Mechanisms of autoantibody production and the relationship between autoantibodies and the clinical manifestations in Sjögren’s syndrome

2006 ◽  
Vol 148 (6) ◽  
pp. 281-288 ◽  
Author(s):  
Hideki Nakamura ◽  
Atsushi Kawakami ◽  
Katsumi Eguchi
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Clinical Manifestations ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Autoantibody Production ◽  
The Relationship ◽  
Sjögren‘S Syndrome

scholarly journals Sjögren’s syndrome: a systemic autoimmune disease

2021 ◽  
Author(s):  
Simone Negrini ◽  
Giacomo Emmi ◽  
Monica Greco ◽  
Matteo Borro ◽  
Federica Sardanelli ◽  
...  
Keyword(s):  
Salivary Gland ◽  
Autoimmune Disease ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Autoantibody Production ◽  
Sjögren‘S Syndrome

AbstractSjögren’s syndrome is a chronic autoimmune disease characterized by ocular and oral dryness resulting from lacrimal and salivary gland dysfunction. Besides, a variety of systemic manifestations may occur, involving virtually any organ system. As a result, the disease is characterized by pleomorphic clinical manifestations whose characteristics and severity may vary greatly from one patient to another. Sjögren’s syndrome can be defined as primary or secondary, depending on whether it occurs alone or in association with other systemic autoimmune diseases, respectively. The pathogenesis of Sjögren’s syndrome is still elusive, nevertheless, different, not mutually exclusive, models involving genetic and environmental factors have been proposed to explain its development. Anyhow, the emergence of aberrant autoreactive B-lymphocytes, conducting to autoantibody production and immune complex formation, seems to be crucial in the development of the disease. The diagnosis of Sjögren’s syndrome is based on characteristic clinical signs and symptoms, as well as on specific tests including salivary gland histopathology and autoantibodies. Recently, new classification criteria and disease activity scores have been developed primarily for research purposes and they can also be useful tools in everyday clinical practice. Treatment of Sjögren’s syndrome ranges from local and symptomatic therapies aimed to control dryness to systemic medications, including disease-modifying agents and biological drugs. The objective of this review paper is to summarize the recent literature on Sjögren’s syndrome, starting from its pathogenesis to current therapeutic options.

scholarly journals Clinical manifestations in anti-Ro52/SS-a antibody-seropositive patients with Sjögren's syndrome

2021 ◽  
pp. 1-11
Author(s):  
Hideki Nakamura ◽  
Shimpei Morimoto ◽  
Toshimasa Shimizu ◽  
Ayuko Takatani ◽  
Shin-ya Nishihata ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Clinical Manifestations ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

scholarly journals Reevaluation for clinical manifestations of HTLV-I-seropositive patients with Sjögren’s syndrome

2015 ◽  
Vol 16 (1) ◽  
Author(s):  
Hideki Nakamura ◽  
Toshimasa Shimizu ◽  
Yukinori Takagi ◽  
Yoshiko Takahashi ◽  
Yoshiro Horai ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Clinical Manifestations ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sjögren‘S Syndrome

scholarly journals TLR7 Signaling Drives the Development of Sjögren’s Syndrome

2021 ◽  
Vol 12 ◽  
Author(s):  
Yawen Wang ◽  
Annie Roussel-Queval ◽  
Lionel Chasson ◽  
Noël Hanna Kazazian ◽  
Laetitia Marcadet ◽  
...  
Keyword(s):  
Dendritic Cells ◽  
Autoimmune Disease ◽  
Salivary Glands ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Autoantibody Production ◽  
Deficient Mice ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome (SS) is a chronic systemic autoimmune disease that affects predominately salivary and lacrimal glands. SS can occur alone or in combination with another autoimmune disease like systemic lupus erythematosus (SLE). Here we report that TLR7 signaling drives the development of SS since TLR8-deficient (TLR8ko) mice that develop lupus due to increased TLR7 signaling by dendritic cells, also develop an age-dependent secondary pathology similar to associated SS. The SS phenotype in TLR8ko mice is manifested by sialadenitis, increased anti-SSA and anti-SSB autoantibody production, immune complex deposition and increased cytokine production in salivary glands, as well as lung inflammation. Moreover, ectopic lymphoid structures characterized by B/T aggregates, formation of high endothelial venules and the presence of dendritic cells are formed in the salivary glands of TLR8ko mice. Interestingly, all these phenotypes are abrogated in double TLR7/8-deficient mice, suggesting that the SS phenotype in TLR8-deficient mice is TLR7-dependent. In addition, evaluation of TLR7 and inflammatory markers in the salivary glands of primary SS patients revealed significantly increased TLR7 expression levels compared to healthy individuals, that were positively correlated to TNF, LT-α, CXCL13 and CXCR5 expression. These findings establish an important role of TLR7 signaling for local and systemic SS disease manifestations, and inhibition of such will likely have therapeutic value.

scholarly journals STAT4, TRAF3IP2, IL10, and HCP5 Polymorphisms in Sjögren’s Syndrome: Association with Disease Susceptibility and Clinical Aspects

2019 ◽  
Vol 2019 ◽  
pp. 1-8 ◽  
Author(s):  
Serena Colafrancesco ◽  
Cinzia Ciccacci ◽  
Roberta Priori ◽  
Andrea Latini ◽  
Giovanna Picarelli ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Variant Allele ◽  
Clinical Aspects ◽  
Autoantibody Production ◽  
Focus Score ◽  
Risk Alleles ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome (SS) is a chronic autoimmune condition characterized by autoantibody production, sicca syndrome, and periepithelial lymphocytic lesions in target tissues. A predisposing genetic background is likely, and, to date, several polymorphisms in non-HLA genes have been explored with interesting results. We investigated the association between the STAT4, TRAF3IP2, HCP5, and IL10 polymorphisms and SS susceptibility and their possible role in the modulation of clinical and laboratory features. 195 consecutive patients with SS were enrolled and clinical and laboratory data were collected. 248 age- and sex-matched healthy subjects were used as controls. Genotyping was performed by allelic discrimination assays. A case-control association study and a phenotype-genotype correlation analysis were performed. A genetic risk profile was developed considering the risk alleles. Both the variant alleles of rs7574865 in the STAT4 gene and rs3099844 in the HCP5 gene were significantly more prevalent in patients than in controls (OR=1.91 and OR=2.44, respectively). The variant allele of rs3024505 of IL10 resulted to be a susceptibility allele (OR=1.52), while the variant allele of rs1800872 seemed to confer a protective effect for the development of the disease (OR=0.65). A risk genetic profile showed a higher probability to develop the disease in subjects with at least three risk alleles; subjects with 4 risk alleles were not observed in the controls. HCP5 rs3099844 was associated with anti-SSA (P=0.006, OR=3.07) and anti-SSB (P=0.005, OR=2.66) antibodies, severity of focus score (P=0.03, OR=12), and lymphoma development (P=0.002, OR=7.23). Patients carrying the STAT4 rs7574965 variant allele had a higher risk of monoclonal component and leukopenia (P=0.002, OR=7.6; P=0.048, OR=2.01, respectively). We confirmed the association of SS with the STAT4 and IL10 genes and we describe a novel association with HCP5. In particular, we describe an association of this specific SNP of HCP5 not only with disease development but also with autoantibody production and focus score suggesting a potential contribution of this variant to a more severe phenotype.

scholarly journals Current insights into the relationship between the gut microbiome and Sjögren’s syndrome

2020 ◽  
Vol 19 (1) ◽  
Author(s):  
Taco A. van der Meulen ◽  
Frans G. M. Kroese ◽  
Hendrika Bootsma ◽  
Fred K. L. Spijkervet ◽  
Arjan Vissink
Keyword(s):  
Sjögren’S Syndrome ◽  
Gut Microbiome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
The Relationship ◽  
Sjögren‘S Syndrome

scholarly journals MicroRNA in Sjögren’s Syndrome: Their Potential Roles in Pathogenesis and Diagnosis

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
M. Reale ◽  
C. D’Angelo ◽  
E. Costantini ◽  
M. Laus ◽  
A. Moretti ◽  
...  
Keyword(s):  
B Cells ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Lymphocytic Infiltration ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Exocrine Glands ◽  
Autoantibody Production ◽  
Systemic Autoimmunity ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome (SS) or sicca syndrome was described by Swedish ophthalmologist Sjögren in the year 1933 for the first time. The etiology of the SS is multifunctional and includes a combination of genetic predisposition and environmental as well as epigenetic factors. It is an autoimmune disease characterized by features of systemic autoimmunity, dysfunction, and inflammation in the exocrine glands (mainly salivary and lacrimal glands) and lymphocytic infiltration of exocrine glands. In fact, the involvement of lacrimal and salivary glands results in the typical features of dry eye and salivary dysfunction (xerostomia). Only in one-third of the patients also present systemic extraglandular manifestations. T cells were originally considered to play the initiating role in the autoimmune process, while B cells were restricted to autoantibody production. In recent years, it is understood that the roles of B cells are multiple. Moreover, autoantibodies and blood B cell analysis are major contributors to a clinical diagnosis of Sjögren’s syndrome. Recently, there has been rising interest in microRNA implication in autoimmunity. Unfortunately, to date, there are only a few studies that have investigated their participation in SS etiopathogenesis. The purpose of this work is to gather the data present in the literature to clarify this complex topic.

Similarities and Differences Between Primary and Secondary Sjögren’s Syndrome

2010 ◽  
Vol 37 (4) ◽  
pp. 800-808 ◽  
Author(s):  
GABRIELA HERNÁNDEZ-MOLINA ◽  
CARMEN ÁVILA-CASADO ◽  
FRANCISCO CÁRDENAS-VELÁZQUEZ ◽  
CARLOS HERNÁNDEZ-HERNÁNDEZ ◽  
MARÍA LUISA CALDERILLO ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sensitivity Analyses ◽  
Autoantibody Production ◽  
Secondary Sjögren’S Syndrome ◽  
Oral Symptoms ◽  
Secondary Sjögren's Syndrome ◽  
Sjögren‘S Syndrome

Objective.To define the clinical, serological, and histopathological characteristics of primary (pSS) and secondary Sjögren’s syndrome (SS).Methods.Fifty subjects with pSS and 300 with connective tissue diseases (CTD; systemic lupus erythematosus 100, rheumatoid arthritis 100, scleroderma 100) were selected randomly from our patient registry. Selected patients were assessed for fulfillment of the American-European Consensus Group criteria for SS using a 3-phase approach: screening (European questionnaire, Schirmer-I test, wafer test), confirmatory (fluorescein staining test, nonstimulated whole salivary flow, anti-Ro/La antibodies), and lip biopsy (H&E and immunohistochemical staining for anti-CD20 and anti-CD45RO scored by morphometry).Results.All patients with pSS and 65 with CTD met criteria for SS. Oral symptoms (pSS = 92% and secondary SS = 84%; p = 0.02), parotid enlargement (pSS 56%, secondary SS 9.2%; p < 0.001), and higher prevalence (pSS 82%, secondary SS 41%; p < 0.001) and titers of anti-Ro/La antibodies were more common in pSS. Extraglandular manifestations were similar in both groups, except for Raynaud’s phenomenon, which was more common in those with secondary SS (pSS 16% vs secondary SS 41%; p = 0.001). These results remained after 3 different sensitivity analyses. The prevalence of focal infiltration was also similar in both SS varieties; however, a higher B:T cell ratio and higher expression of CD20 cells (2922 vs 607.5 positive cells; p < 0.001) were observed in pSS.Conclusion.A higher frequency of oral symptoms and parotid enlargement and stronger B cell activity (autoantibody production and lymphocyte infiltration) were observed in pSS. Whether these results reflect a true difference between the 2 disease entities or derive from underlying variables remains uncertain.

scholarly journals DIAGNOSIS OF THE SYNDROME OF SCHEGREN. REVIEW

2018 ◽  
Vol 13 (3-4) ◽  
pp. 81-87
Author(s):  
D.V. Topchy
Keyword(s):  
Salivary Glands ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Connective Tissue Diseases ◽  
Clinical Manifestations ◽  
Magnetic Resonance Tomography ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Technical Equipment ◽  
Sjögren‘S Syndrome

Relevance. Sjogren's syndrome is included in the group of diffuse connective tissue diseases and ranks third among them. Sjögren's syndrome remains unidentified in half the cases of the disease with clinical manifestations. Diagnosis of Sjogren's syndrome in some cases has serious difficulties, which is due to both large polymorphism of Sjogren's syndrome and insufficient awareness of practical doctors in the clinic and diagnosis of this disease. Objective is to generalize the literature data on the possibilities of using radiation methods for early diagnosis of Sjogren's syndrome. Materials and methods. The literature sources on the possibility of using sialography, ultrasound examination, scintigraphy, magnetic resonance tomography of the salivary glands in the diagnosis of Sjogren's syndrome are analyzed. Data on the use of radiation diagnostics methods for revealing the initial signs of the disease, for assessing prevalence, for studying the salivary gland vascularization, for differential diagnosis of Sjogren's syndrome with sialadenitis and sialozema are presented. Results. Most authors consider sialografy to be a highly informative technique, which helps to identify symptoms in Sjogren's syndrome. In the ultrasound study, the authors determine the bilateral increase in the size of the parotid salivary glands. The advantage of scintigraphy of salivary glands in comparison with other methods is that the functions of all four major salivary glands (parotid and submaxillary) can be simultaneously evaluated with the help of a single injection of the isotope 99-m Tc-pertechnetate. Conclusion. In the presence of technical equipment, a comprehensive examination using methods of radiation diagnosis in case of suspicion of Sjogren's syndrome can become an origin of early, accurate and practically meaningful information.

Sign in / Sign up

Export Citation Format

Share Document