Table 1

Baseline demographics and clinical characteristics of 41 patients with eosinophilic granulomatosis with polyangiitis treated with rituximab

Sex F/M21/20
Age at first rituximab, years, median (IQR)54 (38.5–61)
Prior disease duration, months, median (IQR)46 (11–95.5)
ANCA, number of patients (%)
 Positive (including IF)18 (44)
 Negative23 (56)
 Positive C- or P-ANCA (only IF)*5 (12)
 PR3-ANCA4 (10)
 MPO-ANCA9 (22)
Biopsy-proven disease28 (68)
Number of prior immunosuppressive therapies, median (IQR)2 (1–3)
Immunosuppressive drugs prior to rituximab, number of patients (%)
 Cyclophosphamide27 (66)
  Cumulative cyclophosphamide dose, grams, median (IQR)12 (8.7–32.5)
 Azathioprine21 (51)
 Mycophenolate mofetil20 (49)
 Methotrexate11 (27)
 Intravenous immunoglobulin7 (17)
 Leflunomide3 (7)
 Alemtuzumab2 (5)
 Etanercept2 (5)
 Infliximab2 (5)
 Mepolizumab1 (2)
 Omalizumab1 (2)
DEI score, median (IQR) at first rituximab treatment8 (8–10)
BVAS median (IQR) at first rituximab treatment11 (6–17.5)
Organ involvement according to DEI, number of patients (%)
 Lung (including asthma)40 (98)
 Ear, nose and throat35 (85)
 Arthralgia/arthritis22 (54)
 Skin20 (49)
 Peripheral nervous system12 (29)
 Renal10 (24)
 Gastrointestinal tract9 (22)
 Heart9 (22)
 Eyes5 (12)
 Central nervous system1 (2)
  • *One patient with positive IF and unavailable ELISA data.

  • ANCA, antineutrophil cytoplasmic antibodies; BVAS, Birmingham Vasculitis Activity Score; DEI, Disease Extent Index; MPO, myeloperoxidase; PR3, proteinase-3.