Table 2.

Characteristics and therapy of patients with EGPA with and without frequent relapse. Values of p < 0.05 were considered statistically significant. Values are mean ± SD unless otherwise specified.

VariablesFrequently Relapsing EGPA, n = 35Infrequently Relapsing EGPA, n = 70Univariate Analysis, pRisk Ratio95% CI
Current age, yrs59.4 ± 17.062.8 ± 14.8NS
Male/female, n15/2022/48NS††
Atopic/nonatopic17/1827/35NS††
Age at onset of asthma, yrs44.2 ± 15.643.5 ± 16.0NS
Age at onset of EGPA, yrs52.5 ± 19.454.6 ± 15.0NS
Duration from asthma onset to EGPA, yrs8.4 ± 11.110.2 ± 10.8NS
Total no. relapses of vasculitis5.4 ± 4.81.5 ± 1.8< 0.01
Total no. relapses of vasculitis per yr1.03 ± 0.950.16 ± 0.16< 0.01
Length of observation, yrs7.3 ± 7.88.9 ± 5.6NS
At onset of EGPA
  WBC, /ml16,621 ± 798015,725 ± 7923NS
  Blood eosinophils, /ml9192 ± 66308771 ± 7027NS
  MPO-ANCA, positive/negative, n12/2217/48NS††
  PR3-ANCA, positive/negative, n2/323/60NS††
  IC > 1.5 mg/ml, positive/negative, n9/157/32NS††
  BNP, pg/ml182.4 ± 437.288.3 ± 178.8NS
Clinical manifestations of EGPA, %
  Asthma97.1100NS††
  Paranasal sinusitis90.990.6NS††
  Multiple polyneuropathy10098.6NS††
  Minimum MMT score3.3 ± 1.03.4 ± 0.8NS
  Pulmonary infiltrates78.167.2NS††
  Myocardial involvement, %
    Symptoms or abnormalities on 1 or more cardiac investigations91.471.9< 0.05††2.8720.974–8.464
    Symptoms and abnormalities on 1 or more cardiac investigations68.635.9< 0.01††2.4141.332–4.375
    Abnormalities on 1 or more cardiac investigations without symptoms22.935.9NS††
    Symptoms without abnormalities on one or more cardiac investigations00NS††
  Gastrointestinal tract, %
    Symptoms or abnormalities on endoscopy and biopsy90.073.8NS††
    Symptoms and abnormalities on endoscopy and biopsy63.332.8< 0.01††2.3031.244–4.263
    Abnormalities on endoscopy and biopsy without symptoms10.024.6NS††
    Symptoms without abnormalities on endoscopy and biopsy16.716.4NS††
  Liver, gall bladder, pancreas25.015.3
  Renal involvement, %*45.230.3NS††
    Proteinuria, 0.5 g/day or > 50 mg/dl37.914.8< 0.05††2.0581.190–3.561
    Eosinophils in urine50.033.3NS††
    Nephritis, nephrosis, or renal failure23.39.4NS††
  Skin involvement77.465.6NS††
  Arthralgia58.142.4NS††
  Myalgia20.626.3NS††
  CNS involvement20.032.3NS††
  Otitis media31.424.3NS††
No. organs involved
  Cumulative organ involvement**5.1 ± 1.64.4 ± 1.9NS
Five factor score 19961.5 ± 0.90.9 ± 0.8< 0.01
Five factor score 20091.8 ± 1.11.0 ± 1.0< 0.01
BVAS at onset37.3 ± 8.830.4 ± 7.3< 0.01
ACR criteria 4/6, 5/6, 6/6, n8/9/1824/15/31NS††
Initial treatment
  PSL as initial dose, mg43.9 ± 15.143.1 ± 11.9NS
  Steroid pulse, yes/no (%)24/10 (70.6)38/25 (60.3)NS††
  Immunosuppressant at initial phase, yes/no (%)32/3 (91.4)47/23 (67.1)< 0.01
  CYC/AZA/CSA/MTX/RTX, n25/2/5/0/038/3/4/2/0NS††
  CYC at initial phase, yes/no (%)26/9 (74.2)41/27 (60.3)NS††
  Immunosuppressant as maintenance use, yes/no (%)27/7 (79.4)31/37 (45.6)< 0.01††
  CYC/AZA/CSA/MTX/RTX, n8/12/3/4/02/5/17/7/0< 0.01††
  IVIG, yes/no (%)31/4 (88.6)40/30 (57.4)< 0.01††
  No. times IVIG administered3.4 ± 3.01.5 ± 2.1< 0.01
  PSL as maintenance dose, mg11.6 ± 6.26.6 ± 4.9< 0.01
  • * Renal involvement including proteinuria or eosinophils in urine or glomerular nephritis or nephrosis or renal dysfunction.

  • ** Cumulative organ involvement, excluding asthma and sinusitis.

  • Two-way ANOVA with repeated measures between 2 groups.

  • †† Chi-square test revealed no significant differences in frequency between the 2 groups. EGPA: eosinophilic granulomatosis with polyangiitis; WBC: white blood cells; MPO-ANCA: myeloperoxidase-specific antineutrophil cytoplasmic antibody; PR3: protein 3; IC: immune complex; BNP: B type natriuretic peptide; MMT: manual muscle test; CNS: central nervous system; BVAS: Birmingham Vasculitis Activity Score; ACR: American College of Rheumatology; PSL: prednisolone; CYC: cyclophosphamide; AZA: azathioprine; CSA: cyclosporine; MTX: methotrexate; RTX: rituximab; IVIG: intravenous immunoglobulin; NS: not significant.