | 17 (85) | 6 (30) |
| Ear, nose, throat | 20 (100) | 18 (90) |
| Bloody nasal discharge/crusting/ulcer | 14 | |
| Sinus involvement | 14 | |
| Conductive hearing loss | 2 | |
| Subglottic inflammation | 2 | |
| Musculoskeletal | 15 (75) | 10 (50) |
| Arthralgias/arthritis | 9 | |
| Myalgias | 1 | |
| Cutaneous | 12 (60) | 8 (40) |
| Purpura | 4 | |
| Skin nodules | 3 | |
| Rash | 1 | |
| Mucous membranes | 5 (25) | 1 (5) |
| Lung | 14 (70) | 6 (30) |
| Nodule/cavity | 4 | |
| Infiltrate | 1 | |
| Endobronchial disease | 1 | |
| Kidney | 8 (40) | 0 |
| Eye | 6 (30) | 0 |
| Nerve | 4 (20) | 1 (5) |
| Medication usage, n (%) | Before study entry (ever) | At entry and during trial |
|---|---|---|
| Cyclophosphamide intermittent | 4 (20) | 0 |
| Cyclophosphamide daily | 11 (55) | 0 |
| Azathioprine | 10 (50) | 3 (15) |
| Methotrexate | 15 (75) | 7 (35) |
| Mycophenolate mofetil | 5 (25) | 4 (20) |
| Etanercept | 1 (5) | 0 |
| Infliximab | 1 (5) | 0 |
| Rituximab | 5 (25) | 0 |
| Plasmapheresis | 1 (5) | 0 |
| Trimethoprim/sulfamethoxazole* | 6 (30) | 0 |
| Intravenous immunoglobulin | 1 (5) | 0 |
| Glucocorticoids | 20 (100) | Entry 13 (65), |
| During 15 (75) |
*
Defined as a dose of trimethoprim 800 mg/sulfamethoxazole 160 mg twice a day (does not include doses as used for pneumocystis prophylaxis).
BVAS/WG, Birmingham Vasculitis Activity Score for Wegener’s Granulomatosis; cANCA, cytoplasmic antineutrophil cytoplasmic antibodies; GPA, granulomatosis with polyangiitis (Wegener’s); MPO, myeloperoxidase; pANCA, perinuclear antineutrophil cytoplasmic antibodies; PR3, proteinase 3; VDI, Vasculitis Damage Index.