Table 1.
Clinical, serological, and immunological features of the patient cohort at baseline and after treatment with glucocorticoids
| Patients with IgG4-RD before GC (n = 30) | Healthy control subjects (n = 20) | p Value | Patients with IgG4-RD after GC (n = 30) | p Value | |
|---|---|---|---|---|---|
| Definite IgG4-RD, n (%) | 29 (97%) | ||||
| Probable IgG4-RD, n (%) | 1 (3%) | ||||
| Possible IgG4-RD, n (%) | 0 (0%) | ||||
| Age, yr, median | 70 (58–73) | 54 (46–65) | 0.005 | ||
| Male sex, n (%) | 23 (77%) | 12 (60%) | |||
| ESR (0–20 mm/h) | 18 (10–35) | ||||
| CRP (< 6 mg/L) | 5 (2–6) | ||||
| IgG4-RD RI (0–3) | 6 (6–9) | 2 (1–2.25) | 0.0001 | ||
| Serum IgG4 (< 135 mg/dl) | 313 (206–507) | 191 (87–230) | 0.0001 | ||
| CD19+ B cells (cells/ml) | 162,000 (105,750–217,750) | 236,000 (200,000–299,000) | 0.0002 | 163,500 (100,750–233,500) | 0.131 |
| CD20+ B cells (cells/ml) | 144,500 (93,000–201,700) | 224,000 (199,000–279,000) | 0.0001 | 150,500 (85,500–226,250) | 0.1 |
| Naive B cells (cells/ml) | 15,120 (8895–29,140) | 23,810 (17,930–54,020) | 0.01 | 7485 (4195–14,018) | 0.0001 |
| Percentage of CD19+ B cells | 10.55 (7.94–15.49) | 13.02 (7.89–19.39) | 0.35 | 4.78 (3.14–8.33) | 0.0001 |
| Memory B cells (cells/ml) | 26,475 (13,040–55,450) | 37,170 (21,900–57,190) | 0.25 | 41,800 (21,148–69,435) | 0.026 |
| Percentage of CD19+ B cells | 18.5 (9.26–27.31) | 16.60 (9.18–26.34) | 0.62 | 22.89 (11.14–32.50) | 0.028 |
| Plasmablasts (cells/ml) | 2515 (1023–5550) | 340 (170–600) | 0.0001 | 270 (210–1198) | 0.0001 |
| Percentage of CD19+ B cells | 1.25 (0.6–4.51) | 0.19 (0.05–0.29) | 0.0001 | 0.23 (0.1–0.79) | 0.0001 |
| Plasma cells (cells/ml)a | 278 (0–1332) | 0 (0–0) | 0.0005 | 55 (0–423) | 0.0006 |
| Percentage of CD19+ B cellsa | 0.23 (0–1.27) | 0 (0–0) | 0.0001 | 0.07 (0–0.64) | 0.0008 |
| Organ involvement, n (%) | |||||
| Pancreas | 20 (66%) | ||||
| Aorta and retroperitoneum | 7 (23.3%) | ||||
| Lymph nodes | 5 (16.6%) | ||||
| Biliary tree | 5 (16.6%) | ||||
| Salivary glands | 2 (6.6%) | ||||
| Lacrimal glands | 2 (6.6%) | ||||
| Lung | 2 (6.6%) | ||||
| Orbit | 1 (3.3%) | ||||
| Nasal sinuses | 1 (3.3%) | ||||
| Meninges | 1 (3%) | ||||
| Kidney | 1 (3.3%) | ||||
Abbreviations: CRP C-reactive protein, ESR Erythrocyte sedimentation rate, IgG4-RD RI IgG4-related disease responder index, GC Glucocorticoids
Results are expressed as median (IQR), except where indicated otherwise
a Results expressed as mean (range)