Clinical and laboratory characteristics of IgG4-RD patients
| All IgG4-RD patients (n=37) | IgG4-RD patients treated with RTX (n=12) | |
|---|---|---|
| Age, mean (years) | 59 (range 33–82) | 60.4 (range 44–77) |
| Gender (% male) | 68% | 82% |
| Race | 76% Caucasian | 64% Caucasian |
| Plasmablast count, median (/mL) | 4698 (range 610–79 524) | 6356 (range 1145–41 589) |
| Serum IgG4, mean (normal <135 mg/dL) | 612 (range 5.3–4780) | 1014 (range 26–4780) |
| Number with normal serum IgG4 (%) | 13 (36%) | 3 (25%) |
| C3, mean (normal 86–184 mg/dL) | 94.3 | 90 |
| C4, mean (normal 16–38 mg/dL) | 17.4 | 14.4 |
| ESR, mean (normal 0–20 mm/h) | 37 | 39.4 |
| CRP, mean (normal <8 mg/L) | 16.6 | 15.4 |
| IgG4-RD Responder Index (mean) | 11 (range 3–36) | 13.8 (range 3–36) |
| Average no. of organs involved | 2.6 (range 1–6) | 2.8 (range 1–6) |
| Orbit | 7 patients | |
| Parotid | 7 patients | |
| Sinusitis | 1 patient | |
| Palate | 3 patients | |
| Lymphadenopathy | 6 patients | |
| Lungs | 6 patients | |
| RPF | 6 patients | |
| Sclerosing mesenteritis | 1 patient | |
| Pancreas | 8 patients | |
| Liver | 2 patients | |
| Biliary tract | 2 patients | |
| Gallbladder | 1 patient | |
| Kidney | 3 patients | |
| Other | 1 patient |
CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; IgG4-RD, IgG4-related disease; RPF, retroperitoneal fibrosis; RTX, rituximab.