Demographic Data |
|
|
Age at Onset (median, IQR) |
55 (4–83) |
Age at Diagnosis (median, IQR) |
61 (4–84) |
Sex (n, % male) |
68, 68% |
Ethnicity |
|
Caucasian (n, %) |
69, 69% |
Asian (n, %) |
12, 12% |
Hispanic (n, %) |
5, 5% |
African American (n, %) |
4, 4% |
Other(n, %) |
10, 10% |
|
Clinical Data |
|
|
Clinically Active (n, %) |
100, 100% |
IgG4-RD-RI (median, IQR) |
4 (0–20) |
On Treatment (n, %) |
14, 14% |
|
Organ Distribution |
|
|
Multi-organ Involvement (n, %) |
68, 68% |
Number of Organs Involved (median, IQR) |
3 (1–10) |
Lymph Nodes (n, %) |
41, 41% |
Submandibular Gland (n, %) |
48, 48% |
Pancreas (n, %) |
32, 32% |
Parotid Gland (n, %) |
26, 26% |
Lacrimal Gland (n, %) |
31, 31% |
Lungs (n, %) |
18, 18% |
Retroperitoneum (n, %) |
20, 20% |
Kidney (n, %) |
21, 21% |
Bile Ducts (n, %) |
14, 14% |
|
Laboratory Parameters |
|
|
Elevated Total IgG, >1,295 mg/dL (n, %) |
63, 63% |
Elevated serum IgG1, >928.6 mg/dL (n, %) |
43, 43% |
Elevated serum IgG2, >700.3 mg/dL (n, %) |
39, 39% |
Elevated serum IgG3, >176.1 mg/dL (n, %) |
36, 36% |
Elevated serum IgG4, >86.4 mg/dL (n, %) |
76, 76% |
Elevated serum IgE, >114 kU/L (n, %) |
54, 54% |
Low serum IgM, <53 mg/dL (n, %) |
39, 39% |
Hypocomplementemia, C3 < 81 mg/dL or C4 < 12 mg/dL (n, %) |
30, 30% |
Elevated C-reactive protein, >8.0 mg/L (n, %) |
35, 35% |
Elevated erythrocyte sedimentation rate, >13 mm/hr (n, %) |
63, 63% |