Development Of pSS Research Articles

Hepatitis Delta Virus Detected in Salivary Glands of Sjögren's Syndrome Patients and Recapitulates a Sjögren's Syndrome-Like Phenotype in Vivo.

Background:Low-level, chronic viral infections have been suspect in the development of select autoimmune diseases, including primary Sjögren's syndrome (pSS). Multiple studies have shown stimulation of antiviral response pathways in pSS tissues suggestive of a viral infection. Yet, with this data in hand, a causal link between a viral infection and development of pSS had not been identified. Therefore, a study was designed to further define the viral landscape within pSS-affected salivary gland tissue to identify potential viral-mediated triggers in the pathogenesis of this autoimmune disease.Methods:A viral microarray was utilized to measure viral transcripts present in salivary gland tissue from patients diagnosed with pSS compared to healthy controls. Murine models of salivary gland localized HDV antigen expression were developed to evaluate the capacity of a chronic HDV signature to trigger the development of a pSS-like phenotype.Results:Through this analysis, two distinct viral profiles were identified, including the increased presence of hepatitis delta virus (HDV) in 50% of pSS patients evaluated. Presence of HDV antigen and sequence were confirmed in minor salivary gland tissue. Patients with elevated HDV levels in salivary gland tissue were negative for detectible hepatitis B virus (HBV) surface antigen and antibodies to HBV or HDV. Expression of HDV antigens in vivo resulted in reduced stimulated saliva flow, increase in focal lymphocytic infiltrates, and development of autoantibodies.Conclusion:Identification of HDV in pSS patients and induction of a complete pSS-like phenotype in vivo provides further support of a viral-mediated etiopathology in the development of pSS.

Risk of Sjögren's syndrome in Taiwanese female adults with irregular menstrual cycles: a population-based case-control study.

Primary Sjögren's syndrome (pSS) is a progressive systemic autoimmune disorder with a strong female predominance. Hormonal influences are thought to play a role in the development of pSS. However, no studies have specifically evaluated the association between irregular menstrual cycles and pSS. Therefore, using a health claims database, this study investigated the risk of pSS in women with irregular menstrual cycles. We conducted a case-control study using the Taiwan's National Health Insurance Research Database. A total of 360 patients diagnosed with pSS (International Classification of Diseases, ninth revision, clinical modification, ICD-9-CM code 710.2) between 2001 and 2012 were identified. Controls were frequency-matched at a rate of 5:1 to the cases by five-year age interval and index year. Both cases and controls were retrospectively traced back until 2001 for the diagnosis of irregular menstrual cycles (ICD-9-CM code 626.4). The risk of pSS was assessed using multivariate logistic regression analyses. Irregular menstrual cycles were significantly associated with pSS [adjusted odds ratio, (AOR) = 1.38, p = 0.027], after adjusted for insured amount, urbanization level, and thyroid disorder. In addition, when the data were stratified by three age categories, only the patients in the age category of 45-55 years showed significant association between irregular menstrual cycles and pSS (AOR = 1.74, p = 0.005). In this nationwide, population-based case-control study, we found a significant increased risk of pSS in female patients with irregular menstrual cycles, particularly those in their mid-forties to mid-fifties.

Association of BAFF and IL-17A with subphenotypes of primary Sjögren's syndrome.

Primary Sjögren's syndrome (pSS) is an autoimmune disease affecting exocrine glands. Both autoreactive T cells and B cells are involved in the development of pSS, but their exact contribution to the pathogenesis is not clear. Here, we aimed to investigate the association of B-cell activating factor (BAFF) and interleukin (IL)-17A with subphenotypes of pSS. Peripheral blood samples were collected from 31 pSS patients and 28 healthy controls. The serum levels of BAFF and IL-17A were quantified by sandwich ELISA. The increased circulating BAFF levels are associated with higher immunoglobulin G (IgG) levels (P=0.0167) and anti-Ro/SS antigen A autoantibody (P=0.032), while the elevated circulating levels of IL-17A are associated with lower C3 levels (P=0.0213) and higher focus score of salivary gland tissue (P=0.002). Our results show that BAFF and IL-17A are associated with different subphenotypes of pSS, suggesting both humoral and cellular immune response are involved in the pathogenesis of pSS.

The Clinical Relevance of IL-17-Producing CD4+CD161+ Cell and Its Subpopulations in Primary Sjögren's Syndrome.

Objective. Th17 cells have been demonstrated to play an important role in the onset and development of primary Sjögren's syndrome (pSS). In this study, we evaluated the expansion and clinical significance of circulating CD4+CD161+ T cell and its “effector” (CD4+CD25−CD161+ T cell) and “regulatory” (CD4+CD25+CD161+ T cell) subpopulations. Methods. Fifty-eight pSS patients and 16 healthy controls (HCs) were recruited in our study. The cell populations and intracellular IL-17 expression were analyzed by flow cytometry. The disease activity was evaluated by the EULAR-SS Disease Activity Index (ESSDAI). Autoantibodies were measured by ELISA or indirect immunofluorescence assay. Results. The CD161+ T cell fractions showed higher proportions of IL-17-producing cells. The frequencies of the overall CD4+CD161+ T cell population and its effector subset were positively correlated with disease activity parameters and more severe disease manifestations. A significant elevation of the CD4+CD25+CD161+ T cell subpopulation was observed in the peripheral blood of pSS patients compared to HCs and this subset showed decreased regulatory functions compared with the CD4+CD25+CD161− population. Conclusion. Circulating CD4+CD161+ T cell populations associated with pSS disease activity and severity. These cells might be involved in the development of pSS and could be potential therapeutic targets in the treatment of pSS.

Upregulated expression level of the growth factor, progranulin, is associated with the development of primary Sjögren's syndrome.

The aim of the present study was to investigate the expression and effect of progranulin (PGRN) in patients with primary Sjögren’s syndrome (pSS). In total, 26 newly diagnosed pSS patients and 26 healthy subjects were enrolled in this study. The serum levels of PGRN and the inflammatory factor, interleukin-6 (IL-6), were detected using ELISA. In addition, the mRNA expression levels of these molecules were detected by quantitative polymerase chain reaction. The serum levels of PGRN and IL-6 in the pSS patients increased significantly compared with the healthy controls (P<0.05). During the remission stages, the levels of PGRN and IL-6 were comparable to those of the healthy controls. The serum level of PGRN in the pSS patients was shown to correlate with that of IL-6 in the pre-treatment and post-treatment stages. PGRN was upregulated in the pSS patients, indicating a possible role of PGRN in the pathogenesis and development of pSS.

Increased levels of serum galectin-3 in patients with primary Sjögren’s syndrome: Associated with interstitial lung disease

ObjectivesTo explore the potential values of serum galectin-3 (Gal-3) levels in diagnosis of interstitial lung disease (ILD) for patients with primary Sjögren’s syndrome (pSS). MethodsThe concentrations of serum Gal-3 and interleukin (IL)-17 were measured by enzymelinked immunosorbent assay (ELISA) in 87 patients with pSS and 30 healthy controls (HC). The levels of plasma C-reactive protein (CRP), rheumatoid factor (RF), immunoglobulin (Ig)G, complement (C3), albumin (ALB) and Fibrinogen (FIB) and erythrocyte sedimentation rate (ESR) were measured. ILD was identified on high-resolution computed tomography. ResultsThe levels of serum Gal-3 and IL-17 were significantly higher in pSS patients than in HC. Stratification analyses indicated significantly higher levels of Gal-3 in pSS patients with ILD and in those with positive ANCA. In comparison with that of pSS patients without ILD, significantly higher levels of ESR, CRP, FIB, IgG, C3 and lower ALB were detected in pSS patients with ILD. The levels of galectin-3 were correlated positively with the values of CRP, FIB, IgG or IL-17 in patients with pSS. ConclusionsThese findings suggest that higher levels of serum galectin-3 may be associated with the development of pSS, particularly with ILD.

A Method for Impact Analysis of Cyclic Changes within Innovation Processes of PSS

Innovation processes are subject to various internal and external cycles. Changes of the environment of the system from a business perspective such as the readiness of new technologies or shifting requirements due to changing customer demands, or other cycles can affect the system as they significantly influence its innovation process. Especially companies providing PSS are affected by these cycles because physical product and service aspects as well as a high number of stakeholders have to be integrated during the design and development phase. While in conventional development processes dynamics caused by the occurrence of cycles already play a major role, the influence of dynamic aspects in the development of PSS is further enhanced. In particular, the high degree of crosslinking between the different phases of the innovation process leads to highly dynamic responses within the innovation process. A lack of knowledge and the challenging handling of these cycles and their dependencies and effects lead to challenges. In highly dynamic systems, the classical approaches of process modelling often reach their limits, as the depicted elements and relations are usually assumed to be static. Consequently, it is hard to predict the dynamic impact of cyclic changes on the innovation process of PSS. There are approaches to model the dynamics of systems such as System Dynamics, agent-based modelling or discrete event simulation. Especially System Dynamics is used throughout the public and private sector for policy analysis and design. It is a method to analyse and simulate the dynamic behaviour of systems on a high level of abstraction. Therefore, within this paper, a System Dynamics based impact analysis of cyclic changes within innovation processes of PSS is suggested. We provide background information on the innovation process of PSS, how they are challenged by cycles and how this approach can be used for modelling innovation processes and on the influence of cycles on these processes. An exemplary case study is presented to show the application of our approach on an innovation process of a PSS.

Innate Immune Signaling Induces Interleukin-7 Production from Salivary Gland Cells and Accelerates the Development of Primary Sjӧgren’s Syndrome in a Mouse Model

Elevated IL-7 in the target tissues is closely associated with multiple autoimmune disorders, including Sjögren’s syndrome (SS). We recently found that IL-7 plays an essential role in the development and onset of primary SS (pSS) in C57BL/6.NOD-Aec1Aec2 mice, a well-defined mouse model of primary SS. However, environmental signals that cause excessive IL-7 production are not well-characterized. Innate immune signaling plays a critical role in shaping the adaptive immune responses including autoimmune responses. We and others have previously shown that innate immune signaling can induce IL-7 expression in lungs and intestines of C57BL/6 mice. In this study, we characterized the effects of poly I:C, a double-stranded RNA analog and toll-like receptor 3 agonist, on the induction of IL-7 expression in salivary glands and on pSS development. We showed that poly I:C administration to C57BL/6 mice rapidly induced IL-7 expression in the salivary glands in a type 1 IFN- and IFN-γ-dependent manner. Moreover, poly I:C-induced IL-7 contributed to the optimal up-regulation of CXCL9 in the salivary glands, which may subsequently promote recruitment of more IFN-γ-producing T cells. Repeated administration of poly I:C to C57BL/6.NOD-Aec1Aec2 mice accelerated the development of SS-like exocrinopathy, and this effect was abolished by the blockade of IL-7 receptor signaling with a neutralizing antibody. Finally, poly I:C or a combination of IFN-α and IFN-γ induced IL-7 gene expression and protein production in a human salivary gland epithelial cell line. Hence, we demonstrate that IL-7 expression in the salivary gland cells can be induced by poly I:C and delineate a crucial mechanism by which innate immune signals facilitate the development of pSS, which is through induction of IL-7 in the target tissues.

Spray-On Socks: Ethics, Agency, and the Design of Product-Service Systems

It may be absurd to suggest that people in the future would choose to consume sock-services as convoluted as the ones de-scribed. However, the development of PSS as a model of provision makes clear that such schemes are being considered and it is certainly not beyond the realm of imagination to suppose that producers are thinking of ways to make these kinds of arrangements a reality. Through an analysis of the interaction of the ontological coordinates set by PSS itself—the product, the service, and the system—it is argued that the key challenge that the designer faces in such situations is ethical in character. Not because designers are considered to be the ultimate arbiters of good or bad; rather, since the focus of research has moved from the nature of objects to the behavior of people, it is suggested that design for use has become the design of use. The intention, therefore, is to question what conditions govern the way in which designers can operate in the emerging systemic paradigm of design, making, and provision.

FRI0042 Baff signaling is abnormally regulated through JAK pathways in peripheral monocytes in patients with primary sjÖgren’s syndrome

BackgroundB cell activating factor belonging to the TNF superfamily (BAFF) regulates proliferation, differentiation and survival of B cells and plays a pivotal role in the pathogenesis of autoimmune diseases such...

The Association between TNF-α, IL-10 Gene Polymorphisms and Primary Sjögren’s Syndrome: A Meta-Analysis and Systemic Review

ObjectivePrevious studies have evaluated the associations of TNF-α, IL-10 gene polymorphisms and susceptibility to pSS, but the results remained controversial. To assess the associations between TNF-α-308, IL-10-1082, -819, -592 polymorphisms and pSS risk, a meta-analysis was conducted.MethodThe available articles were searched in PubMed, EMBASE and MEDLINE. ORs with 95% CIs were calculated to determine the strength of associations by fixed-effects or random-effects models. The data about IL-10-1082, -819, -592 polymorphisms were analyzed in the additive, dominant and recessive modes. The associations between haplotypes of IL-10 gene and susceptibility to pSS were also assessed.ResultsA total of 9 relevant studies were identified in the meta-analyses. TNF-α-308 A allele was significantly associated with pSS (OR = 1.8, 95% CI: 1.53–2.13). The IL-10 -1082 G allele and the genotype “GCC/ATA” were identified as a candidate genetic risk factor for pSS. Under the dominant model for −819 and −582, the overall ORs suggested that individuals with genotype (CC+TC) or (CC+AC) may have a 59% increased risk of pSS in Caucasians population (OR = 1.59, 95% CI:1.09–1.23). Besides, the genotype “ATA/ATA” may be a protective factor against the development of pSS in Caucasians (OR = 0.40, 95% CI:0.19–0.84).ConclusionThe meta-analysis demonstrated TNF-α-308 A, IL-10-1082 G allele were significantly associated with pSS susceptibility, supporting these alleles were predisposing factors for pSS. In Caucasian population, the genotype “ATA/ATA” may be a protective factors.

Interleukin-10 gene polymorphisms (-1082 A/G, -819 C/T, -592 C/A) in primary Sj�gren's syndrome

Event Abstract Back to Event Interleukin-10 gene polymorphisms (-1082 A/G, -819 C/T, -592 C/A) in primary Sjögren's syndrome Edith Oregon-Romero1*, Mirna Vázquez-Villamar1, Diana C. Salazar-Camarena1, Beatriz A. Treviño-Talavera1, Gerardo Orozco-Barocio2, José F. Muñoz-Valle1, Yeminia M. Valle-Delgadillo1 and Claudia A. Palafox-Sánchez1, 2 1 Universidad de Guadalajara, Mexico 2 Hospital General de Occidente, Servicio de Reumatología, Mexico Introduction: Primary Sjögren´s syndrome (pSS) is an autoimmune disease of unknown etiology affecting primarily salivary and lacrimal exocrine glands. It is characterized by a progressive lymphocytic infiltration where B cells produce a great variety of antibodies, including those against ribonucleoproteins Ro and La. IL-10 plays an important role in pSS by promoting the differentiation of B cells inducing antibody production. Three polymorphic sites located at positions -1082 (A/G), -819 (C/T) and -592 (C/A) in the IL-10 promoter show a linkage disequilibrium and form haplotypes that may influence the IL-10 production and consequently an increased activity of the disease. Objective: To determine the frequency of haplotypes in the IL-10 promoter gene in patients with pSS. Methodology: The study included 113 Mexican mestizo patients with pSS, classified according to the American-European Consensus criteria, from the Department of Rheumatology (Hospital General de Occidente, SSJ). As control group, 154 clinically healthy subjects were included. The polymorphisms were genotyped by PCR/RFLP technique. Statistical analysis was carried out with the Stata 9.0 and EmHapFre software. Results: Polymorphisms were in Hardy–Weinberg equilibrium (-1082 A/G p=0.8377, -819C/T p= 0.1174, -592 C/A p=0.7953) and showed high linkage disequilibrium (100%, pc=1.42 x 10-34).The most frequent haplotypes were: ACC (41-61%), ATT (21.38%), GTA (14.86%), and GCC (11.18%). GTC haplotype was associated with a 4.35-fold less probability to develop pSS [OR; 95%CI: 0.23 (0.064-0.792) p= 0.01]. Conclusion: Our results suggest that carriers of the GTC haplotype have a protective status for the development of pSS. Keywords: primary Sjögren´s syndrome, Antibodies, IL-10 promoter, Linkage Disequilibrium, Haplotypes Conference: 15th International Congress of Immunology (ICI), Milan, Italy, 22 Aug - 27 Aug, 2013. Presentation Type: Abstract Topic: Immune-mediated disease pathogenesis Citation: Oregon-Romero E, Vázquez-Villamar M, Salazar-Camarena DC, Treviño-Talavera BA, Orozco-Barocio G, Muñoz-Valle JF, Valle-Delgadillo YM and Palafox-Sánchez CA (2013). Interleukin-10 gene polymorphisms (-1082 A/G, -819 C/T, -592 C/A) in primary Sjögren's syndrome. Front. Immunol. Conference Abstract: 15th International Congress of Immunology (ICI). doi: 10.3389/conf.fimmu.2013.02.00346 Copyright: The abstracts in this collection have not been subject to any Frontiers peer review or checks, and are not endorsed by Frontiers. They are made available through the Frontiers publishing platform as a service to conference organizers and presenters. The copyright in the individual abstracts is owned by the author of each abstract or his/her employer unless otherwise stated. Each abstract, as well as the collection of abstracts, are published under a Creative Commons CC-BY 4.0 (attribution) licence (https://creativecommons.org/licenses/by/4.0/) and may thus be reproduced, translated, adapted and be the subject of derivative works provided the authors and Frontiers are attributed. For Frontiers’ terms and conditions please see https://www.frontiersin.org/legal/terms-and-conditions. Received: 15 Mar 2013; Published Online: 22 Aug 2013. * Correspondence: Dr. Edith Oregon-Romero, Universidad de Guadalajara, Guadalajara, Mexico, oregon_edith@hotmail.com Login Required This action requires you to be registered with Frontiers and logged in. To register or login click here. Abstract Info Abstract The Authors in Frontiers Edith Oregon-Romero Mirna Vázquez-Villamar Diana C Salazar-Camarena Beatriz A Treviño-Talavera Gerardo Orozco-Barocio José F Muñoz-Valle Yeminia M Valle-Delgadillo Claudia A Palafox-Sánchez Google Edith Oregon-Romero Mirna Vázquez-Villamar Diana C Salazar-Camarena Beatriz A Treviño-Talavera Gerardo Orozco-Barocio José F Muñoz-Valle Yeminia M Valle-Delgadillo Claudia A Palafox-Sánchez Google Scholar Edith Oregon-Romero Mirna Vázquez-Villamar Diana C Salazar-Camarena Beatriz A Treviño-Talavera Gerardo Orozco-Barocio José F Muñoz-Valle Yeminia M Valle-Delgadillo Claudia A Palafox-Sánchez PubMed Edith Oregon-Romero Mirna Vázquez-Villamar Diana C Salazar-Camarena Beatriz A Treviño-Talavera Gerardo Orozco-Barocio José F Muñoz-Valle Yeminia M Valle-Delgadillo Claudia A Palafox-Sánchez Related Article in Frontiers Google Scholar PubMed Abstract Close Back to top Javascript is disabled. Please enable Javascript in your browser settings in order to see all the content on this page.

Association between genetic variants in the tumour necrosis factor/lymphotoxin α/lymphotoxin β locus and primary Sjögren's syndrome in Scandinavian samples

ObjectivesLymphotoxin β (LTB) has been found to be upregulated in salivary glands of patients with primary Sjögren's syndrome (pSS). An animal model of pSS also showed ablation of the lymphoid...

Primary Sjögren syndrome in a 2-year-old patient: role of the dentist in diagnosis and dental management with a 6-year follow-up

BACKGROUND. Primary Sjögren syndrome is a rare autoimmune disease, especially in children, mainly affecting girls (77%), and usually diagnosed around 10 years of age. Diagnosis during childhood is difficult, especially because of the diversity of the clinical presentation and difficulty obtaining reliable history data, accounting for a higher frequency of underdiagnosed cases. Differential conditions should be considered, especially the ones that promote xerostomia, such as diabetes, ectodermal dysplasia, rheumatoid arthritis, scleroderma, systemic lupus erythematosus, sarcoidosis, lymphoma, HIV and HTLV infection. Conditions associated with parotid enlargement should also be excluded, including juvenile recurrent parotitis (JRP), sialadenosis, sarcoidosis, lymphoma, infectious parotitis caused by streptococcal and staphylococcal infections, viral infections (paramyxovirus, Epstein-Barr virus, cytomegalovirus, and parvovirus), and diffuse infiltrative lymphocytosis syndrome (associated with HIV infection), and rare congenital conditions, such as polycystic parotid disease. CASE REPORT. A paediatric female patient was referred to our clinic for dental treatment complaining about dry mouth, oral discomfort, and dysphagia. The patient presented five of the required criteria to establish the diagnosis of pSS, including ocular symptoms, oral symptoms, evidence of keratoconjunctivitis sicca, focal sialadenitis confirmed by minor salivary gland biopsy, and evidence of major salivary gland involvement. Our patient did not have positive SS-A and SS-B autoantibodies. According to the literature, about 29% of individuals with pSS can present seronegativity for SS-A (anti-Ro) antibodies and about 33% can present seronegativity for SS-B (anti-La) antibodies. CONCLUSION. To the best of our knowledge, this is the youngest patient reported in the scientific English literature with pSS. Primary Sjögren syndrome has a wide clinical and immunologic spectrum and may progress with increased morbidity. Clinicians must be aware of the development of pSS in such an early age and exclude all possible differential findings to provide early diagnosis and treatment.

Status review and research strategies on product-service systems

As present trends in economic and population growth continue, the natural environment is increasingly being stressed. More and more researchers, institutes and programmes have paid attention to product-service systems (PSSes) in the last decade because PSS integrates tangible artefact and intangible service to achieve sustainability, improve enterprise competitiveness, and meet customer needs better. In order to respond to the industrial trend towards PSS and frame the related research, the state-of-the-art of PSS research and development are reported. Furthermore, in order to integrate fragmental PSS solutions, this paper proposes a framework for product-service lifecycle management (PSLM) and technologies of PSS development. So, four categories are focused on: review of PSS, research about PSS development, a framework for PSLM, and technologies for PSLM (modular product-service development and ontology-based product-service knowledge expression and reuse). The preliminary solution of the implementation roadmap and technologies for PSLM which need to be perfected is proposed to lay a basis for further development and application of PSS for the benefit of industries.

Regulatory mechanisms for the production of BAFF and IL-6 are impaired in monocytes of patients of primary Sjögren's syndrome

IntroductionIn this study, we investigated possible aberrations of monocytes from patients with primary Sjögren's syndrome (pSS). We focused on B-cell-activating factor of the TNF family (BAFF) and IL-6 because they are both produced by monocytes and are known to be involved in the pathogenesis of pSS.MethodsPeripheral monocytes were prepared from both pSS patients and normal individuals. The cells were stimulated in vitro with IFN-γ, and the amounts of IL-6 and soluble BAFF (sBAFF) produced by the cells were quantitated. The effect of sBAFF itself on the production of IL-6 was also studied. To investigate the response of pSS monocytes to these stimuli, the expression levels of the genes encoding BAFF receptors and IL-6-regulating transcription factors were quantitated.ResultsPeripheral pSS monocytes produced significantly higher amounts of sBAFF and IL-6 than normal monocytes did, even in the absence of stimulation. The production of these cytokines was significantly increased upon stimulation with IFN-γ. The elevated production of IL-6 was significantly suppressed by an anti-BAFF antibody. In addition, stimulation of pSS monocytes with sBAFF induced a significant increase in IL-6 production. Moreover, the expression levels of a BAFF receptor and transcription factors regulating IL-6 were significantly elevated in pSS monocytes compared to normal monocytes.ConclusionsThe results of the present study suggest that the mechanisms underlying the production of sBAFF and IL-6 are impaired in pSS monocytes. Our research implies that this impairment is due to abnormally overexpressed IL-6-regulating transcription factors and a BAFF receptor. These abnormalities may cause the development of pSS.

Serum immunogiobulin A and its clinical role in primary Sjogren's syndrome

Objective To study the relationship between elevated serum level of immunoglobulin A (IgA) and other abnormality in immunological parameters in patients with primary Sjogren's syndrome (pSS)and those with viscera involvement based on determinations of serologic,other laboratory and clinical features.Methods Serum IgA level of 108 patients with pSS,seven males and 101 females with an age of 30～78 years and length of disease course of 0.2～20.0 years,was detected by turbidimetry.Clinical and laboratory parameters in patients were compared between different groups.Remits Elevated serum IgA level was found in 48 (44.4%) of 108 patients with pSS.Elevated erythrocyte sedimentation rate(ESR) was found in 91.7 percent of those with elevated sertum IgA and 63.3 percent in those with normal IgA(P<0.05).Serum positivity of rheumatoid factor(RF) was significantly higher in those with elevated serum IgA (70.8%)than those with normal IgA (48.3%,P<0.05).Pulmonary interstitial fibrosis could be found in 22.9 percent of those with elevated serum IgA,and in 8.3 percent of those with normal IgA(P<0.05).Occurrences of primary biliary cirrhosis and autoimmune hepatitis were higher in those with elevated serum IgA than in those with normal IgA,but not reaching statistical significance.Conclusion Serum IgA level misht play an important role in occurrence and development of pSS,and could be used as an indicator reflecting activity and severity of the disease,as well. Key words: Primary sjogren's syndrome; Immunoglobulin A; Serologic tests

Incidence of cancer in a cohort of patients with primary Sjögren's syndrome

To compare the incidence of subsequent cancers in a cohort of patients with primary Sjögren's syndrome (pSS) with that of the general population in the same region of England. A retrospective analysis was carried out on 112 patients who had attended the out-patients department at University College Hospital, London, from 1979 onwards. Patients were followed up from diagnosis of pSS to diagnosis of first subsequent cancer, death, loss to follow-up or 31 December 2003 (the censoring date) to determine the person-years at risk for each individual. The expected numbers of subsequent cancers were calculated from sex-/age-/period-specific rates for the general population of southeast England, derived from registrations at the Thames Cancer Registry. Standardized incidence ratios (SIRs) were then calculated as the ratio of observed to expected numbers of cancers, along with 95% confidence intervals (CIs). Separate analyses were performed for all malignant cancers combined, lymphomas and non-lymphoid cancers. Among the 112 patients with pSS, 25 developed cancer (either before or after development of pSS), with lymphoma occurring in 11 cases. Nine patients had two cancers. There was a significantly elevated incidence of lymphomas in pSS patients compared with the general population (SIR 37.5, 95% CI 20.7-67.6). For non-lymphoid cancer, the observed increase in incidence was small and not statistically significant (SIR 1.5, 95% CI 0.9-2.6). This study confirms that there is an increased incidence of lymphoma in patients with pSS. An increase in the incidence of other cancers was not proven but the observation that some patients developed more than one cancer raises the possibility that there may be a subgroup of patients who are at greater risk of developing cancer.

Viruses contribute to the development of Sjögren's syndrome

Viruses contribute to the development of Sjögren's syndrome

Planning support systems: an inventory of current practice

Planning support systems are a subset of computer-based geo-information instruments, each of which incorporates a unique suite of components that planners can utilise to explore and manage their particular activities. The components may include data sets, computer algorithms and display facilities, as well as more abstract theoretical constructs, knowledge and modelling capabilities. PSS are used to support the planning process by communicating information as well as by generating solutions. An Internet-based inventory was established in 2000–2001 to identify the existence and use of PSS in planning practice worldwide. In this paper we take a closer look at the instruments recorded in the inventory and some of their main characteristics. Some recommendations are made for the development and application of PSS in the future.