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Sjogren’s syndrome - Top 30 Publications

Degradation of proteoglycan 4/lubricin by cathepsin S: Potential mechanism for diminished ocular surface lubrication in Sjögren's syndrome.

Sjögren's syndrome (SS) is an autoimmune disease affecting the lacrimal and salivary glands with hallmark clinical symptoms of dry eye and dry mouth. Recently, markedly increased cathepsin S (CTSS) activity has been observed in the tears of SS patients. Proteoglycan 4 (PRG4), also known as lubricin, is an effective boundary lubricant that is naturally present on the ocular surface. While PRG4 is susceptible to proteolytic digestion, the potential effect of CTSS on PRG4 remains unknown. The objective of this study was to assess the ability of CTSS to enzymatically degrade purified PRG4, and PRG4 naturally present in human tears, and alter ocular surface boundary lubricating properties. To assess the potential time course and dose-dependency of PRG4 digestion by CTSS, full-length recombinant human PRG4 (rhPRG4) was incubated at 37 °C with or without CTSS in an enzymatic digestion buffer. Digestion of PRG4 by CTSS was also examined within normal human tear samples, both with and without supplementation by rhPRG4. Finally, digestion of endogenous PRG4 by CTSS, and the effect of a CTSS inhibitor, was examined in SS tears on Schirmer strips. Digestion products were separated on 3-8% SDS-PAGE and visualized by protein staining and western blotting. The boundary lubricating ability of rhPRG4 samples was assessed using an in vitro human eyelid-cornea friction test. Finally, SDS-PAGE protein stain bands resulting from rhPRG4 digestion were submitted for tandem mass spectrometry analysis to confirm their identity as PRG4 and identify non-tryptic cleavage sites. CTSS digested rhPRG4 in a time and dose dependent manner. CTSS digestion of rhPRG4 at 1% resulted in a time dependent decrease in the full-length, ∼460 kDa, monomeric rhPRG4 band, and an appearance of lower MW fragments. After 20 h, no full-length rhPRG4 was observed. Furthermore, with an increased relative enzyme concentration of 3%, no protein bands were observed after 2 h, indicating complete digestion of rhPRG4. Western blotting demonstrated PRG4 is present in normal human tears, and that rhPRG4, tears, and tears supplemented with rhPRG4 incubated with 3-9% CTSS demonstrated decreased intensity of high MW PRG4 bands, indicative of partial degradation by CTSS. Similarly, western blotting of PRG4 in SS tears incubated with CTSS demonstrated decreased intensity of high MW PRG4 bands, which was reversed in the presence of the CTSS inhibitor. CTSS treatment of rhPRG4 resulted in an increased friction coefficient, compared to untreated controls. Lastly, the lower MW bands were confirmed to be PRG4 fragments by tandem mass spectrometry, and 6 non-tryptic cleavage sites were identified. rhPRG4 is susceptible to proteolytic digestion by CTSS, both alone and in human tears, which results in diminished ocular surface boundary lubricating ability. Moreover, endogenous PRG4 is susceptible to proteolytic digestion by CTSS, both in normal and SS tears. Given the elevated activity of CTSS in SS tears, and the role intact PRG4 plays in ocular surface health and lubrication, degradation of PRG4 by CTSS is a potential mechanism for diminished ocular surface lubrication in SS. Collectively these results suggest that tear supplementation of PRG4 may be beneficial for SS patients.

Comparison of corneal biomechanics in Sjögren's syndrome and non-Sjögren's syndrome dry eyes by Scheimpflug based device.

To compare the corneal biomechanics of Sjögren's syndrome (SS) and non-SS dry eyes with Corneal Visualization Scheimpflug Technology (CorVis ST).

Haematological Malignancies in Systemic Sclerosis Patients: Case Reports and Review of the World Literature.

Background. The association of systemic sclerosis (SSc) and haematological cancers was reported in a large number of case reports and cohort studies, describing SSc patients with highly heterogeneous clinical pictures. Objective. We reviewed the literature to better describe SSc patients with haematological malignancies. Methods. SSc cases complicated by haematological malignancies described in the world literature were collected; other 2 cases referred to our centre were reported. Results. One hundred-thirty SSc subjects were collected from 1954 up to date. The mean age of patients at cancer diagnosis was 56.1 ± 16.7 years; 72% of patients were females. In 60% of cases, the diagnosis of haematological malignancy was described within 5 years of SSc diagnosis. In 7.8% of cases, coexistence of Sjögren's syndrome or other autoimmune disorders was cited. Sixty-six cases with lymphoma (in the majority of cases B-cell neoplasms), 28 with leukaemia (chronic lymphocytic form in 9), 14 with multiple myeloma plus one solitary IgM plasmocytoma, and 16 with myeloproliferative disorders were found. No specific SSc subsets seem to be related to haematological malignancies. Conclusions. We remarked the importance of clinical work-up in SSc, in order to early diagnose and treat eventual occult haematological malignancies, especially during the first years of the disease.

Sjögren's syndrome is associated with negatively variable impacts on domains of health-related quality of life: evidence from Short Form 36 questionnaire and a meta-analysis.

The purpose of this article was to systematically review the literature to identify the impact of primary Sjögren's syndrome (pSS) on specific health-related quality of life (HRQoL) domains.

Corrigendum: The Core Pattern Analysis on Chinese Herbal Medicine for Sjögren's syndrome: A Nationwide Population-Based Study.

This corrects the article DOI: 10.1038/srep09541.

Scleral Contact Lenses in an Academic Oculoplastics Clinic: Epidemiology and Emerging Considerations.

To describe the role and efficacy of scleral contact lenses (SCLs) in the treatment of progressive keratopathy in patients who have undergone periocular surgical procedures, to investigate the financial impact of these surgical interventions, and to demonstrate the role of oculoplastic surgery in improving scleral contact lens fit.

Characteristics of labial gland Mesenchymal Stem Cells of healthy individuals and patients with Sjögren syndrome: a preliminary study.

Sjögren's Syndrome (SS) is a systemic autoimmune disease characterized by focal lymphocytic infiltration into exocrine organs such as salivary and lacrimal glands, resulting in dry mouth and eyes, and other systemic injuries. The mesenchymal stem cells (MSCs) in the salivary glands of healthy individuals and patients with SS have not been extensively studied. The aim of this study was to eluciadate the characteristics of MSCs from the labial glands of healthy controls and of those from patients with SS in order to elucidate the related pathogenesis and uncover potential avenues for novel clinical interventions. Labial glands from patients with SS and healthy subjects were obtained, and MSCs isolated and cultured using the tissue adherent method. The MSC characteristics of the cultured cells were confirmed using morphology, proliferation, colony forming-unit (CFU) efficiency, and multipotentiality, including osteogenic, adipogenic, and salivary gland differentiation. The MSCs from the healthy controls and SS patients expressed characteristic mesenchymal stem cell markers including CD29, CD44, CD73, CD90, and CD105, and were negative for CD34, CD45, and CD106,also negative for the salivary gland epithelium markers (CD49f and CD117). Labial gland MSCs from both groups were capable of osteogenic and adipogenic differentiation. The CFU efficiency and adipogenic differentiation potential of MSCs were significantly lower in the SS group compared to the healthy controls. Cells from both groups could also be induced into salivary gland-like cells. Real-time PCR and immunofluorescence staining showed that the gene and protein expression of AMY1, AQP5, and ZO-1 in cells from the SS group were lower than that in cells from the healthy group. Thus, MSCs from the labial glands in patients with SS could lack certain characteristics and functions, especially related to salivary secretion. These preliminary data provided insights that could lead to the development of novel therapeutic strategies for the treatment of SS.

Are the women with Sjögren's Syndrome satisfied with their sexual activity?

Females with Sjögren's Syndrome (SS) often experience vaginal dryness and dyspareunia, along with glandular and extraglandular symptoms. We aimed to evaluate sexual function and life quality in women with SS.

Research and progress on ClC‑2 (Review).

Chloride channel 2 (ClC-2) is one of the nine mammalian members of the ClC family. The present review discusses the molecular properties of ClC‑2, including CLCN2, ClC‑2 promoter and the structural properties of ClC‑2 protein; physiological properties; functional properties, including the regulation of cell volume. The effects of ClC‑2 on the digestive, respiratory, circulatory, nervous and optical systems are also discussed, in addition to the mechanisms involved in the regulation of ClC‑2. The review then discusses the diseases associated with ClC‑2, including degeneration of the retina, Sjögren's syndrome, age‑related cataracts, degeneration of the testes, azoospermia, lung cancer, constipation, repair of impaired intestinal mucosa barrier, leukemia, cystic fibrosis, leukoencephalopathy, epilepsy and diabetes mellitus. It was concluded that future investigations of ClC‑2 are likely to be focused on developing specific drugs, activators and inhibitors regulating the expression of ClC‑2 to treat diseases associated with ClC‑2. The determination of CLCN2 is required to prevent and treat several diseases associated with ClC‑2.

Surgical Aortic Valve Replacement Following Early Sapien-XT Valve Failure: A First.

Early degeneration of prosthetic aortic valve in transcatheter aortic valve replacement (TAVR) is a rare complication.

Markedly high salivary and lacrimal CXCL17 levels in primary Sjögren's Syndrome.

Updates on Sjögren's syndrome: from proteomics to protein biomarkers.

Primary Sjögren's syndrome (pSS) is a complex heterogeneous autoimmune disorder, typically affecting exocrine glands. Recently, a great interest has arisen in searching for novel biomarkers able to improve the diagnostic work-up of the disease as well as the general assessment and the prognostic stratification of pSS patients. From this perspective, salivary proteomics has appeared as a promising tool considering that salivary proteins may closely reflect the underlying disease processes in the salivary glands. Areas covered: Here we will provide an update on the state of the art of proteomics in pSS, focusing in particular on putative novel biomarkers for the disease. There is a special focus on candidate salivary protein and their role in non-invasive diagnosis of pSS. Expert commentary: Proteomics represents an emerging throughput omics-based approach for use in diagnosis of pSS. The studies that have been presented in this review have provided major contributions towards the identification of putative protein biomarkers, that once validated, could be able not only to contribute to a non-invasive diagnosis of pSS but also to the stratification of different disease subsets, ultimately allowing a better comprehension of the disease.

Myeloid Populations in Systemic Autoimmune Diseases.

Systemic autoimmune diseases (SADs) encompass a wide spectrum of clinical signs as a reflection of their complex physiopathology. A variety of mechanisms related with the innate immune system are in the origin of the loss of self-tolerance in these diseases, and for most of them, the myeloid leukocytes are key actors. Monocytes, macrophages, dendritic cells, and neutrophils are first-line immune effectors located in the interface between innate and adaptive immunity. They are crucial in the organization of the local and systemic responses to damage-associated molecular patterns (DAMPs) and determine the intensity, orientation, and duration of the local immune response through the expression of chemokines, costimulatory or protolerogenic factors. In this review, we summarize the current knowledge about the role of the main myeloid populations in the induction and maintenance of systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), primary antiphospholipid antibody syndrome (PAPS), systemic sclerosis (SSc), and Sjögren's syndrome (SjS), based on the data from both mouse preclinical models and patients. According to these data, our challenge in the next few years is to better dissect the fine mechanisms underlying the pathological role of myeloid cells in these diseases in order to define specific cell subsets or proteins that can be potential targets for drug development.

Correlation between corneal innervation and inflammation evaluated with confocal microscopy and symptomatology in patients with dry eye syndromes: a preliminary study.

To evaluate corneal innervation and inflammatory cell infiltration using in vivo confocal microscopy (IVCM) and to correlate these findings with subjective symptoms of dry eye, as measured by the Ocular Surface Disease Index (OSDI) in patients with non-Sjögren's (NSDE) and Sjögren's syndrome dry eyes (SSDE).

Comparison of the diagnostic value of four scoring systems in primary sjögren's syndrome patients.

We attempted to evaluate the diagnostic value of different salivary gland ultrasonography (SGUS) scoring systems for primary sjögren's syndrome (pSS).

Systemic manifestations of primary Sjögren's syndrome in the NOD.B10Sn-H2(b)/J mouse model.

Animal models that recapitulate human disease are crucial for the study of Sjögren's Syndrome (SS). While several SS mouse models exist, there are few primary SS (pSS) models that mimic systemic disease manifestations seen in humans. Similar to pSS patients, NOD.B10Sn-H2(b)/J (NOD.B10) mice develop exocrine gland disease and anti-nuclear autoantibodies. However, the disease kinetics and spectrum of extra-glandular disease remain poorly characterized in this model. Our objective was to characterize local and systemic SS manifestations in depth in NOD.B10 female mice at early and late disease time points. To this end, sera, exocrine tissue, lung, and kidney were analyzed. NOD.B10 mice have robust lymphocytic infiltration of salivary and lacrimal tissue. In addition, they exhibit significant renal and pulmonary inflammation. We identified numerous autoantibodies, including those directed against salivary proteins. In conclusion, the NOD.B10 model recapitulates both local and systemic pSS disease and represents an excellent model for translational studies.

Coexistence of ulcerative colitis and Sjögren's syndrome in a patient with Takayasu's arteritis and Hashimoto's thyroiditis.

A 31-year-old woman with a 15-year history of Takayasu's arteritis (TA) and a 13-year history of Hashimoto's thyroiditis presented with hematochezia. She received a diagnosis of Sjögren's syndrome at 1 month before her visit to Kyungpook National University Medical Center. Her colonoscopic findings were compatible with a diagnosis of ulcerative colitis (UC). She was treated with oral mesalazine, and her hematochezia symptoms subsequently disappeared. The coexistence of UC and TA has been reported; however, reports on the coexistence of UC and Sjögren's syndrome, or of UC and Hashimoto's thyroiditis are rare. Although the precise etiologies of these diseases are unknown, their presence together suggests that they may have a common pathophysiologic background. Furthermore, in patients with autoimmune or vascular diseases, including TA, systemic manifestations should be assessed with consideration of inflammatory bowel diseases including UC in the presence of gastrointestinal symptoms such as diarrhea and hematochezia.

Multiomic disease signatures converge to cytotoxic CD8 T cells in primary Sjögren's syndrome.

Multiomics study was conducted to elucidate the crucial molecular mechanisms of primary Sjögren's syndrome (SS) pathology.

Classification and diagnostic criteria in Sjögren's syndrome: a long-standing and still open controversy.

Sialendoscopic management of autoimmune sialadenitis: a review of literature.

Autoimmune diseases of major salivary glands include Sjögren's syndrome and a complex of disorders classified as immunoglobulin G4-related diseases. These pathologies are characterised by an autoimmune reaction mediated by T-helper lymphocytes that targets the ducts of exocrine glands in Sjögren's syndrome and glandular parenchyma in immunoglobulin G4-related diseases. Immunoglobulin G4-related diseases represent recently introduced multi-organ diseases that also involve the salivary glands. However, the morbid conditions once known as Mikulicz's disease and Kuttner's tumour were recently considered as two variants of immunoglobulin G4-related diseases affecting the major salivary glands ( immunoglobulin G4-related sialadenitis). This review briefly summarises the pathogenesis and clinical features of autoimmune diseases of the major salivary glands, focusing on the diagnostic and therapeutic role of sialendoscopy.

Salivary biomarkers and proteomics: future diagnostic and clinical utilities.

Saliva testing is a non-invasive and inexpensive test that can serve as a source of information useful for diagnosis of disease. As we enter the era of genomic technologies and -omic research, collection of saliva has increased. Recent proteomic platforms have analysed the human salivary proteome and characterised about 3000 differentially expressed proteins and peptides: in saliva, more than 90% of proteins in weight are derived from the secretion of three couples of "major" glands; all the other components are derived from minor glands, gingival crevicular fluid, mucosal exudates and oral microflora. The most common aim of proteomic analysis is to discriminate between physiological and pathological conditions. A proteomic protocol to analyze the whole saliva proteome is not currently available. It is possible distinguish two type of proteomic platforms: top-down proteomics investigates intact naturally-occurring structure of a protein under examination; bottom-up proteomics analyses peptide fragments after pre-digestion (typically with trypsin). Because of this heterogeneity, many different biomarkers may be proposed for the same pathology. The salivary proteome has been characterised in several diseases: oral squamous cell carcinoma and oral leukoplakia, chronic graft-versus-host disease Sjögren's syndrome and other autoimmune disorders such as SAPHO, schizophrenia and bipolar disorder, and genetic diseases like Down's Syndrome and Wilson disease. The results of research reported herein suggest that in the near future human saliva will be a relevant diagnostic fluid for clinical diagnosis and prognosis.

RAPID3 correlates with ESSPRI and other patient-reported outcomes in patients with primary Sjögren's syndrome.


Secondary Sjogren's Syndrome Presenting with Distal Tubular Acidosis and Quadriparesis.

A 52-year-old female patient was admitted to Intensive Care Unit with complaints of quadriparesis. Investigations revealed distal renal tubular acidosis (DRTA) secondary to Sjogren's syndrome with involvement of the parotid and thyroid glands. Laboratory investigations showed hyperchloremic metabolic acidosis and an alkaline urine pH with clinical signs of sicca syndrome. Sjogren's syndrome is associated with DRTA and occurrences of quadriparetic hypokalemia, nephrolithiasis, and osteomalacia can be prevented with early diagnosis and lifelong treatment with potassium and alkali replacement.

Malignancy as a comorbidity in rheumatic diseases: a retrospective hospital-based study.

Patients with Rheumatic diseases (RDs) are at an increased risk of malignancies compared with the general population. The aim of this study was to examine the relative frequency of several cancers in a single homogeneous cohort of patients with different RDs. Patients diagnosed with rheumatoid arthritis (RA), Ankylosing spondylitis (AS), Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermatomyositis (DM), or polymyositis were included. Out of 3982 adult residents admitted to the division of rheumatology, 61 malignancies were observed. The 2009 National Central Cancer Registry (NCCR) of China served as the reference for calculating standardized ratio (SR). The malignancy frequency had no difference between RDs with malignancy and the general population. Patients with SS and DM/PM showed an increased risk of non-Hodgkin's lymphoma (SR for SS patients = 9.709, 95% confidence interval (CI) = 4.602 to 17.916; SR for DM/PM = 35.714, 95% CI = 25.001 to 49.527). Patients with DM/PM and SSc showed an increased risk of lung cancer (SR for DM/PM = 10.638, 95% CI = 5.245 to 19.131; SR for SSc patients = 7.752, 95% CI = 3.295 to 15.309). Patients with SS and DM/PM showed an increased risk of ovary cancer (SR for SS patients = 8.177, 95% CI = 3.566 to 15.888; SR for DM/PM = 32.258, 95% CI = 22.126 to 45.490). Patients with SLE showed an increased risk of cervix cancer (SR for AS patients = 6.897, 95% CI = 2.748 to 14.144). Patients with AS showed an increased risk of pancreas cancer (SR for AS patients = 7.576, 95% CI = 2.181 to 15. 071). Different RDs have an increased risk of particular cancers. Among hematologic cancers, the risk of non-Hodgkin's lymphoma was higher than general population. Among solid tumors, the risk of cancers of the lung, ovary, cervix, and pancreas was higher than general population.

Difference in clinical presentation between women and men in incident primary Sjögren's syndrome.

A more severe disease phenotype has been reported in men compared to women in several rheumatic diseases. However, studies have not conclusively established sex-related clinical features in primary Sjögren's syndrome (pSS). In this study, we therefore investigated the clinical presentation of pSS in women and men at diagnosis.

The P2X7 receptor-NLRP3 inflammasome complex predicts the development of non-Hodgkin's lymphoma in Sjogren's syndrome: a prospective, observational, single-center study.

P2X7 receptor (P2X7R), trigger of acute inflammatory responses via the NLRP3 inflammasome, is hyperfunctioning in patients with Sjogren Syndrome (SS), where it stimulates IL-18 production. Some patients with SS develop a mucosa-associated lymphoid tissue non-Hodgkin's lymphoma (MALT-NHL).

Associations between maternal risk factors of adverse pregnancy and birth outcomes and the offspring epigenetic clock of gestational age at birth.

A recent study has shown that it is possible to accurately estimate gestational age (GA) at birth from the DNA methylation (DNAm) of fetal umbilical cord blood/newborn blood spots. This DNAm GA predictor may provide additional information relevant to developmental stage. In 814 mother-neonate pairs, we evaluated the associations between DNAm GA and a number of maternal and offspring characteristics. These characteristics reflect prenatal environmental adversity and are expected to influence newborn developmental stage.

Is hydroxychloroquine effective in treating primary Sjogren's syndrome: a systematic review and meta-analysis.

To systematically review and assess the efficacy and safety of hydroxychloroquine (HCQ) for treating primary Sjogren's syndrome (pSS).

MALT Lymphoma of Minor Salivary Glands in a Sjögren's Syndrome Patient: a Case Report and Review of Literature.

Sjögren's syndrome is a chronic systemic disease, characterized by lymphocytic infiltration and destruction mainly of the salivary and lacrimal glands, resulting in xerostomia and xeropthalmia. Sjögren's syndrome patients have a 44-fold excess risk for the development of non-Hodgkin's lymphoma particularly mucosa-associated lymphoid tissue (MALT) lymphoma, prevalently affecting the major salivary glands. In this report, a rare case of MALT lymphoma of minor salivary glands in a patient with Sjögren's syndrome is described. A review of the published cases of MALT lymphoma located in the minor salivary glands of patients with Sjögren's syndrome is provided.

Functional Analysis of Dendritic Cells Generated from T-iPSCs from CD4+ T Cell Clones of Sjögren's Syndrome.

Although it is important to clarify the pathogenic functions of T cells in human samples, their examination is often limited due to difficulty in obtaining sufficient numbers of dendritic cells (DCs), used as antigen-presenting cells, especially in autoimmune diseases. We describe the generation of DCs from induced pluripotent stem cells derived from T cells (T-iPSCs). We reprogrammed CD4+ T cell clones from a patient with Sjögren's syndrome (SS) into iPSCs, which were differentiated into DCs (T-iPS-DCs). T-iPS-DCs had dendritic cell-like morphology, and expressed CD11c, HLA-DR, CD80, CD86, and also BDCA-3. Compared with monocyte-derived DCs, the capacity for antigen processing was similar, and T-iPS-DCs induced the proliferative response of autoreactive CD4+ T cells. Moreover, we could evaluate T cell functions of the patient with SS. In conclusion, we obtained adequate numbers of DCs from T-iPSCs, which could be used to characterize pathogenic T cells in autoimmune diseases such as SS.