Univariate analysis of survival in EGPA. Values are mean ± SD unless otherwise specified.
| Variables | Alive at Study Endpoints, n = 97 | Death, n = 17 | p |
|---|---|---|---|
| Current age or age at death, yrs | 56.8 ± 16.4 | 74.4 ± 9.4 | < 0.01† |
| Male/female, n | 33/64 | 8/9 | NS†† |
| Atopic/nonatopic, n | 48/42 | 7/10 | NS†† |
| Age at onset of asthma, yrs | 40.1 ± 15.8 | 55.4 ± 10.4 | < 0.01† |
| Age at onset of EGPA, yrs | 49.3 ± 16.3 | 69.2 ± 9.2 | < 0.01† |
| Duration from onset asthma to EGPA, yrs | 8.8 ± 10.7 | 12.9 ± 13.3 | NS† |
| Total no. relapses of vasculitis | 2.5 ± 3.7 | 2.6 ± 2.4 | NS† |
| Total no. relapses of vasculitis per yr | 0.33 ± 0.42 | 0.84 ± 1.36 | < 0.01† |
| Length of observation, yrs | 8.5 ± 6.9 | 6.2 ± 5.0 | NS† |
| At onset of EGPA | |||
| WBC, /ml | 15,615 ± 7954 | 14,899 ± 5899 | NS† |
| Blood eosinophils, /ml | 8213 ± 6877 | 8974 ± 5902 | NS† |
| MPO-ANCA, positive/negative, n | 27/66 | 5/11 | NS†† |
| PR3-ANCA, positive/negative, n | 7/84 | 0/16 | NS†† |
| IC > 1.5 mg/ml, positive/negative, n | 17/45 | 1/8 | NS†† |
| BNP, pg/ml | 106.8 ± 319.3 | 234.6 ± 406.9 | NS† |
| Clinical manifestations of EGPA, % | |||
| Asthma | 97.9 | 100 | NS†† |
| Paranasal sinusitis | 92.3 | 87.5 | NS†† |
| Multiple polyneuropathy | 97.9 | 100 | NS†† |
| Minimum MMT score | 3.6 ± 0.8 | 2.8 ± 1.1 | < 0.01† |
| Pulmonary infiltrates | 64.2 | 81.3 | NS†† |
| Myocardial involvement, % | |||
| Symptoms or abnormalities on 1 or more cardiac investigations | 74.1 | 81.3 | NS†† |
| Symptoms and abnormalities on 1 or more cardiac investigations | 43.0 | 56.3 | NS†† |
| Abnormalities on 1 or more cardiac investigations without symptoms | 31.2 | 25.0 | NS†† |
| Symptoms without abnormalities on 1 or more cardiac investigations | 0 | 0 | NS†† |
| Gastrointestinal tract, % | |||
| Symptoms or abnormalities on endoscopy and biopsy | 77.6 | 64.3 | NS†† |
| Symptoms and abnormalities on endoscopy and biopsy | 41.2 | 42.9 | NS†† |
| Abnormalities on endoscopy and biopsy without symptoms | 25.9 | 0 | < 0.05†† |
| Symptoms without abnormalities on endoscopy and biopsy | 10.6 | 21.4 | NS†† |
| Liver, gall bladder, pancreas | 15.5 | 23.5 | NS†† |
| Renal involvement, %* | 29.1 | 53.8 | NS†† |
| Proteinuria, 0.5 g/day or > 50 mg/dl | 17.7 | 41.7 | NS†† |
| Eosinophils in urine | 31.3 | 66.7 | NS†† |
| Nephritis, nephrosis, or renal failure | 10.0 | 30.8 | < 0.05†† |
| Skin involvement | 67.8 | 60.0 | NS†† |
| Arthralgia | 49.4 | 26.7 | NS†† |
| Myalgia | 24.1 | 13.3 | NS†† |
| CNS involvement | 30.0 | 17.6 | NS†† |
| Otitis media | 26.6 | 11.8 | NS†† |
| No. organs involved | |||
| Cumulative organ involvement** | 4.5 ± 1.6 | 4.6 ± 1.6 | NS† |
| Five factor score 1996 | 1.2 ± 1.1 | 0.9 ± 0.8 | NS† |
| Five factor score 2009 | 1.0 ± 0.9 | 2.2 ± 1.5 | < 0.01† |
| BVAS at onset | 30.8 ± 9.2 | 35.5 ± 7.9 | NS† |
| ACR criteria 4/6, 5/6, 6/6, n | 29/22/43 | 3/6/8 | NS† |
| Treatment | |||
| PSL as initial dose, mg | 43.3 ± 12.6 | 42.1 ± 14.9 | NS† |
| Steroid pulse, yes/no (%) | 55/35 (61.1) | 11/6 (64.7) | NS†† |
| Immunosuppressant at initial phase, yes/no (%) | 69/28 (71.1) | 13/4 (76.5) | NS†† |
| CYC/AZA/CSA/MTX/RTX, n | 53/6/8/2/0 | 12/0/1/0/0 | NS†† |
| CYC at initial phase, yes/no (%) | 55/41 (57.3) | 12/5 (70.6) | NS†† |
| Immunosuppressant as maintenance use, yes/no (%) | 56/40 (58.3) | 6/11 (35.3) | NS†† |
| CYC/AZA/CSA/MTX/RTX, n | 9/17/18/12/0 | 3/1/2/0/0 | NS†† |
| IVIG, yes/no (%) | 59/38 (60.8) | 14/3 (82.4) | NS†† |
| No. times IVIG administered | 2.0 ± 2.34 | 2.7 ± 2.9 | NS† |
| PSL as maintenance dose, mg | 8.0 ± 5.0 | 10.3 ± 7.1 | NS† |
↵* 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.