TABLE 6

Treatment and outcome in 157 patients with eosinophilic granulomatosis with polyangiitis

Mean±sd (range) duration of follow-up years7.4±6.4 (0–38.0)
First-line systemic therapy
 Systemic corticosteroids157 (100%)
 Cyclophosphamide53 (34%)
 Azathioprine13 (8%)
 Methotrexate2 (1%)
 Plasma exchange2 (1%)
 Intravenous immunoglobulins1 (1%)
Deaths4 (3%)
Relapses
 Number of patients with relapse78 (50%)
 Patients with 1/2/3/≥4 relapses31/17/13/17
 Number of relapses
  Total196
  Mean±sd number of relapses per patient1.3±2.0
 Peripheral blood eosinophils >0.5×109 L−1139 (71%)
 Peripheral blood eosinophil count ×109 L−11.0±0.7
 Type of relapse
  Asthma symptoms121 (62%)
  Systemic manifestations72 (37%)
  Pulmonary infiltrates (other than alveolar haemorrhage)9 (5%)
Treatment for relapse
 Corticosteroids78 (100%)
 Cyclophosphamide21 (27%)
 Azathioprine22 (28%)
 Methotrexate11 (14%)
 Mycophenolate mofetil6 (8%)
 Rituximab3 (4%)
 Intravenous immunoglobulins3 (4%)
 Omalizumab6 (8%)
 Imatinib2 (3%)
 Hydroxyurea2 (3%)
 No other systemic therapy required20 (26%)
 Not available10 (13%)
Long-term sequelae43 (27%)
 Peripheral nerve involvement (or damage)14 (9%)#
 Chronic left heart failure (left ventricular ejection fraction <50%)15 (9%)
 Debilitating persistent chronic asthma13 (8%)
 Corticosteroid related side-effects (diabetes, symptomatic osteoporosis, etc.)4 (3%)
 Chronic renal insufficiency4 (3%)
 Chronic debilitating fatigue1 (1%)
 Chronic debilitating rhinosinusitis1 (1%)
  • #: including two patients with visual impairment; : one patient had an implantable defibrillator.