Classification Criteria For Sjögren's Syndrome Research Articles

Kidney manifestations of pediatric Sjögren's syndrome.

Approximately 1% of all patients with Sjögren's syndrome (SS) are children. Unlike the adult form, in which sicca syndrome is the main presentation, in children, the most common clinical finding is recurrent enlargement of the salivary glands. In pediatric SS, extraglandular manifestations represent a significant feature and, among these, kidney manifestations are relevant. Kidney involvement is observed in 5-20.5% of children with SS, most frequently tubulointerstitial nephritis. This injury can lead to serious phenotypes, including distal kidney tubular acidosis with the development of severe hypokalemia, which can lead to ECG abnormalities, weakness, and hypokalemic periodic paralysis. Kidney implications in pediatric SS also include nephrolithiasis, nephrocalcinosis, and various types of glomerular damage, which often require immunosuppressive therapies. Laboratory findings are usually comparable to adults, including hyperglobulinemia and high rates of antinuclear antibodies (ANA, 63.6-96.2%), and anti-Ro/SSA (36.4-84.6%). The current classification criteria for SS are inaccurate for the pediatric population, and more specific criteria are needed to improve the diagnostic rate. Due to the rarity of the disease, strong recommendations for treatment are lacking, and several therapeutic strategies have been reported, mostly based on glucocorticoids and disease-modifying antirheumatic drugs, with different outcomes. The aim of this paper is to provide an overview of the kidney implications of pediatric SS based on the latest evidence of the medical literature.

Mortality risk factors in primary Sjögren syndrome: areal-world, retrospective, cohort study.

What baseline predictors would be involved in mortality in people with primary Sjögren syndrome (SjS) remains uncertain. This study aimed to investigate the baseline characteristics collected at the time of diagnosis of SjS associated with mortality and to identify mortality risk factors for all-cause death and deaths related to systemic SjS activity measured by the ESSDAI score. In this international, real-world, retrospective, cohort study, we retrospectively collected data from 27 countries on mortality and causes of death from the Big Data Sjögren Registry. Inclusion criteria consisted of fulfilling 2002/2016 SjS classification criteria, and exclusion criteria included chronic HCV/HIV infections and associated systemic autoimmune diseases. A statistical approach based on a directed acyclic graph was used, with all-cause and Sjögren-related mortality as primary endpoints. The key determinants that defined the disease phenotype at diagnosis (glandular, systemic, and immunological) were analysed as independent variables. Between January 1st, 2014 and December 31, 2023, data from 11,372 patients with primary SjS (93.5% women, 78.4% classified as White, mean age at diagnosis of 51.1 years) included in the Registry were analysed. 876 (7.7%) deaths were recorded after a mean follow-up of 8.6 years (SD 7.12). Univariate analysis of prognostic factors for all-cause death identified eight Sjögren-related variables (ocular and oral tests, salivary biopsy, ESSDAI, ANA, anti-Ro, anti-La, and cryoglobulins). The multivariate CPH model adjusted for these variables and the epidemiological features showed that DAS-ESSDAI (high vs no high: HR=1.68; 95% CI, 1.27-2.22) and cryoglobulins (positive vs negative: HR=1.72; 95% CI, 1.22-2.42) were independent predictors of all-cause death. Of the 640 deaths with available information detailing the specific cause of death, 14% were due to systemic SjS. Univariate analysis of prognostic factors for Sjögren-cause death identified five Sjögren-related variables (oral tests, clinESSDAI, DAS-ESSDAI, ANA, and cryoglobulins). The multivariate competing risks CPH model adjusted for these variables and the epidemiological features showed that oral tests (abnormal vs normal results: HR=1.38; 95% CI, 1.01-1.87), DAS-ESSDAI (high vs no high: HR=1.55; 95% CI, 1.22-1.96) and cryoglobulins (positive vs negative: HR=1.52; 95% CI, 1.16-2) were independent predictors of SjS-related death. The key mortality risk factors at the time of SjS diagnosis were positive cryoglobulins and a high systemic activity scored using the ESSDAI, conferring a 2-times increased risk of all-cause and SjS-related death. ESSDAI measurement and cryoglobulin testing should be considered mandatory when an individual is diagnosed with SjS. Novartis.

Added value of lymphocyte subpopulations in the classification of Sjögren's syndrome

Sjögren's Syndrome (SjS) is a chronic systemic immune-mediated inflammatory disease characterized by lymphocytic infiltration and consequent lesion of exocrine glands. SjS diagnosis and classification remains a challenge, especially at SjS onset, when patients may have milder phenotypes of the disease or uncommon presentations. New biomarkers are needed for the classification of SjS, thus, we aimed to evaluate the added-value of lymphocyte subpopulations in discriminating SjS and non-Sjögren Sicca patients. Lymphocyte subsets from 62 SjS and 63 Sicca patients were characterized by flow cytometry. The 2002 AECG and the 2016 ACR/EULAR SjS classification criteria were compared with clinical diagnosis. The added discriminative ability of joining lymphocytic populations to classification criteria was assessed by the area under the Receiver-Operating-Characteristic Curve (AUC). Considering clinical diagnosis as the gold-standard, we obtained an AUC = 0.952 (95% CI: 0.916–0.989) for AECG and an AUC = 0.921 (95% CI: 0.875–0.966) for ACR/EULAR criteria. Adding Tfh and Bm1 subsets to AECG criteria, performance increased, attaining an AUC = 0.985 (95% CI: 0.968–1.000) (p = 0.021). Th1/Breg-like CD24hiCD27+ and switched-memory B-cells maximized the AUC of ACR/EULAR criteria to 0.953 (95% CI: 0.916–0.990) (p = 0.043). Our exploratory study supports the potential use of lymphocyte subpopulations, such as unswitched memory B cells, to improve the performance of classification criteria, since their discriminative ability increases when specific subsets are added to the criteria.

Ultrasound scoring systems affect the distribution of sialadenitis scores in Sjögren's syndrome: an inter-system reproducibility study.

Salivary gland ultrasonography (SGUS) is commonly employed in the diagnosis and follow-up of patients with Sjögren's syndrome (SS) and multiple scoring systems have been developed to quantify the grade of sialadenitis of major salivary glands (SG). Their diagnostic performance seems overall comparable, however, the parameters evaluated by the various systems are different. The objective of this study was to compare how four different scoring systems affect the distribution of sialadenitis grades. One hundred and three SGUS images from 26 SS patients were blindly scored by two investigators according to the De Vita, Salaffi, Milic and OMERACT scoring systems in independent sessions. The distribution of SGUS images according to De Vita, Salaffi, Milic and OMERACT systems was significantly different. At post-hoc analysis, Milic system performed differently compared to the De Vita (p<0.0001), OMERACT (p<0.0001) and Salaffi (p<0.0001) systems, showing a relative overestimation of sialadenitis grade. Milic scoring system showed to relatively overestimate the grade of sialadenitis compared to De Vita, Salaffi and OMERACT systems. Although all scoring systems seem to be comparable in terms of diagnostic accuracy, in the prospect of selecting one system to be potentially included in future versions of SS classification criteria, it is important to compare their ability to classify SGUS images among the various degrees of sialadenitis.

Diagnostic Performances of Ultrasound Evaluation of Major Salivary Glands According to the 2019 Outcome Measures in Rheumatology Ultrasound Scoring System.

To evaluate the diagnostic performance of ultrasound examination of the salivary glands (US-SG) according to the 2019 Outcome Measures in Rheumatology (OMERACT) US scoring system for Sjögren's syndrome (SS). The present work was a retrospective study based on a multicentric cohort with SS/sicca syndrome. The American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) 2016 classification criteria for SS (a score of ≥4 without ocular staining score), the American-European Consensus Group (AECG) 2002 classification criteria, and clinician experts were considered as reference standards for diagnosis of SS. An OMERACT score of ≥2 according to 2 independent readers defined the diagnosis of SS based on US-SG assessment. Diagnostic performances and interobserver reproducibility of US-SG were assessed. Forty-two patients fulfilling the ACR/EULAR 2016 criteria for SS were compared to 30 control subjects with sicca syndrome. Twenty-five patients were diagnosed as having SS according to US-SG evaluation, and they were more frequently observed in the SS group (52.5%) than in the control group (10.0%) (P < 0.001). US-SG showed an area under the curve (AUC) of 0.751 (95% confidence interval [95% CI] 0.621, 0.882) for the diagnosis of SS (ACR/EULAR 2016 classification). The inclusion of US-SG in the ACR/EULAR 2016 classification improved sensitivity (91.5% versus 89.4%) with limited decrease of specificity (96.0% versus 100%) and with an AUC of 0.975 (95% CI 0.945, 1.00). Similar results were observed when US-SG was included in the AECG 2002 classification criteria. Interobserver reproducibility of a score of ≥2 according to the 2019 OMERACT US scoring system for SS diagnosis was good (κ=0.73 [95% CI 0.64, 0.81]). Histologic lymphocyte infiltration of the minor salivary glands was associated with the OMERACT grading of US-SG. The present study confirms the good specificity of the 2019 OMERACT US classification measures of US-SG for the diagnosis of SS and its feasibility in daily practice.

Características operativas de la ecografía en el diagnóstico del Síndrome de Sjögren

Background and objectiveTo determine the operational characteristics of salivary gland ultrasound (SGU) in the diagnosis of Sjögren's syndrome (SS) in a population of colombian patients with dry symptoms. Materials and methodsStudy of diagnostic tests in patients with dry symptoms who consecutively attended the rheumatology consultation (2018-2020). Sociodemographic and clinical data were obtained through a survey, paraclinical and ophthalmological tests, minor salivary gland biopsy, unstimulated salivary flow and SGU (score 0-6 based on De Vita) were done. Sensitivity, specificity, positive (PPV) and negative (NPV) predictive values (Stata 15®) were calculated. The receiver operating characteristics (ROC) curve was developed. Results102 patients were included (34 SS and 68 non-SS), mean age 55.69 (± 11.93) years, 94% women. Positive ultrasound (score of 2 or more) was more frequent in the SS group, (70.6% vs. 22.1%, P<.0001). The sensitivity was the same for grade 2 and 3 (70.59%), with a higher specificity (89.71%) for grade 3 (PPV 77.42% NPV 85.92). The ROC curve from the sum of the glands by means of ultrasound was better than those of the independent glands. The ROC curve of the ultrasound presented a greater area under the curve (0.72 [0.61-0.82]) than that of the histological analysis (focus score) (0.68 [0.59-0.78]), P=.0252. ConclusionSalivary gland ultrasound is a useful and reliable method for the classification of SS. Its use could be considered in the future within the SS classification criteria.

Antibodies to Both Ro52 and Ro60 for Identifying Sjögren's Syndrome Patients Best Suited for Clinical Trials of Disease-Modifying Therapies.

To assess anti-Ro52 and anti-Ro60 serologic profiles as markers of clinically relevant phenotypic subsets of patients with Sjögren's syndrome (SS). From a cohort of 839 consecutive patients with suspected or established SS seen in our multidisciplinary SS center, we compared the association of key phenotypic features in 390 patients who fulfilled SS classification criteria and in the parent cohort, stratifed by the presence of both anti-Ro60 and anti-Ro52, anti-Ro60 alone, and anti-Ro52 alone. The SS cohort included 227 patients (58%) with both anti-Ro60 and anti-Ro52, 65 (17%) with anti-Ro60 alone, 58 (15%) with anti-Ro52 alone, and 40 (10%) with neither antibody. Those with both anti-Ro60 and anti-Ro52 had a significantly increased prevalence of abnormal ocular surface staining, focal lymphocytic sialadenitis with focus score ≥1, antinuclear antibody ≥1:320, anti-SSB/La, rheumatoid factor, and IgG ≥15.6 gm/liter (P < 0.0016 for all). The groups with isolated anti-Ro52 and anti-Ro60 were equivalent to each other in their phenotypic associations, except for rheumatoid factor, which was higher in the anti-Ro52 alone group. The associations of these Ro antibody serologic profiles were similar in the parent cohort, except for additional associations with salivary gland enlargement and parotid gland ultrasound score. SS patients with both anti-Ro60 and anti-Ro52 antibodies are distinguished by a higher prevalence of markers of B-cell hyperactivity and glandular inflammation. Antibody reactivity to both Ro60 and Ro52 may thus serve as an important inclusion criterion for SS patients in clinical trials where the therapeutic agent targets pathways mediating these pathogenic abnormalities.

Frecuencia del síndrome de Sjögren en pacientes con síntomas secos mediante dos métodos histopatológicos

Introduction and objectiveThe criteria for Sjögren's syndrome (SS) classification (ACR/EULAR 2016), include labial salivary gland (LSG) biopsy using the focus score (FS). But, in some cases it continues to be based on Chisholm and Mason (CM). Our objective was to evaluate the frequency of SS in patients with dry symptoms using FS and CM and to evaluate the degree of inter and intra-observer concordance of the histopathological reading by both methods. Materials and methodCross-sectional study design. All patients with dry symptoms and studies to perform the SS classification criteria (2016) were included. The samples were independently evaluated by two readers. Descriptive statistics was used for the calculation of SS frequency. Agreement (Cohen's kappa coefficient) was analysed (STATA) for each test (CM and FS). Ethics approval was obtained. Results92 patients were included. According to the 2016 criteria, SS was reported in 26.1% patients in whom FS was used and in 34.8% patients in whom CM was used. The degree of intra-observer concordance for the diagnosis of FLS was perfect and moderate-high for observers. Inter-observer agreement was substantial, with kappa values of .77 FS and .75 CM. ConclusionsFS method is a more detailed and specific score that facilitates correct classification. The use of CM as a histopathological classificatory method for SS includes more patients when compared with FS. These results are of relevance to standardise the reading of LSG biopsy in specialized services attending SS patients.

Scleroderma-specific autoantibodies: Should they be included in the diagnostic work-up for Sjögren's syndrome?

ObjectivesSicca complaints are a frequent reason for rheumatologic consultation. Testing for specific antibodies against Ro/SSA and La/SSB antigens and minor salivary gland (MSG) biopsy are among the main tools implemented in the diagnostic work-up. Anticentromere antibodies and sicca manifestations are frequently detected in Sjögren's syndrome (SS) and systemic sclerosis (SSc), respectively. Herein, we aimed to determine the frequency and clinical associations of a wide spectrum of scleroderma (SSc)-specific autoantibodies in consecutive patients referred for evaluation of possible SS. MethodsDemographic, clinico-pathological, and laboratory data were recorded in 216 consecutive patients with sicca complaints. All study participants were tested for SSc-specific autoantibodies (against CENP, PM/Scl, Scl-70, Ku, NOR90, RP11, RP155, fibrillarin, PDGFR, and Th/To) using a commercially available immunoblot kit. According to band intensity, the identified autoantibodies were further classified in those with strong and medium titers. ResultsSSc-specific autoantibodies were detected in 41.7% (90/216) patients evaluated (19% at strong, 22.7% at medium titers) without significant differences between anti-Ro/SSA positive and negative groups. At strong titers was significantly higher in patients with MSG biopsies fulfilling SS histopathological criteria (30% vs 12.5%, p = 0.009). This association remained significant after adjustment for antibodies against Ro/SSA and La/SSB autoantigens [OR 95% (CI): 4.1 (1.5–10.6)]. ConclusionSSc-specific autoantibodies are frequently detected among patients presenting with sicca complaints and at strong but not medium titers are independently associated with MSG biopsy positivity. Taken together, these data imply a useful role of SSc antibody testing in the diagnostic work-up and possibly in the classification criteria for SS.

Value of labial salivary gland histopathology for diagnosis of Sjögren's syndrome in patients with anti‐centromere antibody positivity

Although anti-centromere antibody (ACA)+ Sjögren's syndrome (SS) is considered a subtype of SS, it was not included in the recent American College of Rheumatology/ European League Against Rheumatism (ACR/EULAR) SS classification criteria. ACA+patients without anti-SS-A/Ro antibodies require salivary gland histopathology to fulfill the ACR/EULAR criteria for diagnosis of SS. We reviewed salivary gland histology among ACA+patients referred for the diagnosis of SS using the ACR/EULAR and Japanese criteria which does not require biopsy. Data from 147 ACA+patients with dry eyes and/or mouth who visited our department were retrospectively analyzed. Clinical, immunological, and histological data were collected and statistically analyzed. Sixty-five patients (44%) had undergone labial salivary gland biopsy. The frequency of dry mouth was higher in ACA+patients who had undergone labial salivary gland biopsy than in those who had not (P=.046), while there were no differences in biopsy rates between patients with and without sclerodactyly (P=.51). According to the current ACR/EULAR classification criteria, Greenspan grade of 3 or 4 for labial salivary gland histopathology is required in patients without anti-SS-A/Ro antibody for the diagnosis of SS. Four patients with Greenspan grades <3 and anti-SS-A/Ro antibody met the criteria for SS. In 54 patients in which the ACR/EULAR criteria were met, 53 patients were diagnosed with SS using the Japanese criteria. In ACA+/anti-SS-A/Ro- antibody patients, agreement between ACR/EULAR and Japanese criteria sets was excellent. For easily classifying ACA+patients as SS cases, salivary gland biopsy should be performed in ACA+patients with dry symptoms to identify ACA+ patients.

American Indians Have a Higher Risk of Sjögren's Syndrome and More Disease Activity Than European Americans and African Americans

To describe the clinical and serologic manifestations of Sjögren's syndrome (SS) in ethnic groups of the US. This was a cross-sectional study of 648 patients with primary SS: 20 African American (AA), 164 American Indian (AI), 426 European American (EA), and 38 patients of other races evaluated in a multidisciplinary Sjögren's International Collaborative Clinical Alliance research clinic. AA subjects comprised 3.1% of the SS cohort, much lower than the percentage of AA in the state of Oklahoma (P = 3.01 × E - 05), the US (P = 2.24E - 13), or a systemic lupus erythematosus (SLE) cohort at the same institution (P = 4.26 × 10E - 27). In contrast, the percentage of AI in the SS cohort (25.3%) was much higher than expected (P = 3.17E - 09 versus SLE cohort, P = 6.36 - 26 versus Oklahoma, and P = 8.14E - 96 versus US population). The SS classification criteria were similar between AA and EA, but subjects of AI ancestry had lower rates of abnormal tear and salivary flow, as well as anti-Ro/SSA and anti-La/SSB antibodies. Paradoxically, AI had higher levels of disease activity (mean ± SD European League Against Rheumatism Sjögren's Syndrome Disease Activity Index score 3.77 ± 4.78) in comparison to EA (2.90 ± 4.12; P = 0.011) and more extraglandular manifestations affecting mainly the articular and glandular domains. Meanwhile, AA patients were characterized by higher rates of hypergammaglobulinemia (odds ratio [OR] 1.39 [95% confidence interval (95% CI) 1.39-8.65]; P = 0.01), elevated erythrocyte sedimentation rate (ESR) (OR 3.95 [95% CI 1.46-9.95]; P = 0.009), and parotid enlargement (OR 4.40 [95% CI 1.49-13.07]; P = 0.02). AI are affected at high rates with SS but present with few classical features, potentially preventing timely diagnosis. In contrast to SLE, SS is infrequent and not more severe among AA, but the triad of hypergammaglobulinemia, increased ESR, and parotid enlargement warrants extra vigilance for lymphomagenesis.

A Tutorial on Saliva's Role in Swallowing With a Focus on Sjögren's Syndrome.

Purpose Saliva is integral to swallowing and necessary for oral health. Understanding saliva's origin and properties is important for swallowing assessment and management. Diseases such as Sjögren's syndrome (SS) can affect saliva negatively, often contributing to dysphagia. Our objectives are to (a) highlight saliva's fundamental role in swallowing, (b) provide a bibliometric overview of literature pertaining to SS pathophysiology and effects on saliva, (c) explore implications of salivary changes on swallowing and quality of life in SS and other populations, and (d) provide suggestions for systematic saliva assessment in practice. Method This tutorial reviews saliva production, composition, and involvement in swallowing within health and disease. Using rapid review methodology, we outline the effect of SS on saliva and describe SS etiology, diagnosis, and treatment. We discuss formal saliva assessments and a multidisciplinary approach. Results Saliva plays a vital role in swallowing, particularly lubrication, bolus formation, and oral health. SS affects the salivary glands altering salivary flow rate and composition. We identified 55 studies (N) measuring salivary changes, grouping them according to four strata demarcated by SS classification criteria updates. For some, xerostomia, dysphagia, and reduced life quality result. Formal saliva assessments include the Clinical Oral Dryness Score, Xerostomia Inventory, and Secretion Rating Scale. Multidisciplinary care is optimal for patients with salivary changes. Conclusion Understanding salivary changes in disease may enhance understanding of swallowing and inform dysphagia practice. Expanding swallowing assessments with formal saliva evaluations, and patient perspectives thereof, may aid in developing bespoke treatments, ultimately improving outcomes and quality of life. Supplemental Material https://doi.org/10.23641/asha.12456449.

The Prevalence of Sjögren's Syndrome in Rheumatoid Arthritis Patients and Their Clinical Features.

BackgroundTo estimate the prevalence of Sjögren's syndrome (SS) in patients with rheumatoid arthritis (RA) and to compare the clinical features of RA patients with and without SS.MethodsWe conducted a retrospective study of RA patients who visited a rheumatology clinic in a tertiary referral hospital in Korea between May 20 and July 22, 2016. All patients fulfilled the classification criteria for RA, and the diagnosis of SS was made clinically by rheumatologists and according to the 2002 American-European Consensus Group (AECG), 2012 American College of Rheumatology (ACR), and 2016 ACR/European League Against Rheumatism (EULAR) classification criteria. The prevalence was estimated as the number of SS patients within the total number of RA patients. The disease activity and treatment pattern of RA were compared between patients with and without SS.ResultsAmong 827 RA patients, 72 patients (8.7%) were diagnosed with SS by a rheumatologist, though only 60 patients (7.3%) satisfied the 2002 AECG classification criteria for SS. Fifty-two patients (6.3%) and 56 patients (6.8%) fulfilled the 2012 ACR and 2016 ACR/EULAR classification criteria, respectively. The prevalence of SS in RA patients was 10.5%, 17.0%, and 67.6% in rheumatoid factor, antinuclear antibody (≥ 1:80), and anti-Ro antibody positive patients, respectively.ConclusionThe prevalence of SS among RA patients was 8.7% according to rheumatologists' diagnosis. The presence of SS did not affect the treatment patterns of RA patients. However, the autoantibody profiles and demographics of RA patients with SS differed from those of patients without SS.

Prevalence of Novel Candidate Sjogren Syndrome Autoantibodies in the Dry Eye Assessment and Management (DREAM) Study.

To evaluate the prevalence of novel candidate Sjogren syndrome (SS) autoantibodies [salivary protein-1 (SP-1), parotid secretory protein, carbonic anhydrase 6] in the DRy Eye Assessment and Management (DREAM) cohort, a study evaluating the effectiveness of omega-3 fatty acid supplements for the treatment of dry eye. Participants underwent ocular surface examinations and serological testing for traditional and novel SS autoantibodies. Dry eye assessment and management participants were categorized into the following 3 groups: 1) no history of SS or other autoimmune diseases and negative traditional SS autoantibodies (n = 352); 2) no history of SS but a history of other autoimmune diseases (n = 66); and 3) those who met the 2012 American College of Rheumatology SS classification criteria (n = 52). Eleven percent had a history of SS, and 6% of those without a history of SS most likely had undiagnosed SS. The SS group had a higher prevalence of SP-1 autoantibodies than the group without SS or other autoimmune diseases (33% vs. 19%; P = 0.02) but had no difference in carbonic anhydrase 6 (P = 0.31) or parotid secretory protein autoantibodies (P = 0.33). Participants who were positive for the traditional autoantibodies alone or positive for both traditional and novel autoantibodies had the highest scores for corneal (P = 0.002) and conjunctival staining (P < 0.001). Data from this multicenter, prospective study demonstrated that one of the novel candidate autoantibodies, SP-1, is associated with underlying SS and that novel autoantibodies may be associated with worse ocular surface disease. Future longitudinal studies are needed to evaluate their utility in screening patients with dry eye for SS.

Comparison of performance of the 2016 ACR-EULAR classification criteria for primary Sjögren's syndrome with other sets of criteria in Japanese patients

ObjectivesTo compare the performance of the new 2016 American College of Rheumatology (ACR)-European League Against Rheumatism (EULAR) classification criteria for primary Sjögren's syndrome (SS) with 1999 revised Japanese Ministry of...

The Frequency of Primary Sjogren's Syndrome and Fibromyalgia in Irritable Bowel Syndrome.

Objective:To determine the frequency of sicca complex, Sjogren’s Syndrome (SS) and Fibromyalgia (FM) in patients with Irritable Bowel Syndrome (IBS).Methods:Seventy seven IBS patients who fulfilled the Rome-III criteria were included in the study. All patients were assessed for FM according to the American College of Rheumatology (ACR) 2010 criteria. After examination for objective evidence of sicca complex by Schirmer test, TBUT and Ocular Staining Score (OSS), serological tests were performed. And the diagnosis of SS was made according to the American College of Rheumatology (ACR) classification criteria for SS – 2012.Results:Thirteen (16.9%) of IBS patients had FM. Dry eye was detected in 20(26.0%), 7(9.1%) and 29(37.7%) patients by OSS, Schirmer test and TBUT, respectively. Of 77 patients with IBS, the diagnosis of SS was established in two patients (2.6%).Conclusion:The frequency of Sjogren’s Syndrome among patients with IBS is relatively higher than the general population. All IBS patients should be questioned for dryness of the mouth and eyes, and if necessary, should be evaluated for SS.

Significance and Implications of Patient-reported Xerostomia in Sjögren's Syndrome: Findings From the National Institutes of Health Cohort

BackgroundXerostomia is a chief complaint of patients with Sjögren's syndrome (SS). However, newer proposals for SS classification remove xerostomia and hyposalivation from the criteria list. Given these developments and the importance of patient-centered research outcomes, we sought to evaluate the utility of patient-reported xerostomia with implications for classification criteria, and clinical trials targeting SS treatment modalities. MethodsA nested case-control study was designed within The National Institute of Dental and Craniofacial Research/National Institutes of Health (NIDCR/NIH) SS Cohort - one of the largest SS cohorts in the US. Clinical characteristics of those with and without xerostomia in SS and other salivary gland dysfunctions were compared. Several analytical methods were employed, including multivariable logistic regression modeling. FindingsThe NIDCR/NIH Sjögren's Syndrome Clinic has an open cohort with ongoing enrollment since 1984. This open cohort comprised of 2046 participants by August 27, 2015. Baseline data of 701 SS, 355 Sicca, and 247 ISS participants within the source cohort were analyzed. Xerostomia was highest among SS participants (87.4%, 95% CI: 84.8%–89.8%) compared to Sicca (72.4%, 95% CI: 67.4%–77.0%, p<0.001) and ISS groups (38.1%, 95% CI: 32.0%–44.4%, p<0.001). Those with xerostomia were more likely to have SS than Sicca/ISS (OR=4.98, 95% CI: 3.78–6.56). The ability of xerostomia to screen for SS among those with salivary gland dysfunction was higher than screening for Sicca/ISS. Screening diagnostics of xerostomia were of greater utility compared to hyposalivation. After adjusting for confounding in multivariable modeling, SS participants with xerostomia were more likely to be White (Black/African Americans (OR: 0.40, 95% CI: 0.23–0.68, p-value=0.001) and Asians (OR: 0.49, 95% CI: 0.25–0.96, p-value=0.038) were less likely to have xerostomia compared to Whites), have dry eye symptoms for >3months (OR: 5.80, 95% CI: 3.62–9.28, p-value <0.001), a lower Van Bijsterveld score (OR: 0.55, 95%CI: 0.34–0.90, p-value=0.017), a lower stimulated salivary flow rate (OR: 1.67, 95% CI: 1.06–2.65, p-value=0.028), a focus score of >2 (OR: 1.92, 95% CI: 1.20–3.09, p-value=0.007), and salivary gland swelling (OR: 49.39, 95% CI: 2.02–1206.30, p-value=0.017). Age, gender, fatigue, pain, anxiety, and autoantibodies were not significantly associated with xerostomia. InterpretationFindings from this study indicate that patient-reported xerostomia is highly prevalent among SS patients and is associated with several clinical phenotypes of this complex syndrome, thereby making it an important indicator of SS. The evidence also suggests that xerostomia is not limited to low salivary flow but might be reflective of compositional changes of saliva. Consequently, these findings suggest the need to consider xerostomia in the development of SS classification criteria and in patient-centered outcomes research in SS intervention trials.This research was supported by the Intramural Research Program of the National Institutes of Health (NIH), National Institute of Dental and Craniofacial Research (NIDCR) Grant # DE000704-15. Dr. Baer is supported by RO1-DE-12354-15A1.

Clinical and Serological Features of Sjogren Syndrome in Patients with Rheumatoid Arthritis

Background: It is uncertain whether the Sjogren Syndrome (SS) associated with Rheumatoid Arthritis (RA) represents a clinical entity similar to primary SS (pSS) or merely a manifestation in the clinical spectrum of RA. In the present study, we sought to determine the clinic and serologic features of SS associated with RA in comparison to the RA features using well defined SS classification criteria. Methods: RA patients successively referred for a biologic infusion were questioned on oral and ocular dryness. Schirmer’s test and unstimulated salivary flow were performed in each patient. Patients with subjective oral or ocular dryness and/or 1 abnormal objective test underwent a minor salivary gland biopsy. The diagnosis of secondary SS was based on the criteria of EuropeanAmerican consensus group criteria for SS. Clinical and biological parameters of SS and RA (with measure of disease activity and health status of RA, search for Raynaud’s phenomenon, anti-CCP, RF anti-SSA-positivity and beta2-microglobulin level) were then compared between patients with/without sSS.

FRI0437 How do we Treat Patients with Focus Score ≥1, but not Consistent with the New American College of Rheumatology Classification Criteria for SjÖGren's Syndrome? Evaluation from Study in Japanese Patients

BackgroundOne of the criteria for Sjögren's syndrome (SS) is focal lymphocytic infiltration in minor salivary gland biopsy (MSGB). Few studies have revealed that the proportion of patients with focus score...

FRI0435 Salivary Epidermal Growth Factor (EGF) in SjÖGren's Syndrome: Association between Salivary EGF Levels and the Severity of Intraoral Manifestations

BackgroundXerostomia accompanied in patients with Sjögren's syndrome (SS) relates to reduced salivary flow rate as a result of salivary gland dysfunction. Dry mouth causes various intraoral manifestations such as refractory...