Table 1.
Clinical features of the IgG4-positive and IgG4-negative idiopathic orbital inflammatory disease.
| IgG4-positive (n = 100) | IgG4-negative (n = 65) | Total (n = 165) | p value | |
|---|---|---|---|---|
| Sex [n (%)] | ||||
| Male | 60 (60.0%) | 25 (38.5%) | 85 (51.5%) | 0.007 |
| Female | 40 (40.0%) | 40 (61.5%) | 80 (48.5%) | |
| Age (years) | 43.30 ± 16.50 | 40.65 ± 15.46 | 42.25 ± 16.10 | 0.302 |
| Smoking history [n (%)] | ||||
| Current smoker | 19 (19.0%) | 15 (23.1%) | 34 (20.6%) | 0.527 |
| Non or former smoker | 81 (81.0%) | 50 (76.9%) | 131 (79.4%) | |
| Structures involved [radiological imaging, n (%)] | ||||
| Lacrimal glands | 73 (73.0%) | 43 (66.2%) | 116 (70.3%) | 0.347 |
| Extraocular muscles | 45 (45.0%) | 30 (46.2%) | 75 (45.5%) | 0.884 |
| Orbital fat | 37 (37.0%) | 28 (43.1%) | 65 (39.4%) | 0.435 |
| Optic nerve | 2 (2.0%) | 0 (0) | 2 (1.2%) | 0.520 |
| Conjunctiva | 0 (0) | 0 (0) | 0 (0) | NA |
| Eyelid | 20 (20.0%) | 9 (13.8%) | 29 (17.6%) | 0.310 |
| Paranasal sinusitis | 68 (68.0%) | 30 (46.2%) | 98 (59.4%) | 0.005 |
| Laterality [n (%)] | ||||
| Unilateral | 51 (51.0%) | 52 (80.0%) | 103 (62.4%) | <0.001 |
| Bilateral | 49 (49.0%) | 13 (20.0%) | 62 (37.6%) | |
| Symptom duration (months) [median (IQR)] | 12.00 (4.00–33.00) | 6.00 (2.00–18.00) | 10.00 (3.00–24.00) | 0.070a |
| Clinical manifestation [n (%)] | ||||
| Pain | 17 (17.0%) | 22 (33.8%) | 39 (23.6%) | 0.013 |
| Eyelid swelling/mass | 94 (94.0%) | 63 (96.9%) | 157 (95.2%) | 0.629 |
| Eyelid hyperemia | 39 (39.0%) | 38 (58.5%) | 77 (46.7%) | 0.014 |
| Diplopia | 13 (13.0%) | 19 (29.2%) | 32 (19.4%) | 0.010 |
| Proptosis | 52 (52.0%) | 28 (43.1%) | 80 (48.5%) | 0.262 |
| EOM restriction | 31 (31.0%) | 25 (38.5%) | 56 (33.9%) | 0.323 |
| Decreased visual acuity | 26 (26.0%) | 20 (30.8%) | 46 (27.9%) | 0.504 |
| Ptosis | 12 (12.0%) | 15 (23.1%) | 27 (16.4%) | 0.060 |
| Systemic manifestations [n (%)] | ||||
| Autoimmune disease/allergic history | 16 (16.0%) | 7 (10.9%) | 23 (14.0%) | 0.362 |
| Pulmonary nodules | 14 (14.0%) | 9 (13.8%) | 23 (13.9%) | 0.978 |
| Laboratory findings | ||||
| Eosinophils (%) [median (IQR)] | 3.45 (1.50–7.78) | 1.35 (0.80–2.68) | 2.20 (1.03–5.98) | <0.001a |
| Serum IgG4 (g/L) [median (IQR)] | 3.68 (0.74–13.48) | 0.66 (0.27–0.87) | 1.35 (0.64–9.26) | 0.002a |
| Serum C3 (g/L) | 1.17 ± 0.22 | 1.35 ± 0.25 | 1.24 ± 0.24 | 0.020 |
| Serum C4 (g/L) | 0.29 ± 0.14 | 0.32 ± 0.11 | 0.30 ± 0.13 | 0.585 |
| Follow-up (months) | 19.36 ± 9.66 | 19.69 ± 10.96 | 19.49 ± 10.16 | 0.842 |
| Treatment | ||||
| Surgery+oral glucocorticoids | 83 (95.4%) | 41 (87.2%) | 124 (92.5%) | 0.170 |
| Additional treatment | 4 (4.6%) | 6 (12.8%) | 10 (7.5%) | |
| Treatment responseb | ||||
| Complete response | 42 (48.3%) | 23 (48.9%) | 65 (48.5%) | 0.942 |
| Recurrenceb | ||||
| Recurrence | 28 (32.2%) | 9 (19.1%) | 37 (27.6%) | 0.107 |
p values < 0.05 are in bold.
IQR interquartile range, NA not applicable, EOM extraocular muscle.
aMann–Whitney U-test was used to compare the two groups.
bn = 134.