Session Information
Date: Sunday, November 8, 2015
Title: Sjögren's Syndrome Poster I: Clinical Insights into Sjögren's Syndrome
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose: The histopathological hallmark and a major diagnostic criterion of Sjögren’s syndrome (SS) is the presence of periductal lymphocytic infiltrates in the labial minor salivary glands (LMSG). SS can occur either as an entity alone (primary SS-pSS) or with rheumatoid arthritis (RA) and other autoimmune diseases. Sicca symptoms (primarily ocular) are observed in around 20-25% of RA patients. The present study examines the LMSG infiltrates in sicca-RA patients and compares their composition with this described in pSS.
Methods: 100 consecutive RA patients (2010 ACR criteria) answer a validated sicca symptoms questionnaire. Positive responders were evaluated for ocular and oral dryness including a LMSG biopsy. All samples in addition to haematoxylin and eosin were stained for the immunocyte populations in serial sections [CD3: total T cells, CD4 and CD8: T cell subpopulations, CD20: B cells, CD68: macrophages (ΜΦ), S100 and fascin: interdigitating and follicular dendritic cells (DC)]. Stained cells and total mononuclear cells (MNC) were counted in the entire section. Counts were expressed as cell incidence (percentage of cell type number/total infiltrating MNC number).
Results:
44/100 RA patients responded positively for sicca symptoms. Their anthropometric and clinicoserologic characteristics are shown in Table 1. In total, 30 out of 100 RA patients had LMSG with a focus score ≥ 1. The immune cell composition in LMSG was found to differ from that of pSS [1] (Table 2): The incidence of DC and MΦ was higher, T cells were increased in severe lesions (ANOVA test, p=0.03) and DCs were inverse correlated with lesion severity (S100: p= 0.0004, r=-0.56; fascin: p=0.08, r=-0.29). In sicca-RA patients, MΦ and DC incidence correlated positively with the patients’ RA score (ACR criteria) (CD68: p<0.0001, r=0.65; S100: p=0.01, r=0.41; fascin: p=0.07, r=0.31) and was higher in those who had biopsy focus score<1 and were anti-Ro negative [MΦ (p=0.02) and DC (S100: p=0.002; fascin: p=0.001)].
Conclusion:
The cell composition of the LMSG infiltrates of sicca-RA patients is different compared to that seen in biopsies of pSS patients. These differences, coupled with the known genetic and serologic dissimilarities may further attest for diverse pathophysiologic process operating in the genesis of these two entities.
References
- Characteristics of the minor salivary gland infiltrates in Sjögren’s syndrome. Christodoulou MI, Kapsogeorgou EK, Moutsopoulos HM. J Autoimmun. 2010 34(4):400-7
Table 1. a. ACR 2010 RA criteria, at the time of diagnosis.
|
Patients characteristics |
N=44 |
|
Epidemiological |
|
|
Age, median (range) |
57.2 (32-71) |
|
Sex (m/f) |
2/42 |
|
Total follow up (months); median (range) |
80 (6-264) |
|
RA precedes/coincide/SS precedes No (%) |
20/20/4 (45.5/45.5/9) |
|
Evolution time RA to SS (months); (mean ± SD) |
89.8 ± 79.1 |
|
Criteria |
|
|
Ocular dryness (subjective), No (%) |
38 (86.4) |
|
Oral dryness (subjective), No (%) |
24 (54.5) |
|
Rose Bengal (positive), No (%) |
27 (61.4) |
|
Schirmer’s test (positive), No (%) |
28 (63.6) |
|
Unstimulated salivary flow, No (%) |
23 (52.3) |
|
Biopsy Tarpley score, median (range) |
1 (0-4) |
|
Biopsy Focus score, median (range) |
0.96 (0-12) |
|
RA criteria scorea median (range) |
7 (6-10) |
|
Laboratory |
|
|
Anemia, No (%) |
14 (31.8) |
|
Thrombopenia, No (%) |
3 (6.8) |
|
Leukopenia, No (%) |
1 (2.3) |
|
ANA positive, No (%) |
34 (77.3) |
|
RF positive, No (%) |
34 (77.3) |
|
Anti-CCP positive, No (%) |
23 (52.3) |
|
Anti-Ro/SSA positive, No (%) |
15 (34.1) |
|
Anti-La/SSB positive, No (%) |
2 (4.5) |
|
C3 (mg/dl), mean ± SD |
112.7 ± 24.5 |
|
C4 (mg/dl), mean ± SD |
22.6 ± 7.0 |
|
Clinical |
|
|
Fatigue, No (%) |
10 (22.7) |
|
Fever, No (%) |
11 (25.0) |
|
Raynaud’s phenomenon, No (%) |
14 (31.8) |
|
Morning stiffness, No (%) |
33 (75.0) |
|
Renal/Lung/Liver involvement, No (%) |
8 (18.2) |
|
Serositis, No (%) |
3 (6.8) |
|
Myositis, No (%) |
1 (2.3) |
|
Peripheral nerve involvement, No (%) |
5 (11.4) |
|
Purpura, No (%) |
8 (18.2) |
|
Salivary Gland Enlargement, No (%) |
5 (11.4) |
|
Lymphoma, No (%) |
2 (4.5) |
Table 2. MSG biopsy lesion composition in RA patients with sicca compared to primary SS patients [1]
|
|
Percentage or ratio |
Correlation with MSG lesion severity (No Infiltrates/4mm2) |
|||
|
Primary SS [1] |
RA with sicca |
Primary SS [1] |
RA with sicca |
||
|
%CD20 |
44.19 ± 1.8 |
42.9 ± 1.92 |
r = 0.50 p= 0.001 |
r= 0.131 p= 0.452 |
|
|
%CD3 |
48.07 ± 1.8 |
44.4 ± 1.57 |
r= – 0.65, p < 0.0001 |
r= 0.191 p= 0.281 |
|
|
%CD4 |
32.94 ± 1.9 |
26.4 ± 1.7 |
r= – 0.58 p = 0.0003 |
r= 0.231 p= 0.181 |
|
|
%CD8 |
15.42 ± 1.0 |
17.4 ± 1.16 |
r= – 0.41 p <0.0001 |
r= – 0.256 p= 0.133 |
|
|
%CD68 MΦ |
4.48 ± 0.67 |
5.69 ± 0.48 |
r= 0.46 p= 0.005 |
r= – 0.278 p= 0.105 |
|
|
%S100 DC |
0.70 ± 0.10 |
1.89 ± 0.24 |
r= – 0.40 p = 0.015 |
r= – 0.565 p= 0.0004 |
|
|
%Fascin DC |
1.89 ± 0.22 |
3.96 ± 0.36 |
NS |
r= – 0.294 p= 0.08 |
|
|
%CD3/%CD20 |
1.26 ± 0.12 |
1.15 ± 0.08 |
r= – 0.59 p <0.001 |
r= – 0.046 p= 0.794 |
|
|
%CD4/%CD8 |
2.56 ± 0.29 |
2.02 ± 0.31 |
r= – 0.41 p= 0.015 |
r= 0.285 p= 0.09 |
|
To cite this abstract in AMA style:
Fragoulis G, Reilly J, Kerr S, McInnes IB, Moutsopoulos HM. Sicca Syndrome in Rheumatoid Arthritis: Is It a Real Sjögren’s? [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/sicca-syndrome-in-rheumatoid-arthritis-is-it-a-real-sjgrens/. Accessed .« Back to 2015 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/sicca-syndrome-in-rheumatoid-arthritis-is-it-a-real-sjgrens/