Baseline characteristics and disease activity
| Eosinophilic granulomatosis with polyangitis | IgG4-related disease | P value | |
| Age, years | 60.7±2.8 | 61.5±3.6 | 0.87 |
| Female, n (%) | 18 (81.8) | 8 (40.0) | 0.01 |
| Glandular manifestation, n (%) | 0 (0.0) | 15 (75.0) | <0.001 |
| Ear-nose-throat involvement, n (%) | 14 (63.6) | 9 (45.0) | 0.23 |
| Skin involvement, n (%) | 15 (68.2) | 0 (0.0) | <0.001 |
| Lung/lower airway tract involvement | |||
| Interstitial lung disease, n (%) | 2 (9.1) | 4 (20.0) | 0.31 |
| Migratory pulmonary infiltrates, n (%) | 2 (9.1) | 0 (0.0) | 0.17 |
| Pleural effusion, n (%) | 5 (22.7) | 0 (0.0) | 0.02 |
| Neurological manifestations | |||
| CNS involvement, n (%) | 0 (0.0) | 2 (10.0) | 0.13 |
| Peripheral neuropathy, n (%) | 19 (86.4) | 1 (5.0) | <0.001 |
| Gastrointestinal involvement, n (%) | 4 (18.2) | 0 (0.0) | 0.045 |
| Pancreas involvement, n (%) | 0 (0.0) | 2 (10.0) | 0.13 |
| Heart involvement, n (%) | 2 (9.1) | 0 (0.0) | 0.17 |
| Renal involvement, n (%) | 4 (18.2) | 4 (20.0) | 0.89 |
| Blood count | |||
| White cell count, / x10ˆ9/L | 32.0±9.5 x10ˆ9/L | 6.1±0.5 x10ˆ9/L | 0.01 |
| Eosinophil granulocyte, /μL | 18296.4±5804.7 | 487.2±133.7 | 0.01 |
| Immunoglobulin | |||
| IgG, mg/dL | 2367.6±190.7 | 2992.4±348.7 | 0.13 |
| IgA, mg/dL | 263.0±23.5 | 231.6±33.7 | 0.44 |
| IgM, mg/dL | 149.7±14.3 | 91.9±15.7 | 0.01 |
| IgE, mg/dL | 4466.8±1938.2 | 1563.5±964.5 | 0.25 |
| IgG4, mg/dL | 516.2±47.9 | 1063.0±217.7 | 0.02 |
| IgG4/IgG ratio (%) | 22.2 | 30.9 | 0.03 |
| Blood biochemistry | |||
| C3, mg/dL | 119.2±5.8 | 81.7±7.8 | <0.001 |
| C4, mg/dL | 24.6±2.3 | 16.3±2.3 | 0.01 |
| CH50, U/mL | 51.4±3.3 | 39.3±5.0 | 0.05 |
| CRP, mg/dL | 6.7±1.2 | 0.6±0.4 | <0.001 |
| RF, U/mL | 207.1±44.3 | 51.5±19.0 | <0.001 |
| sIL-2R, U/mL | 2688.0±338.2 | 1245.6±295.0 | <0.001 |
| Positive for MPO-ANCA, n (%) | 9 (40.9) | 0 (0.0) | <0.001 |
| Positive for PR3-ANCA, n (%) | 1 (4.5) | 0 (0.0) | 0.4 |
Glandular manifestation includes involvement of bilateral lacrimal gland, submandibular gland and parotid gland. Results are shown as mean±SEM unless stated otherwise.
CNS, central nervous system; CRP, C reactive protein; MPO-ANCA, myeroperoxidase anti-neutrophil cytoplasmic antibody; PR3-ANCA, proteinase3 anti-neutrophil cytoplasmic antibody; RF, Rheumatoid factor.