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.

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 Cerebellar degeneration in primary Sjögren syndrome: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Seow Lin Chuah ◽  
Ahmad Tirmizi Jobli ◽  
Sharifah Aishah Wan ◽  
Cheng Lay Teh
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Immunosuppressive Agents ◽  
Cerebellar Atrophy ◽  
Central Nervous System Involvement ◽  
Cerebellar Degeneration ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Resonance Imaging ◽  
Primary Sjögren Syndrome

Abstract Background Cerebellar degeneration is a rare and severe presentation of primary Sjögren syndrome. There are few case reports of cerebellar degeneration associated with different autoimmune diseases, especially with systemic lupus erythematosus and neuro-Behcet’s disease. There are only six patients reported worldwide to be affected by cerebellar atrophy associated with primary Sjögren syndrome. In this report, we describe a patient with primary Sjögren syndrome who presented with ataxia due to cerebellar degeneration. Case presentation We report the case of a 37-year-old Chinese woman with primary Sjögren syndrome who presented with ataxia over 3 months associated with tremor of the limbs. Magnetic resonance imaging of the brain revealed bilateral cerebellar atrophy. Based on the presence of cerebellar signs with magnetic resonance imaging brain findings, she was diagnosed as cerebellar degeneration secondary to primary Sjögren syndrome. She was treated with methylprednisolone, hydroxychloroquine, and two cycles of monthly intravenous cyclophosphamide. Subsequently, she refused further treatment, and her neurological symptoms remained the same upon the last clinic review. Primary cerebellar degeneration is rarely associated with primary Sjögren syndrome. The pathogenesis of the neurological manifestations in primary Sjögren syndrome is unclear. Treatment involves corticosteroids and immunosuppressive agents with no consensus of a specific therapy for the management of primary Sjögren syndrome with central nervous system involvement. Conclusions Cerebellar degeneration is a rare presentation of primary Sjögren syndrome. Early diagnosis and treatment of this condition is needed to ensure a good outcome.

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