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ID PMID Title PublicationDate abstract
18752933 Anti-cyclic citrullinated peptide antibodies and IL-23p19 in psoriatic arthritis. 2009 Jan BACKGROUND: Anti-cyclic citrullinated peptide antibodies (anti-CCP) are reported to be found in 5-13% of patients with psoriatic arthritis (PsA). However, whether anti-CCP-positive PsA patients and rheumatoid arthritis (RA) patients have a similar pathophysiological background still remains uncertain. OBJECTIVE: To determine the prevalence of anti-CCP antibodies in patients with PsA and characterize these anti-CCP-positive patients of PsA. METHODS: We measured the serum levels of the anti-CCP antibodies in patients with PsA (n=16), psoriasis (n=15), RA (n=9) and healthy controls (n=11). Serum levels of rheumatoid factor (RF), matrix metalloproteinase-3 (MMP-3), cartilage oligomeric matrix protein (COMP), interleukin (IL)-23p19 and IL-12p40 were also measured in all the samples. RESULTS: Two of the 16 PsA patients (13%) were positive for anti-CCP antibodies with high titers of RF. However, the serum IL-23p19 levels were two orders of magnitude higher in the anti-CCP-positive PsA patients as compared with those in the RA patients and anti-CCP-negative PsA patients. No significant elevation of the serum levels of MMP-3, COMP and IL-12p40 was found in these patients. CONCLUSION: Thirteen percent of the PsA patients were positive for anti-CCP. These patients do not fulfill the American College of Rheumatology (ACR) classification criteria for RA so far. Furthermore, they showed the typical clinical features of PsA rather than those of RA. Although anti-CCP-positive PsA patients may possibly be have a risk of developing RA, we propose that these patients be classified, for the moment, into a independent subtype of PsA, as a different entity from RA.
21077037 Primary Sjögren's Syndrome with two extraglandular sites involvement - case report. 2010 Primary Sjögren's Syndrome (pSS) is a chronic, slowly progressive inflammatory autoimmune disorder, characterised by lymphocytic infiltration of the exocrine glands, leading to decrease of glandular secretion. In 40-60% of pSS patients, extraglandular disease develops. We present the case of a patient with two extraglandular sites involvement in the course of pSS manifesting with progressive respiratory and gastrointestinal symptoms.
20487531 The value of ultrasonography in predicting arthritis in auto-antibody positive arthralgia 2010 INTRODUCTION: Ultrasonography (US) has better sensitivity than clinical evaluation for the detection of synovitis in early rheumatoid arthritis (RA). Patients presenting with arthralgia and a positive anti-citrullinated protein antibodies (ACPA) and/or Rheumatoid Factor (IgM-RF) status are at risk for developing RA. In the present study, US utility and predictive properties in arthralgia patients at risk for the development of arthritis were studied. METHODS: 192 arthralgia patients with ACPA and/or IgM-RF were included. Absence of clinical arthritis was confirmed by two physicians. US was performed by one of two trained radiologists of any painful joint, and of adjacent and contralateral joints. Joint effusion, synovitis and power Doppler (PD) signal in the synovial membrane of the joints and tenosynovitis adjacent to the joint were evaluated and classified on a 4-grade semi-quantitative scale. Grade 2-3 joint effusion, synovitis, tenosynovitis and grade 1-3 Power Doppler signal were classified as abnormal. RESULTS: Forty-five patients (23%) developed arthritis after a mean of 11 months. Inter-observer reliability for synovitis and PD was moderate (kappa 0.46, and 0.56, respectively) and for joint effusion low (kappa 0.23). The prevalence of tenosynovitis was too low to calculate representative kappa values. At joint level, a significant association was found between US abnormalities and arthritis development in that joint for joint effusion, synovitis and PD. At patient level, a trend was seen towards more arthritis development in patients who had US abnormalities for joint effusion, synovitis, PD and tenosynovitis. CONCLUSIONS: US abnormalities were associated with arthritis development at joint level, although this association did not reach statistical significance at patient level. US could potentially be used as a diagnostic tool for subclinical arthritis in seropositive arthralgia patients. However, further research is necessary to improve test characteristics.
21124694 Antibodies to mutated citrullinated vimentin in rheumatoid arthritis: diagnostic value, as 2010 May 24 BACKGROUND: Early definitive diagnosis and effective treatment are mandatory in rheumatoid arthritis (RA) as it can halt the disease progression and subsequent joints destruction. OBJECTIVE: To investigate the diagnostic and prognostic value of anti-mutated citrullinated vimentin (anti-MCV) and its correlation with disease activity, peripheral and axial skeleton affection in RA patients. PATIENTS AND METHODS: A total of 123 patients with different rheumatic diseases were enrolled in a prospective-two year study at Ain Shams University hospital: 64 patients with RA and 59 patients with other rheumatic diseases as controls. RA patients were fulfilling the traditional and the new ACR/EULAR diagnostic criteria for RA. They have been followed up for two years. At baseline, all RA patients were subjected to: Clinical assessment of disease activity by taking full histories, general and local examination, measurement of 28 joint count of tender and swollen joints with calculation of disease activity score (DAS-28) for each patient. Complete blood count, erythrocytes sedimentation rate, C-reactive protein and rheumatoid factor titers were performed. Anti-MCV IgG immunoglobulins' assay was performed at the study endpoint by ELISA. RA patients were then classified into; anti-MCV positive and anti-MCV negative groups for statistical comparison. Plain X-ray was performed on the peripheral joints and scored by the Simple Erosion Narrowing score (SEN-score). Magnetic Resonance Imaging (MRI) scans were carried out to 22 RA patients on cervical and lumbosacral regions. RESULTS: Anti-MCV antibodies were found to be of high sensitivity (79.6%) and specificity (96.6%) in diagnosing RA. The area under the curve was 0.893 at 95% confidence interval (CI), confers an odds ratio of 23.5. Anti-MCV positive RA patients had significantly higher DAS-28 and SEN-scores than anti-MCV negative patients; who were found to have more benign disease with lower incidence of erosions (P < 0.05). MRI scans revealed that; 17/22 (77%) had cervical joints involvement while, 8 (36%) had lumbo-sacral joint lesions (P < 0.05), both were correlated significantly with aggressive peripheral joint disease. CONCLUSION: Anti-MCV antibodies are promising diagnostic and prognostic marker in RA, with high sensitivity and specificity. They may identify a subset of RA patients with aggressive early erosive disease. The axial skeleton-especially the cervical spine-could be affected in RA and this was correlated with aggressive peripheral joints' disease. MRI scanning is a sensitive method for detecting axial skeleton involvement in RA, in attempt for better disease control and outcomes.
20470954 Clinical manifestations and treatment of the pediatric rheumatoid patient. 2010 Apr The management goal of juvenile rheumatoid arthritis (JRA) is to achieve early diagnosis and treatment so that arthritis can be resolved at an early stage, which avoids long-term damage and provides a good outcome of the affected inflammatory joints. This article describes presentation, classification, evaluation, and treatment of JRA as it relates to the foot and ankle. Because the course of JRA is complex and the optimal management is highly variable in each patient, this article can only offer recommendations. Actual treatment should be individualized to meet the conditions of each patient.
21887156 Chronic graft-versus-host disease mimicking rapid progressive rheumatoid arthritis with at 2009 Chronic graft-versus-host disease (cGVHD) may mimic clinical and serological features of various autoimmune diseases. We present a case of a 23-year-old man who developed vitiligo, symmetric polyarthritis, high titre rheumatoid factor, antinuclear antibodies and anti-double stranded DNA antibodies after allogenic peripheral blood stem cell transplantation for severe aplastic anaemia. He was treated with low dose oral steroids, non-steroid anti-inflammatory drugs and azathioprine and clinical improvement of polyarthritis were observed initially. However, atlantoaxial subluxation (C1-C2) and rapid progression of symmetrical joint space narrowing in knees and wrists developed within 1 year. cGVHD mimicking rheumatoid arthritis with unusual presentations was observed in this patient.
19727722 Acute lymphoblastic leukemia masquerading as juvenile rheumatoid arthritis: diagnostic pit 2010 Mar Acute lymphoblastic leukemia (ALL) often presents with osteoarthritic manifestations which may lead to misdiagnosis with juvenile rheumatoid arthritis (JRA). This study was designed to identify ALL patients with initial diagnosis of JRA, compare their clinicolaboratory characteristics and outcome with other ALL patients treated at our center. Case records of 762 patients with ALL were analyzed. Information regarding the clinical-demographic profile, therapy and outcome were recorded. Of the children, 49 (6.4%) had initial presentation mimicking JRA. Asymmetric oligoarthritis was the most common pattern of joint involvement. Majority presented with fever, pallor, arthritis, night pain, and bone pain. None of the routine prognostic factors including age, gender, lymphadenopathy, hepatosplenomegaly, total leukocytes count (TLC), and platelet count were significantly associated with relapse/death. The mean symptom-presentation interval (SPI), hemoglobin was significantly higher whilst the TLC was significantly lower in these patients compared to other ALL patients. The 5 year overall-survival was better than other patients with ALL (p = 0.06, by logrank test). Significantly longer SPI in these patients underscores the need for prompt and early investigations to rule out ALL in patients of JRA with atypical features and pointers of ALL. Children with ALL-mimicking JRA may belong to a subgroup of ALL with a better prognosis.
22043258 Bilirubin as a Protective Factor for Rheumatoid Arthritis: An NHANES Study of 2003 - 2006 2010 Dec 11 BACKGROUND: Rheumatoid arthritis(RA) is a chronic inflammatory, autoimmune polyarthritis, with a prevalence estimated at one percent of the United States(US) population. Serum bilirubin, because of its antioxidant nature, has been conjectured to exert an anti-inflammatory biologic effect. The objective of this study is to discern whether higher serum Total Bilirubin(TBili) levels are protective against RA. METHODS: This is a secondary analysis of National Health and Nutrition Examination Survey (NHANES) data collected between 2003-2006. Study participants completed a comprehensive questionnaire regarding their health history, underwent a physical examination, and had body fluids collected for laboratory studies. In NHANES, to assess for the presence of RA, the following questions were asked: Doctor ever said you had arthritis?" If so, Which type of arthritis. Statistical analysis was performed, using SAS version 9.1, proc survey methods. Participant data were adjusted for demographic characteristics as well as risk factors for RA. RESULTS: NHANES 2003-2006 included 20,470 individuals, chosen as a representative sampling of the entire US population. Exclusion criteria included age less than twenty years or liver dysfunction, defined as history of abnormal liver function tests or liver disease. 8,147 subjects did not have any exclusion criteria and were included in the data analysis. RA is inversely related to the serum level of TBili with an odds ratio of 0.679 (95% CI 0.533-0.865) and remained significant even after adjusting for age, gender, race, education, and tobacco history, with an odds ratio 0.749 (95% CI 0.575 - 0.976). CONCLUSIONS: Our study supports the hypothesis that higher TBili levels are protective against RA. A plausible mechanism for this association would be that the anti-oxidant effects of TBili exert a physiologic anti-inflammatory effect, which provides protection against RA. This explanation is supported by prior studies which show that higher TBili levels are protective against stroke, atherosclerosis, and vasculitis. Further studies are needed to delineate the exact nature of the protective properties of TBili. KEYWORDS: Bilirubin; Rheumatoid arthritis; Antioxidant; Protective.
20018088 A pathway analysis applied to Genetic Analysis Workshop 16 genome-wide rheumatoid arthriti 2009 Dec 15 The identification of several hundred genomic regions affecting disease risk has proven the ability of genome-wide association studies have proven their ability to identify genetic contributors to disease. Currently, single-nucleotide polymorphism (SNP) association analysis is the most widely used method of genome-wide association data, but recent research shows that multi-marker tests of association may provide greater power, especially when more than one mutation is present within a gene and the mutations are in low linkage disequilibrium with each other. Here we use a multi-marker association test based on regression to SNPs located within known genes to obtain a gene-level score of association. We then perform pathway analysis using this score as a measure of gene importance. We use two tests of pathway enrichment - a binomial test and a random set method. By utilizing publicly available gene and pathway information, we identify B cell, cytokine and inflammation response, and antigen presentation pathways as being associated with rheumatoid arthritis. These results confirm known biological mechanisms for auto-immunity disorders, of which rheumatoid arthritis is one.
20018018 Incorporating multiple-marker information to detect risk loci for rheumatoid arthritis. 2009 Dec 15 In genome-wide association studies, new schemes are needed to incorporate multiple-locus information. In this article, we proposed a two-stage sliding-window approach to detect associations between a disease and multiple genetic polymorphisms. In the proposed approach, we measured the genetic association between a disease and a single-nucleotide polymorphism window by the newly developed likelihood ratio test-principal components statistic, and performed a sliding-window technique to detect disease susceptibility windows. We split the whole sample into two sub-samples, each of which contained a portion of cases and controls. In the first stage, we selected the top R windows by the statistics based on the first sub-sample, and in the second stage, we claimed significant windows by false-discovery rate correction on the p-values of the statistics based on the second sub-sample. By applying the new approach to the Genetic Analysis Workshop 16 Problem 1 data set, we detected 212 out of 531,601 windows to be responsible for rheumatoid arthritis. Except for chromosomes 4 and 18, each of the other 20 autosomes was found to harbor risk windows. Our results supported the findings of some rheumatoid arthritis susceptibility genes identified in the literature. In addition, we identified several new single-nucleotide polymorphism windows for follow-up studies.
19933744 Impact of T-cell costimulation modulation in patients with undifferentiated inflammatory a 2010 Mar BACKGROUND: Several agents provide treatment for established rheumatoid arthritis (RA), but a crucial therapeutic goal is to delay/prevent progression of undifferentiated arthritis (UA) or very early RA. OBJECTIVE: To determine the impact of T-cell costimulation modulation in patients with UA or very early RA. METHODS: In this double-blind, phase II, placebocontrolled, 2-year study, anti-cyclic citrullinated peptide (CCP)2-positive patients with UA (not fulfilling the ACR criteria for RA) and clinical synovitis of two or more joints were randomised to abatacept ( approximately 10 mg/kg) or placebo for 6 months; the study drug was then terminated. The primary end point was development of RA (by ACR criteria) at year 1. Patients were monitored by radiography, MRI, CCP2, rheumatoid factor and 28 joint count Disease Activity Score (DAS28) over 2 years. RESULTS: At year 1, 12/26 (46%) abatacept-treated versus 16/24 (67%) placebo-treated patients developed RA (difference (95% CI) -20.5% (-47.4% to 7.8%)). Adjusted mean changes from baseline to year 1 in Genant-modified Sharp radiographic scores for abatacepttreated versus placebo-treated patients, respectively, were 0 versus 1.1 for total score, and 0 versus 0.9 for erosion score. Mean changes from baseline to year 1 in MRI erosion, osteitis and synovitis scores were 0, 0.2 and 0.2, respectively, versus 5.0, 6.7 and 2.3 in the abatacept versus placebo groups. Safety was comparable between groups; serious adverse events occurred in one patient (3.6%) in each group. CONCLUSION: Abatacept delayed progression of UA/very early RA in some patients. An impact on radiographic and MRI inhibition was seen, which was maintained for 6 months after treatment stopped. This suggests that it is possible to alter the progression of RA by modulating T-cell responses at a very early stage of disease. Trial registration number NCT00124449.
19661537 Ultrasound, skin, and joints in psoriatic arthritis. 2009 Aug Over the last decade, ultrasound has been increasingly used in rheumatology for assessing soft tissue involvement in patients with chronic arthritis. In spite of the high number of studies supporting the role and the validity of ultrasound in the assessment of patients with rheumatoid arthritis, the potential of ultrasound imaging in patients with psoriatic arthritis still waits to be adequately investigated. Our report illustrates the most relevant sonographic findings obtainable with the latest generation ultrasound equipment in patients with psoriatic arthritis.
20018021 Genome-wide association analysis of rheumatoid arthritis data via haplotype sharing. 2009 Dec 15 We present computationally simple association tests based on haplotype sharing that can be easily applied to genome-wide association studies, while allowing use of fast (but not likelihood-based) haplotyping algorithms, and properly accounting for the uncertainty introduced by using inferred haplotypes. We also give haplotype sharing analyses that adjust for population stratification. We apply our methods to a genome-wide association study of rheumatoid arthritis available as Problem 1 of Genetic Analysis Workshop 16. In addition to the HLA region on chromosome 6, we find genome-wide significant signals at 7q33 and 13q31.3. These regions contain genes with interesting potential connections with rheumatoid arthritis and are not identified using single single-nucleotide polymorphism methods.
20018071 Allelic based gene-gene interactions in rheumatoid arthritis. 2009 Dec 15 The detection of gene-gene interaction is an important approach to understand the etiology of rheumatoid arthritis (RA). The goal of this study is to identify gene-gene interaction of SNPs at the allelic level contributing to RA using real data sets (Problem 1) of North American Rheumatoid Arthritis Consortium (NARAC) provided by Genetic Analysis Workshop 16 (GAW16). We applied our novel method that can detect the interaction by a definition of nonrandom association of alleles that occurs when the contribution to RA of a particular allele inherited in one gene depends on a particular allele inherited at other unlinked genes. Starting with 639 single-nucleotide polymorphisms (SNPs) from 26 candidate genes, we identified ten two-way interacting genes and one case of three-way interacting genes. SNP rs2476601 on PTPN22 interacts with rs2306772 on SLC22A4, which interacts with rs881372 on TRAF1 and rs2900180 on C5, respectively. SNP rs2900180 on C5 interacts with rs2242720 on RUNX1, which interacts with rs881375 on TRAF1. Furthermore, rs2476601 on PTPN22 also interacts with three SNPs (rs2905325, rs1476482, and rs2106549) in linkage disequilibrium (LD) on IL6. The other three SNPs (rs2961280, rs2961283, and rs2905308) in LD on IL6 interact with two SNPs (rs477515 and rs2516049) on HLA-DRB1. SNPs rs660895 and rs532098 on HLA-DRB1 interact with rs2834779 and four SNPs in LD on RUNX1. Three-way interacting genes of rs10229203 on IL6, rs4816502 on RUNX1, and rs10818500 on C5 were also detected.
19922673 The incidence of juvenile rheumatoid arthritis in Quebec: a population data-based study. 2009 Nov 19 OBJECTIVE: To determine the population incidence of juvenile rheumatoid arthritis (JRA) in Quebec. METHODS: We obtained data from Quebec's physician claims database. Incident cases were defined as having a visit for JRA in 2000, no visit in the previous 3 years, a confirmed diagnosis by an arthritis specialist, or having >/= 2 visits to any physician for JRA, >/= 2 months apart but within 2 years. RESULTS: Cumulative incidence of JRA was 17.8/100,000. Mean age at diagnosis was 9.8 +/- 4.6 years, 68% were female and more persons were diagnosed in winter. Subjects had a median of 10 medical visits over the first year. CONCLUSION: Our population based incidence estimate was similar to others. Children and adolescents with JRA are heavy users of medical care. Additional study of environmental or climate- related triggers may be warranted.
19584730 Emerging new pathways of pathogenesis and targets for treatment in systemic lupus erythema 2009 Sep PURPOSE OF REVIEW: Systemic lupus erythematosus (SLE) and Sjogren's syndrome are chronic inflammatory diseases characterized by the dysfunction of T cells, B cells, and dendritic cells and the production of antinuclear autoantibodies. Here, we evaluate newly discovered molecular and cellular targets for the treatment of SLE and Sjogren's syndrome. RECENT FINDINGS: The mammalian target of rapamycin in T and B cells has been successfully targeted for treatment of SLE with rapamycin or sirolimus both in patients and animal models. Inhibition of oxidative stress, nitric oxide production, interferon alpha, toll-like receptors 7 and 9, histone deacetylase, spleen tyrosine kinase, proteasome function, lysosome function, endosome recycling, and the nuclear factor kappa B pathway showed efficacy in animal models of lupus. B-cell depletion and blockade of anti-DNA antibodies and T-B cell interaction have shown success in animal models, whereas human studies have so far failed to accomplish clinical endpoints, possibly due to inadequacies in study design. SUMMARY: Discovery of novel genes and signaling pathways in lupus pathogenesis offers novel biomarker-targeted approaches for treatment of SLE and Sjogren's syndrome.
20664046 Treatment of primary Sjögren syndrome: a systematic review. 2010 Jul 28 CONTEXT: A variety of topical and systemic drugs are available to treat primary Sjögren syndrome, although no evidence-based therapeutic guidelines are currently available. OBJECTIVE: To summarize evidence on primary Sjögren syndrome drug therapy from randomized controlled trials. DATA SOURCES: We searched MEDLINE and EMBASE for articles on drug therapy for primary Sjögren syndrome published between January 1, 1986, and April 30, 2010. STUDY SELECTION: Controlled trials of topical and systemic drugs including adult patients with primary Sjögren syndrome were selected as the primary information source. RESULTS: The search strategy yielded 37 trials. A placebo-controlled trial found significant improvement in the Schirmer and corneal staining scores, blurred vision, and artificial tear use in patients treated with topical ocular 0.05% cyclosporine. Three placebo-controlled trials found that pilocarpine was associated with improvements in dry mouth (61%-70% vs 24%-31% in the placebo group) and dry eye (42%-53% vs 26%). Two placebo-controlled trials found that cevimeline was associated with improvement in dry mouth (66%-76% vs 35%-37% in the placebo group) and dry eye (39%-72% vs 24%-30%). Small trials (<20 patients) found no significant improvement in sicca outcomes for oral prednisone or hydroxychloroquine and limited benefits for immunosuppressive agents (azathioprine and cyclosporine). A large trial found limited benefits for oral interferon alfa-2a. Two placebo-controlled trials of infliximab and etanercept did not achieve the primary outcome (a composite visual analog scale measuring joint pain, fatigue, and dryness); neither did 2 small trials (<30 patients) testing rituximab, although significant results were observed in some secondary outcomes and improvement compared with baseline. CONCLUSIONS: In primary Sjögren syndrome, evidence from controlled trials suggests benefits for pilocarpine and cevimeline for sicca features and topical cyclosporine for moderate or severe dry eye. Anti-tumor necrosis factor agents have not shown clinical efficacy, and larger controlled trials are needed to establish the efficacy of rituximab.
19777237 Clinical significance of ¹⁸F-fluoro-dexoxyglucose positron emission tomography in patie 2010 Nov Adult-onset Still's disease (AOSD) is a multi-systemic inflammatory disease that usually presents with high fever and variable systemic features. The pathogenesis and etiology of AOSD have not yet been clearly determined. In addition, there is no diagnostic test for AOSD. Even though some useful diagnostic criteria or laboratory findings, such as serum ferritin levels, have been proposed, useful imaging studies for the diagnosis or follow-up of AOSD have not been developed. We performed (18)F-fluoro-dexoxyglucose positron emission tomography ((18)F-FDG PET) on two AOSD patients who presented with a fever of unknown origin. In these patients, we initially identified abnormally increased FDG uptake in multiple lymph nodes, the spleen, or bone marrow. We then identified significantly decreased uptake during a follow-up study. On the basis of these cases, we suggest that (18)F-FDG PET may have the potential in the diagnosis of AOSD, as well as monitor clinical changes in the disease. More further investigation of (18)F-FDG PET in AOSD is needed in larger population.
20671520 Pathogenesis of Sjögren's syndrome and therapeutic consequences. 2010 Sep PURPOSE OF REVIEW: To summarize recent findings on new pathogenic mechanisms of interaction between genetic and environmental factors and between innate and adaptive immunity in primary Sjögren's syndrome and to reconcile pathogenesis and treatment by focusing on the crucial pathogenic steps that could be targeted by emerging therapies. RECENT FINDINGS: Regarding genetic predisposition, the functional relevance of IRF5 and STAT4 gene polymorphisms in the activation of type I interferon pathways has been demonstrated. It has also been shown that the isolated stimulation of innate immunity in mice can result in dryness, which precedes lymphocytic infiltrates in salivary glands. In animal models, possible environmental triggers of the disease, such as oestrogen deficiency and/or infection by Epstein-Barr virus, can lead to innate immune followed by autoimmune epithelitis. The IFN-BAFF-B lymphocyte pathogenic axis is, therefore, targeted by numerous drugs currently in evaluation. The development of consensus disease activity scores and patient-related outcomes might help to initiate new controlled trials. The first positive randomized controlled trial with rituximab has been recently published. SUMMARY: Hopefully, persistent and joint efforts by many teams to improve the knowledge on the pathogenesis of the disease may allow identification of new therapeutic targets in Sjögren's syndrome.
20553243 Sjögren's syndrome: a review of aetiology, pathogenesis, diagnosis and management. 2010 Jun Sjögren's syndrome is a chronic autoimmune disease that affects many individuals within the community. Despite this, its exact aetiology and pathogenesis is still unclear. Sjögren's syndrome affects many organ systems in the body. However, for dental practitioners it is important to recognize the many oral and dental manifestations that are associated with the syndrome. In addition to these oral manifestations, this review will discuss the systemic manifestations of Sjögren's syndrome as well as the current understanding of factors that have a role in its aetiology and pathogenesis. Furthermore, this review will highlight the difficulties and complexities that are inherent in the diagnosis of Sjögren's syndrome and the important role that dental practitioners can play in the management of its oral manifestations. The effective management of oral manifestations and minimization of oral disease in patients with Sjögren's syndrome can result in improved quality of life for these patients.