Table 1.
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.