Relation of Autoimmune Cytopenia to Glandular and Systemic Manifestations in Primary Sjögren Syndrome: Analysis of 113 Korean Patients

2015 ◽  
Vol 42 (10) ◽  
pp. 1817-1824 ◽  
Author(s):  
Jung Hee Koh ◽  
Jennifer Lee ◽  
So-Hyang Chung ◽  
Seung-Ki Kwok ◽  
Sung-Hwan Park
Keyword(s):  
Ocular Surface ◽  
Activity Index ◽  
Disease Activity Index ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Ocular Surface Disease Index ◽  
Drug Induced ◽  
Primary Sjögren Syndrome ◽  
Autoimmune Cytopenia ◽  
Schirmer I Test

Objective.To investigate the characteristics of patients with primary Sjögren syndrome (pSS) who have autoimmune cytopenia.Methods.We analyzed 113 participants from the Korean Initiative of Primary Sjögren Syndrome, a prospective pSS cohort. Autoimmune cytopenia was defined as autoimmune origin neutropenia, anemia, and/or thrombocytopenia without vitamin or iron deficiency, or drug-induced cytopenia. To identify the association between autoimmune cytopenia and the clinical characteristics of pSS, extraglandular manifestations were analyzed according to the European League Against Rheumatism Sjögren’s syndrome disease activity index (ESSDAI) definition. Xerophthalmia was assessed with the Ocular Surface Disease Index, Schirmer I test, ocular stain score (OSS), and tear film breakup time.Results.The median total ESSDAI score was 2 (interquartile range 1–6). About a quarter of patients had no systemic activity. Autoimmune cytopenia was observed in 23.9% of patients (n = 27). Moderate biological features were more frequently observed in patients with autoimmune cytopenia than in patients without [10 (37%) and 11 (12.8%), respectively, p = 0.016]. Articular involvement was exhibited in 1 patient with autoimmune cytopenia, but in 23 patients (27.4%) without autoimmune cytopenia (p = 0.013). Higher OSS (p = 0.002) and lower mean Schirmer I test (p = 0.029) were observed in patients with autoimmune cytopenia than in those without. Neutrophils and lymphocytes negatively correlated with OSS (ρ = −0.204, p = 0.041 and ρ = −0.230, p = 0.020, respectively).Conclusion.Autoimmune cytopenia is closely associated with severe ocular surface damage in pSS. Therefore, assessment of xerophthalmia by ophthalmologists may be mandatory, particularly in patients with pSS with cytopenia, even if patients do not complain of eye dryness.

scholarly journals Abdominal symptoms during Sjogren’s syndrome: a pilot study

2021 ◽  
Vol 61 (1) ◽  
Author(s):  
Simon Parreau ◽  
Jérémie Jacques ◽  
Stéphanie Dumonteil ◽  
Sylvain. Palat ◽  
Sophie Geyl ◽  
...  
Keyword(s):  
Symptom Score ◽  
Activity Index ◽  
Central Nervous System Involvement ◽  
Disease Activity Index ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Primary Sjögren Syndrome ◽  
Abdominal Symptoms ◽  
Patient Reported ◽  
Therapeutic Recommendation

Abstract Background Abdominal symptoms in patients with primary Sjögren syndrome (pSS) are poorly documented. The objective of the study was to describe the abdominal symptoms of patients with pSS and to assess their association with characteristics of the disease. Methods One hundred and fifty patients with pSS were evaluated using a composite global symptom score for abdominal symptoms and their severity. Data concerning the clinical and biological characteristics of pSS and abdominal disorders were also collected. Results Of the patients with pSS, 95% suffered from abdominal symptoms (median global symptom score 7.5 ± 5.5 points out of 30). More than half of the patients experienced abdominal tension (68%), upper abdominal pain (54%), abdominal discomfort (58%) and/or constipation (54%). Regarding the pSS activity, in relation to European League Against Rheumatism (EULAR) Sjögren syndrome disease activity index score items, general and central nervous system involvement wereassociated with a high global symptom score. The EULAR Sjogren Syndrome Patient Reported Index (ESSPRI) symptom score was positively correlated with the global symptom score (p < 0.01). Multivariate analysis showed a significant association between a high global symptom score and SSA seronegativity, gastroparesis, and ESSPRI score (p < 0.01 for each). Conclusions The majority of patients with pSS suffered abdominal symptoms. There is currently no therapeutic recommendation because of the lack of information on the underlying pathophysiological mechanisms. Trial registration NCT03157011. Date of registration: July 17, 2017.

The Interleukin 33/ST2 Axis in Patients with Primary Sjögren Syndrome: Expression in Serum and Salivary Glands, and the Clinical Association

2014 ◽  
Vol 42 (2) ◽  
pp. 264-271 ◽  
Author(s):  
Seung Min Jung ◽  
Jaeseon Lee ◽  
Seung Ye Baek ◽  
Jae Ho Lee ◽  
Jennifer Lee ◽  
...  
Keyword(s):  
Salivary Glands ◽  
Activity Index ◽  
Disease Activity Index ◽  
Serum Levels ◽  
Interferon Γ ◽  
Sjögren Syndrome ◽  
Epithelial Cell Line ◽  
Sjogren Syndrome ◽  
Interleukin 33 ◽  
Primary Sjögren Syndrome

Objective.To evaluate the expression of interleukin 33 (IL-33) and its receptor in sera and salivary tissues of patients with primary Sjögren syndrome (pSS), and to investigate the association with clinical profiles.Methods.Serum IL-33 and soluble ST2 (sST2) of 55 patients with pSS and 48 controls were determined by ELISA and assessed for clinical correlation. The expression of IL-33/ST2 in salivary tissues was investigated by immunohistochemical staining and was further characterized by confocal microscopy. We also measured IL-33 production in salivary glandular epithelial cells by proinflammatory stimuli.Results.Serum levels of IL-33 and sST2 were higher in patients with pSS compared to those in controls (p = 0.018 and p < 0.0001, respectively). Among patients with pSS, sST2 concentration was associated with thrombocytopenia (p = 0.029) and correlated with disease duration (p = 0.013) and the European League Against Rheumatism Sjögren Syndrome Disease Activity Index (p = 0.042). The expression of IL-33 and ST2 was elevated in salivary glands of patients with pSS with grade 2 inflammation, and diminished in advanced inflammation. In patients with pSS, IL-33 was mainly observed in epithelial and endothelial cells of glandular tissue. The production of IL-33 mRNA by salivary gland epithelial cell line increased under stimulation with interferon-γ.Conclusion.The expression of IL-33 and its receptor was elevated in sera and salivary tissues of patients with pSS. These results suggest that the IL-33/ST2 axis might have a role in the pathogenesis of pSS.

Detailed Analysis of the Articular Domain in Patients with Primary Sjögren Syndrome

2017 ◽  
Vol 44 (3) ◽  
pp. 292-296 ◽  
Author(s):  
Rada V. Moerman ◽  
Suzanne Arends ◽  
Petra M. Meiners ◽  
Arjan Vissink ◽  
Frederik K.L. Spijkervet ◽  
...  
Keyword(s):  
Disease Activity ◽  
Disease Activity Score ◽  
Activity Index ◽  
Disease Activity Index ◽  
Sjögren Syndrome ◽  
Joint Disease ◽  
Sjogren Syndrome ◽  
Reactive Protein ◽  
Primary Sjögren Syndrome ◽  
European League Against Rheumatism

Objective.We used the 28-joint Disease Activity Score (DAS28) and the European League Against Rheumatism Sjögren’s Syndrome Disease Activity Index (ESSDAI) articular domain to assess the effect of rituximab (RTX) and abatacept (ABA) on articular involvement in primary Sjögren syndrome (pSS).Methods.Patients with pSS treated with RTX (n = 18) or ABA (n = 13) and having a DAS28 erythrocyte sedimentation rate (ESR)/C-reactive protein (CRP) level ≥ 3.2 at baseline were selected. Generalized estimating equations were used to analyze the DAS28 and ESSDAI articular domain over time.Results.In the RTX group, DAS28-ESR/CRP decreased significantly up to 48 weeks. In the ABA group, DAS28-ESR/CRP decreased significantly up to 24 weeks. DAS28 correlated significantly with ESSDAI articular domain.Conclusion.DAS28 is useful to evaluate the effect of biologicals on articular involvement in patients with pSS.

Cryoglobulinemia in Sjögren Syndrome: A Disease Subset that Links Higher Systemic Disease Activity, Autoimmunity, and Local B Cell Proliferation in Mucosa-associated Lymphoid Tissue

2017 ◽  
Vol 44 (8) ◽  
pp. 1179-1183 ◽  
Author(s):  
Luca Quartuccio ◽  
Chiara Baldini ◽  
Roberta Priori ◽  
Elena Bartoloni ◽  
Francesco Carubbi ◽  
...  
Keyword(s):  
Disease Activity ◽  
Activity Index ◽  
Systemic Disease ◽  
Disease Activity Index ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Systemic Activity ◽  
Primary Sjögren Syndrome ◽  
System P ◽  
European League Against Rheumatism

Objective.To compare systemic disease activity by validated tools, i.e., the European League Against Rheumatism Sjögren Syndrome Disease Activity Index (ESSDAI) and the Clinical ESSDAI (ClinESSDAI) scores, between primary Sjögren syndrome (pSS) with positive serum cryoglobulins and pSS without serum cryoglobulins.Methods.There were 825 consecutive patients with pSS who were retrospectively evaluated.Results.The ESSDAI and the ClinESSDAI scores were significantly higher in cryoglobulin-positive patients (p < 0.0001, for both scores). Cryoglobulinemia was significantly associated with these domains: constitutional (p = 0.003), lymphadenopathy (p = 0.007), glandular (p = 0.0002), cutaneous (p < 0.0001), peripheral nervous system (p < 0.0001), hematological (p = 0.004), and biological (p < 0.0001).Conclusion.Cryoglobulin-positive patients show the highest systemic activity in pSS.

scholarly journals Tear ATG5 as a Potential Novel Biomarker in the Diagnosis of Sjögren Syndrome

Diagnostics ◽  
2021 ◽  
Vol 11 (1) ◽  
pp. 71
Author(s):  
Yong-Soo Byun ◽  
Hyun Jung Lee ◽  
Soojung Shin ◽  
Moon Young Choi ◽  
Hyung-Seung Kim ◽  
...  
Keyword(s):  
Ocular Surface ◽  
Area Under The Curve ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Ocular Surface Disease Index ◽  
Dry Eyes ◽  
Potential Biomarker ◽  
Surface Staining ◽  
Breakup Time

Autophagy has been suggested to have an important role in the pathogenesis of Sjögren syndrome (SS). We previously identified that autophagy related 5 (ATG5) was elevated in the tear and conjunctival epithelial cells of SS dry eyes (DE) compared to non-SS DE. The purpose of this study was to investigate the role of tear ATG5 as a potential biomarker in the diagnosis of SS. To confirm this hypothesis, we evaluated the tear ATG5 concentration, and other ocular tests (Schirmer I, tear breakup time (TBUT), ocular surface staining (OSS) score, ocular surface disease index (OSDI)) in SS and non-DE, and compared their diagnostic performance to discriminate SS from non-SS DE. Tear ATG5 showed the greatest area under the curve (AUC = 0.984; 95% CI, 0.930 to 0.999) among the tests, and a 94.6% sensitivity and 93.6% specificity at a cutoff value of >4.0 ng/mL/μg. Our data demonstrated that tear ATG5 may be helpful as an ocular biomarker to diagnose and assess SS. In the future, the diagnostic power of tear ATG for SS should be validated.

Prognostic markers of lymphoma development in primary Sjögren syndrome

Lupus ◽  
2019 ◽  
Vol 28 (8) ◽  
pp. 923-936 ◽  
Author(s):  
S Retamozo ◽  
P Brito-Zerón ◽  
M Ramos-Casals
Keyword(s):  
Risk Factors ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Sjögren Syndrome ◽  
Systemic Autoimmune Disease ◽  
Low Grade ◽  
Sjogren Syndrome ◽  
Close Link ◽  
Systemic Involvement ◽  
Primary Sjögren Syndrome

Sjögren syndrome is a systemic autoimmune disease that principally affects women between the fourth and sixth decades of life who present with sicca symptomatology caused by dryness of the main mucosal surfaces. The clinical spectrum of Sjögren syndrome extends from dryness to systemic involvement. Since 1978, Sjögren syndrome has been closely associated with an enhanced risk of lymphoma, one of the most severe complications a patient may develop. Primary Sjögren syndrome patients have a 10–44-fold greater risk of lymphoma than healthy individuals, higher than that reported for systemic lupus erythematosus and rheumatoid arthritis. The close link between lymphoma and Sjögren syndrome is clearly exemplified by the very specific type of lymphoma arising in Sjögren syndrome patients, mainly low-grade B-cell lymphomas (predominantly a marginal zone histological type) with primary extranodal involvement of the major salivary glands (overwhelmingly parotid), with a primordial role of cryoglobulinemic-related markers (both clinical and immunological). The most recent studies support a higher number of risk factors detected in an individual leads to a higher lymphoma risk. A close follow-up of high-risk groups with longitudinal assessments of all known risk factors, including cryoglobulin-related markers and EULAR Sjögren's syndrome disease activity index measurement in particular, is mandatory.

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