Local and Systemic IKK and NF-B Signaling Associated with Sjögren’s Syndrome Immunopathogenesis
Table 1
Characteristics of the pSS patients included in the study.
Feature
pSS total ()
pSS-I ()
pSS-II ()
pSS-III ()
Controls ()
General
Age, mean ± SD years
50.6 ± 8.8
48.6 ± 6.8
49.8 ± 10.6
52.9 ± 8.0
52.2 ± 7.3
Female/male
31/2
9/1
10/1
12/0
25/1
Disease duration, mean ± SD months
21.0 ± 29.2
8.2 ± 4.5
19.5 ± 18.9
42.2 ± 40.0
NA
Histological (MSG biopsy)
Biopsy focus score (number of lymphocytic foci/4 mm2), mean ± SD, median (range)
2.9 ± 1.7 2.6 (1.0–6.4)
1.1 ± 0.1 1.1 (1.0–1.3)
2.5 ± 0.4 2.6 (2.1–3.0)
4.8 ± 1.1 5.0 (3.1–6.4)
0
Germinal centers (GC) (+/−)
3/30
0/10
1/10
2/10
0
Constitutional symptoms (+/−)
12/21
1/9
2/9
9/3
0
Clinical
Lymphadenopathy (+/−)
17/16
3/7
3/8
11/1
0
Lymphoma (+/−)
0/33
0/10
0/11
0/12
0
Glandular swelling (+/−)
9/24
1/9
1/10
7/5
0
Arthritis (+/−)
11/22
5/5
4/7
2/10
3/23
Cutaneous involvement (+/−)
5/28
0/10
2/9
3/9
0
Lung involvement (+/−)
6/27
1/9
1/10
4/8
0
Renal involvement (+/−)
2/31
0/10
1/10
1/11
0
Muscular involvement (+/−)
2/31
0/10
0/11
2/10
0
Peripheral neuropathy (+/−)
3/30
0/10
1/10
2/10
0
Central neuropathy (+/−)
3/30
0/10
1/10
2/10
0
Hematologic disorder (+/−)
15/18
5/5
6/5
4/8
2/24
Raynaud’s phenomenon (+/−)
3/30
0/10
1/11
2/10
NA
Laboratory
Anti-SSa (%)
87.9
80.0
81.8
100.0
11.5
Anti-SSb (%)
36.3
20.0
36.3
50.0
3.8
ESR, mean ± SD mm/h
40.6 ± 27.8
33.6 ± 28.2
39.0 ± 26.2
48.4 ± 25.4
19.2 ± 15.1
RF, mean ± SD U/L
238.4 ± 294.9
101.4 ± 246.7
204.4 ± 253.3
375.0 ± 320.8
25.1 ± 25.2
C3, mean ± SD mg/dL
99.3 ± 37.0
75.71 ± 40.3
89.6 ± 42.3
123.8 ± 19.6
96.6 ± 33.4
C4, mean ± SD mg/dL
18.8 ± 9.8
14.3 ± 8.1
14.4 ± 11.2
13.8 ± 7.3
20.3 ± 10.1
Cryoglobulinemia (%)
3.0
0
0
1/11
0
Hypergammaglobulinemia (%)
57.6
4/6
4/7
11/1
0
ESSDAI scores, mean ± SD
13.5 ± 9.8
5.5 ± 2.9
9.5 ± 5.4
23.7 ± 7.6
NA
pSS-I, pSS-II, and pSS-III: pSS patients with mild, intermediate, and advanced MSG lesions based on histological grading, respectively; ESR: normal range 0–20 mm/h, RF: normal range 0–20 U/L, C3: normal range 75–140 mg/dL, C4: normal range 10–40 mg/dL, hypergammaglobulinemia: IgG level > 20 g/L, and NA: not applicable. Statistical analysis was done between patients with pSS-II and patients with pSS-I, or patients with pSS-III and patients with pSS-II or pSS-I (pSS-II + pSS-I). , , and .
