Baseline characteristics of IgG4-RD subjects (n=30)
| Parameter | Value | |
|---|---|---|
| Age, years (mean±SD) | 61±11 | |
| Male gender, n (%) | 26 (87) | |
| Ethnic group, n (%) | ||
| White | 26 (87) | |
| Black | 1 (3) | |
| Asian | 3 (10) | |
| Duration of IgG4-RD, months (mean, range) | 70 (1–540) | |
| Biopsy-proven disease, n (%) | 28 (93) | |
| Number of organs affected, mean (range) | 3.5 (1–8) | |
| Organ site involvement (beginning superiorly), n (%) | ||
| Ophthalmic | 8 (27) | |
| Lacrimal glands | 4 (13) | |
| Orbital pseudotumor | 3 (10) | |
| Orbital myositis | 1 (3) | |
| Other | 2 (7) | |
| Major salivary gland | 18 (60) | |
| Parotid | 8 (27) | |
| Submandibular | 13 (43) | |
| Sublingual | 3 (10) | |
| Ear, nose, and throat | 7 (23) | |
| Sinusitis | 5 (17) | |
| Nasal disease | 3 (10) | |
| Pharyngeal mass | 2 (7) | |
| Pulmonary | 5 (17) | |
| Pulmonary pseudotumor | 4 (13) | |
| Pulmonary nodules | 2 (7) | |
| Multiple ground-glass opacities | 1 (3) | |
| Interstitial lung disease | 1 (3) | |
| Pericardial disease | 3 (10) | |
| Kidney disease | 7 (23) | |
| Tubulointerstitial nephritis | 4 (10) | |
| Multiple kidney opacities | 2 (7) | |
| Kidney pseudotumor | 1 (3) | |
| Soft tissue rim surrounding kidneys | 1 (3) | |
| Retroperitoneal fibrosis | 3 (10) | |
| Aortitis | 2 (7) | |
| Thoracic | 1 (3) | |
| Abdominal | 1 (3) | |
| Autoimmune pancreatitis | 18 (60) | |
| Biliary duct disease | 10 (33) | |
| Distal common bile duct stricture | 2 (7) | |
| Proximal (intra and extra hepatic) strictures | 7 (23) | |
| Prostatitis | 2 (7) | |
| Lymphadenopathy | 18 (60) | |
| Submandibular | 6 (20) | |
| Cervical | 8 (27) | |
| Supraclavicular | 2 (7) | |
| Axillary | 5 (17) | |
| Mediastinal | 10 (33) | |
| Hilar | 8 (27) | |
| Abdominal | 2 (7) | |
| Pelvic | 5 (17) | |
| Inguinal | 5 (17) | |
| Constitutional symptoms | 5 (17) | |
| Skin disease | 1 (3) | |
| Disease activity | ||
| IgG4-RD Responder Index score (mean±SD) | 11±7 | |
| Physician global assessment, mm (mean±SD) | 63±22 | |
| Laboratory parameters | Normal | |
| Serum IgG4, mg/dL (mean, range) | 625 (22–4780) | (2.4–121) |
| Serum IgG, mg/dL (mean, range) | 1951 (727–5320) | (767–1590) |
| C3, mg/dL (mean, range) | 95 (30–143) | (81–157) |
| C4, mg/dL (mean, range) | 15 (2–25) | (12–39) |
| ESR, mm/h (mean, range) | 37 (6–123) | (0–13) |
| CRP, mg/L (mean, range) | 17 (0.5–189.6) | (<8.0) |
| Treatment history | ||
| Total prednisone dose equivalent administered over 28 days preceding enrolment, mg (mean, range) | 42 (0–850) | |
| Previous glucocorticoid course, n (%) | 22 (73) | |
| Previous DMARD treatment, n (%) | 5 (17) | |
| Previous RTX treatment, n (%) | 6 (20) | |
CRP, C-reactive protein; DMARD, disease modifying anti-rheumatic drug; ESR, erythrocyte sedimentation rate; RD, related disease; RTX, rituximab.