Table 1.

Characteristics of 121 patients with EGPA. Values are mean ± SD unless otherwise specified.

CharacteristicsAll Patients with EGPA
Current age, yrs61.2 ± 16.7
Male/female, n42/79
Age at onset of asthma, yrs43.4 ± 16.4
Age at onset of EGPA, yrs53.3 ± 17.0
Duration from onset asthma to EGPA, yrs9.4 ± 10.9
Length of observation, yrs7.8 ± 6.6
At onset of EGPA
  WBC, /ml15,772 ± 7623
  Blood eosinophils, /ml8528 ± 6764
  MPO-ANCA, %, n = 11529.6
  PR3-ANCA, %, n = 1136.2
  IC > 1.5 mg/ml, %, n = 7326.0
Cumulative manifestations during followup, %
  Paranasal sinusitis91.2
  Multiple polyneuropathy98.3
  Minimum MMT score3.50 ± 0.90
  Pulmonary infiltrates67.6
  Myocardial involvement
    Symptoms or abnormalities on 1 or more cardiac investigations73.9
    Symptoms and abnormalities on 1 or more cardiac investigations42.6
    Abnormalities on 1 or more cardiac investigations without symptoms31.3
    Symptoms without abnormalities on 1 or more cardiac investigations0
  Gastrointestinal tract
    Symptoms or abnormalities on endoscopy and biopsy78.6
    Symptoms and abnormalities on endoscopy and biopsy41.7
    Abnormalities on endoscopy and biopsy without symptoms23.3
    Symptoms without abnormalities on endoscopy and biopsy13.6
  Liver, gall bladder, pancreas17.3
  Renal involvement35.2
    Proteinuria, 0.5 g/day or > 50 mg/dl22.7
    Eosinophils in urine41.5
    Nephritis, nephrosis, or renal failure15.1
  Skin involvement67.9
  CNS involvement29.2
  Otitis media24.2
No. organs involved
  Cumulative organ involvement, n‡‡4.6 ± 1.8
Five factor score 19961.0 ± 0.9
Five factor score 20091.2 ± 1.1
BVAS at onset31.5 ± 9.0
ACR criteria 4/6, 5/6, 6/639/28/54
Initial treatment
  PSL as initial dose, mg42.6 ± 13.0
  mPSL pulse, yes/no (%)69/42 (62.1)
  Immunosuppressant at initial treatment, yes/no (%)85/36 (70.2)
  CYC/AZA/CSA/MTX/RTX, n68/6/9/2/0
  Immunosuppressant at maintenance, yes/no (%)62/55 (53.0)
  CYC/AZA/CSA/MTX/RTX, n12/18/20/12/0
  IVIG, yes/no (%)76/45 (62.8)
  PSL as maintenance dose, mg8.3 ± 5.4
  • Renal involvement including proteinuria or eosinophils in urine or glomerular nephritis or nephrosis or renal dysfunction.

  • ‡‡ Cumulative organ involvement, excluding asthma and sinusitis. EGPA: eosinophilic granulomatosis with polyangiitis; WBC: white blood cells; MPO-ANCA: myeloperoxidase-specific antineutrophil cytoplasmic antibody; PR3: protein 3; IC: immune complex; MMT: manual muscle test; CNS: central nervous system; BVAS: Birmingham Vasculitis Activity Score; ACR: American College of Rheumatology; PSL: prednisolone; mPSL: methylprednisolone; CYC: cyclophosphamide; AZA: azathioprine; CSA: cyclosporine; MTX: methotrexate; RTX: rituximab; IVIG: intravenous immunoglobulin.