Table 2.
Clinical, serological, and immunological features of relapsing and nonrelapsing patients at baseline and after treatment with glucocorticoids
| Relapsers (n = 10) | Nonrelapsers (n = 5) | p Value | |
|---|---|---|---|
| Definite IgG4-RD (%) | 9 (90%) | 5 (100%) | |
| Probable IgG4-RD (%) | 1 (10%) | 0 (0%) | |
| Possible IgG4-RD (%) | 0 (0%) | 0 (0%) | |
| Age, yr, median | 69 (60–71) | 73 (64–80) | 0.13 |
| Male, n (%) | 9 (90%) | 3 (60%) | |
| Multiorgan involvement (> 1 organ) | 7 (70%) | 4 (80%) | |
| Baseline | |||
| ESR (0–20 mm/h) | 10 (9–23) | 15 (8–20) | 0.59 |
| CRP (< 6 mg/L) | 5 (4–6.5) | 10 (5–46) | 0.06 |
| IgG4-RD RI (0–3) | 9 (6–9) | 12 (9–12) | 0.22 |
| Eosinophils (< 300 cell/μl) | 300 (300–500) | 200 (150–300) | 0.034 |
| Serum IgG4 (< 135 mg/dl) | 364 (232–1090) | 498 (328–947) | 0.5 |
| IgE (mU/ml) | 308 (2–1488) | 733 (271–1554) | 0.11 |
| Prednisone dose (mg/d) | 5 (0–5.5) | 5 (2.5–5) | 0.99 |
| CD19+ B cells (cells/ml) | 138,500 (97,500–172,500) | 144,000 (103,000–162,000) | 0.66 |
| CD20+ B cells (cells/ml) | 114,000 (86,250–150,000) | 128,000 (82,000–140,500) | 0.57 |
| Naive B cells (cells/ml) | 14,170 (9518–24,198) | 11,170 (2915–38,650) | 0.35 |
| Percentage of CD19+ B cells | 11.4 (9.5–13.7) | 11.52 (2.33–24.37) | 0.09 |
| Memory B cells (cells/ml) | 20,450 (10,790–36,070) | 48,590 (11,305–62,095) | 0.44 |
| Percentage of CD19+ B cells | 15.79 (10.25–23.9) | 26.48 (6.9–47.88) | 0.67 |
| Plasmablasts (cells/ml) | 3280 (985–9868) | 5400 (3825–8000) | 0.39 |
| Percentage of CD19+ B cells | 3.26 (0.84–7.8) | 3.38 (2.06–4.82) | 0.76 |
| Plasma cells (cells/ml)a | 420 (0–1332) | 489 (146–1300) | 0.86 |
| Percentage of CD19+ B cellsa | 0.37 (0–1.27) | 0.27 (0.1–0.49) | 0.95 |
| After 6 mo of treatment | |||
| ESR (0–20 mm/h) | 5 (3–21) | 9 (8–20) | 0.29 |
| CRP (< 6 mg/L) | 2 (1–2.25) | 2 (1.5–4) | 0.47 |
| IgG4-RD RI (0–3) | 2.5 (1.75–3.25) | 2 (2–2.5) | 0.62 |
| Eosinophils (< 300 cell/μl) | 200 (100–325) | 100 (100–200) | 0.37 |
| Serum IgG4 (< 135 mg/dl) | 182.5 (107–729) | 257 (211–406) | 0.42 |
| IgE (mU/ml) | 107 (2–299) | 425 (384–466) | 0.13 |
| Prednisone dose (mg/d) | 5 (0–5.62) | 5 (2.5–5) | 0.99 |
| CD19+ B cells (cells/ml) | 174,500 (93,750–222,250) | 128,000 (64,500–157,500) | 0.2 |
| CD20+ B cells (cells/ml) | 165,000 (84,750–208,500) | 128,000 (52,500–154,000) | 0.24 |
| Naive B cells (cells/ml) | 7860 (3988–13,585) | 7380 (2950–15,460) | 0.8 |
| Percentage of CD19+ B cells | 3.51 (2.57–4.13) | 9.27 (4.16–15.73) | 0.1 |
| Memory B cells (cells/ml) | 60,540 (21,148–75,428) | 18,360 (9045–34,650) | 0.05 |
| Percentage of CD19+ B cells | 27.46 (19.06–34.9) | 24.19 (6.43–37.65) | 0.89 |
| Plasmablasts (cells/ml) | 355 (138–1263) | 1310 (565–3350) | 0.07 |
| Percentage of CD19+ B cells | 0.27 (0.07–0.53) | 0.88 (0.36–5.3) | 0.03 |
| Plasma cells (cells/ml)a | 56 (0–333) | 143 (0–423) | 0.22 |
| Percentage of CD19+ B cellsa | 0.05 (0–0.32) | 0.19 (0–0.53) | 0.16 |
Abbreviations: CRP C-reactive protein; ESR erythrocyte sedimentation rate; IgG4-RD RI IgG4-Related Disease Responder Index
Results are expressed as median (IQR), except where indicated otherwise
a Results expressed as mean (range)