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.

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.

scholarly journals Associations between Sjögren Syndrome, Sociodemographic Factors, Comorbid Conditions, and Scleritis in a Taiwanese Population-Based Study

2022 ◽  
Vol 12 (1) ◽  
pp. 105
Author(s):  
Ren-Long Jan ◽  
Chung-Han Ho ◽  
Jhi-Joung Wang ◽  
Sung-Huei Tseng ◽  
Yuh-Shin Chang
Keyword(s):  
Logistic Regression ◽  
Lupus Erythematosus ◽  
Population Based ◽  
Sjögren Syndrome ◽  
Systemic Autoimmune Disease ◽  
Sjogren Syndrome ◽  
Research Database ◽  
Continuous Variables ◽  
Comorbid Conditions ◽  
Ocular Surgery

This nationwide, population-based, retrospective, matched case–control study included 111,960 newly diagnosed patients with scleritis who were identified by the International Classification of Diseases, Ninth Revision, Clinical Modification code 379.0, selected from the Taiwan National Health Insurance Research Database. Demographic characteristics, Sjögren syndrome, and comorbid conditions within 1 year before the scleritis diagnosis were examined using univariate logistic regression analyses, and a paired t-test was used for continuous variables. Adjusted logistic regression was used to compare the prognosis odds ratio (OR) of the patients with scleritis with the controls. After adjustment for confounders, patients with Sjögren syndrome were remarkably more likely to have scleritis than the controls (OR = 33.53, 95% confidence interval (CI) = 27.43–40.97, p < 0.001). Other conditions found to have increased odds of scleritis included post ocular pterygium, glaucoma, and scleral surgery (OR  =  4.01, 95% CI  =  3.64–4.43; OR  =  3.16, 95% CI  =  2.24–4.47; OR  =  6.83, 95% CI  =  5.34–8.74, respectively); systemic infections, such as syphilis, tuberculosis, and a human herpes viral infection (OR  =  4.01, 95% CI  =  2.93–5.50; OR  =  2.24, 95% CI  =  1.94–2.58; OR  =  8.54, 95% CI  =  8.07–9.03, respectively); and systemic diseases, such as rheumatoid arthritis, granulomatous vasculitis, systemic lupus erythematosus, ankylosing spondylitis, and gout (OR  =  2.93, 95% CI  =  2.66–3.23; OR  =  7.37, 95% CI  =  3.91–13.88; OR  =  3.18, 95% CI  =  2.63–3.85; OR  =  5.57, 95% CI  =  4.99–6.22; OR  =  2.84, 95% CI  =  2.72–2.96, respectively). The results strongly support an association between Sjögren syndrome, post ocular surgery, systemic infection disease, systemic autoimmune disease, and scleritis.

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