Baseline characteristics of 30 patients with EGPA treated with IFN-α. Values are n (%) unless otherwise specified.
| Characteristics | All, n = 30 |
|---|---|
| Female | 16 (53) |
| Age at first IFN-α, yrs, median (IQR) | 51 (44–58) |
| Prior disease duration, mos, median (IQR) | 72 (30.8–108) |
| ANCA | 1 (3) |
| Peripheral eosinophil count, × 109/l, median (IQR) | |
| At diagnosis | 2 (0.8–3.4) |
| At initiation of IFN therapy | 0.5 (0.11–0.9) |
| Biopsy performed | 24 (80) |
| Biopsy-proven vasculitis | 11 (37) |
| BVAS at first IFN-α, median (IQR) | 6 (4–13.5) |
| Five-factor score | |
| 0 | 22 (73) |
| 1 | 7 (23) |
| 2 | 1 (4) |
| DEI, median (IQR) | 6 (4–8) |
| Organ involvement at entry according to DEI | |
| Lung | 30 (100) |
| ENT | 26 (87) |
| Cardiac | 12 (40) |
| Peripheral nervous system | 10 (33) |
| Central nervous system | 1 (3) |
| Skin | 8 (27) |
| Eyes | 2 (7) |
| Arthralgia, arthritis | 2 (7) |
| Gastrointestinal tract | 1 (3) |
| Evidence of polyangiitis* | 22 (73) |
| Immunosuppressive drugs prior to IFN-α | |
| Prednisolone | 30 (100) |
| Dose at entry, mg/day, median (IQR) | 17.5 (10–20) |
| Cyclophosphamide | 4 (13) |
| Azathioprine | 3 (10) |
| Omalizumab | 6 (20) |
| Methotrexate | 2 (7) |
| Mycophenolate mofetil | 1 (3) |
| Rituximab | 1 (3) |
↵* Defined as presence of vasculitis on biopsy or presence of strong clinical surrogate for polyangiitis (i.e., myocardial ischemia because of coronaritis, palpable purpura, alveolar hemorrhage, scleritis, mononeuritis multiplex). EGPA: eosinophilic granulomatosis with polyangiitis; IFN-α: interferon-α; ANCA: antineutrophil cytoplasmic antibodies; BVAS: Birmingham Vasculitis Activity Score; DEI: Disease Extent Index; IQR: interquartile range.