sSS Patients Research Articles

Comparative Analysis of Glandular and Extraglandular Manifestations in Primary and Secondary Sjögren's Syndrome: A Study in Two Academic Centers in North-East Romania.

This study investigates the clinical characteristics and differences between primary Sjögren's Syndrome (pSS) and secondary Sjögren's Syndrome (sSS) in a cohort of 50 patients. Conducted across two academic facilities in North-East Romania, the study emphasizes the importance of glandular and extraglandular manifestations, focusing on salivary flow rates, pH levels, and buffer capacity. Patients were diagnosed using the 2016 ACR-EULAR classification criteria, with a detailed examination including salivary tests, biopsies, and antibody presence. The findings highlight significant differences between pSS and sSS, particularly in salivary function, with pSS patients exhibiting more severe glandular dysfunction. The study also notes a higher prevalence of inflammatory joint involvement in sSS patients, often associated with rheumatoid arthritis. Statistical analysis revealed correlations between salivary parameters and disease progression, underscoring the necessity of tailored treatment strategies. The research suggests that lower salivary flow rates and altered pH levels in pSS patients contribute to compromised oral health, including increased dental cavities and periodontal disease. The study's results contribute to a deeper understanding of Sjögren's Syndrome and reinforce the need for multidisciplinary management to address both systemic and oral health complications in these patients.

Gynecological symptoms in primary and secondary Sjögren's syndrome and the effect of the disease on sexuality.

Sjögren's syndrome (SS) is an autoimmune systemic disease affecting many organs and systems, such as genital system. This study aimed to present the gynecological symptoms of patients who were followed up in an outpatient clinic because of primary Sjögren's syndrome (pSS) and secondary Sjögren's syndrome (sSS) and to show how the disease affected sexuality. This study is a cross-sectional study conducted between 2019 and 2020. The study sample consisted of 60 pSS patients, 42 sSS patients, and 52 healthy control subjects. All the participants were questioned about sexuality, and completed the 36-item Short Form Survey, Hospital Anxiety and Depression Scale, Health Assessment Questionnaire, and Modified Hill questionnaire. The patients had a mean age of 55.6 ± 11.85years in pSS, 59.39 ± 11.18years in sSS, and 56.1 ± 10.46years in healthy control subjects. Vaginal and vulvar dryness and dyspareunia were present at a significantly higher rate in SS, especially in pSS, compared with the control subjects. The Health Assessment Questionnaire score was significantly lower in the pSS group than in the sSS group. Arthralgia, myalgia, and fatigue were prominent in all SS patients. Gynecological symptoms, sexual ability, and the effects of the disease on sexuality should be questioned in all SS patients. It is very important that we evaluate the gynecological symptoms of both pSS and sSS patients and the effect of the disease on these symptoms. The small number of patients and healthy control subjects is a limitation. The gynecological and musculoskeletal symptoms negatively affected sexuality in patients with pSS and sSS, and the negative effect of the disease on sexuality was more pronounced in the pSS group.

Clinical profiles of SS-ILD compared with SS-NILD in a Chinese population: a retrospective analysis of 735 patients

Background Interstitial lung disease (ILD) is a serious complication in patients with Sjögren’s syndrome (SS). Most studies on primary SS (pSS) with ILD are limited in sample size, and studies on secondary SS (sSS) with ILD are rare. This study aimed to elucidate both primary and secondary SS-associated ILD (SS-ILD) based on a large cohort. Methods The medical records of hospitalized patients diagnosed with SS at the Second Xiangya Hospital of Central South University from January 2010 to May 2020 were retrospectively reviewed. Clinical manifestations, medical history, biological results and imaging data were collected. Results Of the 735 SS patients enrolled in this study, 563 (76.6%) were diagnosed with pSS, 172 (23.4%) were diagnosed with sSS. Additionally, 316 (43.0%) were diagnosed with SS-ILD. No significant difference was found between the pSS and sSS groups concerning the incidence of ILD (p = .718). Factors associated with SS-ILD were older age (p < .001), male sex (p = .032), female sex at menopause (p = .002), Raynaud’s phenomenon (p < .001), low levels of albumin (p = .010) and respiratory symptoms (p < .001). The SS-ILD group showed higher counts of platelets (p < .001). The three most frequent high-resolution CT (HRCT) findings of SS-ILD were irregular linear opacities (42.7%), grid shadows (30.7%) and pleural thickening (28.5%). NSIP (56.3%) was the most frequent HRCT pattern. Compared with pSS patients with ILD (pSS-ILD) patients, sSS patients with ILD (sSS-ILD) patients had a higher incidence of proteinuria (p < .001) and hypercreatinaemia (p = .013), a higher level of erythrocyte sedimentation rate (ESR) (p = .003), low levels of complement 3 (C3) (p = .013), lymphocytes (p = .009) and leukocytes (p = .024), and worse DLCO (%Pred) (p = .035). Conclusions ILD is a common pulmonary involvement in both pSS patients and sSS patients. Older age, male sex, female sex at menopause, Raynaud’s phenomenon, low albumin levels and respiratory symptoms are risk factors associated with SS-ILD. NSIP is important HRCT feature of SS-ILD. sSS-ILD patients showed worse laboratory results and pulmonary function. KEY MESSAGE Older age, male sex, female sex at menopause, Raynaud’s phenomenon, low albumin levels and respiratory symptoms are risk factors associated with SS-ILD. SS-ILD patients show higher counts of platelets and less purpura. sSS-ILD patients have worse laboratory results and pulmonary function.

Tissue-Specific Autoantibodies Improve Diagnosis of Primary Sjögren's Syndrome in the Early Stage and Indicate Localized Salivary Injury.

Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands. Due to the absence of specific clinical manifestations and biomarkers in the early stage, pSS is generally underrecognized. To elucidate the role of the tissue-specific autoantibodies (TSAs), i.e., anti-CA6, anti-SP1, and anti-PSP antibodies, we enrolled 137 pSS patients, 32 secondary Sjögren's syndrome (sSS) patients, and 127 healthy controls (HCs), whose serum and saliva samples were collected. TSA levels were detected by ELISA, and the clinical and laboratory data was reviewed from the medical records. The analysis results showed the following: (1) Compared to HCs, the serum IgA levels of anti-CA6, anti-SP1 and anti-PSP were significantly higher in pSS as well as in sSS patients, and anti-CA6 IgG was also notably higher in pSS patients. (2) The positivity of anti-CA6, anti-PSP and all the three antibodies together were significantly increased in anti-SSA-negative pSS patients. (3) The average IgM levels of anti-CA6 and anti-SP1 decreased as the disease duration extended. (4) The anti-CA6-positive patients have significantly higher levels of serum IgA, while the anti-PSP-positive group has a notably higher serum IgM level. (5) Another autoantibody specific to the salivary glands, anti-α-fodrin antibody, was elevated in TSA-positive patients, especially in the anti-CA6-positive group. (6) Preliminary detection of saliva TSAs showed that all the IgG levels of these three antibodies increased significantly in pSS patients. In conclusion, TSAs improve diagnosis of pSS in the early stage, especially in anti-SSA-negative patients, and their tissue-specific nature indicates localized salivary injury, which deserves further studies to clarify the mechanism.

Additional Tunisian patients with Sanjad–Sakati syndrome: A review toward a consensus on diagnostic criteria

Background and objectivesSanjad–Sakati syndrome (SSS; OMIM 241410) is a rare autosomal recessive disorder found almost exclusively in people of Arab origin. It is characterized by congenital hypoparathyroidism, severe prenatal and postnatal growth retardation, and distinct facial dysmorphism. The molecular pathology of this syndrome was shown to be due to a mutation in the tubulin-specific chaperone E (TBCE) gene in chromosomal area 1q42-q43. We aimed to detect and confirm the common mutation responsible for SSS in Tunisian patients and review the literature in order to create a set of clinical diagnostic criteria that might provide appropriate indications for molecular testing. MethodsThree Tunisian patients with clinical feature of SSS were examined via direct Sanger sequencing of exon 3 of the TBCE gene. ResultsMutation analysis of the TBCE gene revealed the common 12-bp (155-166del) deletion in three new patients, thus raising the number of reported SSS patients to 73. Reviewing the literature, we suggest a scoring system that assigns one point each for major criteria and one half point for minor criteria. Interpretation and conclusionsSSS is an autosomal recessive disorder found in the Middle Eastern population with an estimated incidence of 1 per 40,000–100,000 live births in Saudi Arabia. Reviewing the literature on both its clinical and biochemical characteristics, we suggest for the first time, based on defined major and minor SSS criteria, a clinical scoring system for the diagnosis of SSS. On the one hand, an established scoring system will provide appropriate indications for molecular testing and, on the other hand, reviewed data on SSS will help delineate the phenotype and draw a distinction between differential diagnoses.

A Prospective Study on the Characteristics of Sensitive Skin Syndrome in Chinese Patients Attending a Private Clinic-Based Setting in Hong Kong

A clinic based prospective study was carried out during the period of 1st May 2018 to 30 April 2019 in a private clinic located in the Centre of Kowloon district; Hong Kong. The objectives were to study the proportion of clinically diagnosed SSS patients attending the private clinic setting, demographic and clinical characteristics. The study suggested that SSS was a common disease encountered in the private dermatology clinic with a female gender predominance over the face region and at the age group of age 20 to 30. Our study also suggested that SSS patients could be identified by a validated ten item Sensitive Scale–10 questionnaire with a cut-off score of above 20. This is in accordance with that reported in the documented literature. A cut off value of patients scored above 20 in the questionnaire without concomitant skin disease at the same time can be diagnosed to have probable SSS. The score may be effectively used as a monitoring tool of the progress of SSS after management including counseling explanation, education and treatment.

Levels of EGF and VEGF in patients with primary and secondary Sjögren's syndrome.

Aberrant angiogenesis plays a role in the pathogenesis of Sjögren's syndrome (SS). The aim of this study was to compare the levels of vascular endothelial growth factor (VEGF) and epidermal growth factor (EGF) in stimulatory parotid saliva and in serum in healthy subjects (HS), patients with primary SS (pSS) and secondary SS (sSS) and to evaluate the expression of EGF, proangiogenic VEGF165 and antiangiogenic VEGF165 b mRNA isoforms. Additionally, we determined the salivary levels of serine/arginine splicing factor (SRSF1), which regulates VEGF165 and VEGF165 b expression. The study comprised 34 women (16 with pSS and 18 with sSS) and healthy subjects for blood and saliva sampling. EGF and VEGF levels in saliva and serum and salivary SRSF1 levels were determined by enzyme-linked immunosorbent assay (ELISA). The expression of VEGF165 , VEGF165 b and EGF in peripheral blood mononuclear cells (PBMC) was evaluated by quantitative polymerase chain reaction (qPCR). There were no differences in the levels of EGF, VEGF, SRSF1 and in the expression of the EGF, VEGF165 and VEGF165 b between HS and SS patients, or between pSS and sSS patients. The salivary levels of VEGF165 and EGF were significantly higher in pSS, sSS and HS than serum levels. Levels of SRSF1 correlated positively with VEGF and EGF levels. Levels of EGF, VEGF and SRSF1 correlated with each other. The balance of VEGF isoforms is not disturbed in SS. Saliva is more sensitive for the detection of EGF and VEGF than serum, but salivary levels of those proteins are not representative for SS.

Clinical features and risk factors of neurological involvement in Sj\xf6gren\u2019s syndrome

BackgroundTo investigated distinct manifestations of Sjögren’s syndrome (SS) patients with neurological complications and the potential risk factors associated with neurological complications in SS, and to produce a disease evaluation and neurological involvement prediction for SS.Methods566 patients who fulfilled the 2002 classification criteria for SS from the Rheumatology Department of the First Affiliated Hospital of Wenzhou Medical University were included in the cross-sectional study. Clinical, immunological and histological characteristics were surveyed, and potential risk factors for neurological complications were examined by multivariate analysis.ResultsAmong 566 SS patients, 184 (32.5%) patients had neurological involvement, with more than 10% got limbs pain, limbs numbness and cerebral infarction, respectively. Of these 184 SS patients with neurological complications, secondary SS (sSS) patients had a higher prevalence of peripheral nervous system (PNS) involvement than primary SS (pSS) patients (31.1 vs. 19%). And sSS patients showed higher total ESSPRI score and higher prevalence of xerostomia and low C3, C4 levels with more liver, articular involvement and saliva gland atrophy, and more severe lymphocyte infiltration in salivary glands than pSS patients. As for the specific factors associated with neurological involvement, low C3 level were found to be significant in pSS or sSS patients who were younger 50 year old, and ANA positivity, cardiac involvement, saliva gland atrophy were demonstrated to be associated in elder pSS patients. And xerophthalmia was found to be associated in sSS patients.ConclusionLow complement (C3) levels, xerophthalmia, ANA positive, cardiac involvement and labial salivary gland histological result were good ways to predict neurological complications in different subgroups of SS, which might provide insight into better clinical decision-making, especially at early stages of the disease.

Meibomian Gland Dysfunction in Primary and Secondary Sjögren Syndrome

Purpose: We hypothesized that women with primary (pSS) and secondary Sjögren syndrome (sSS; with systemic lupus erythematosus [SLE] or rheumatoid arthritis [RA]) have meibomian gland dysfunction (MGD). We sought to test our hypothesis. Methods: Subjects with pSS, sSS + SLE, sSS + RA, and non-SS-related MGD were recruited from the Sjögren’s Syndrome Foundation or outpatient clinics at Tufts University School of Dental Medicine or Brigham and Women’s Hospital. The control population was recruited from the Greater Boston area. After providing written informed consent, the subjects underwent an eye examination and/or completed two questionnaires that assess symptoms of dry eye disease (DED). Results: Our results demonstrate that pSS and sSS patients have MGD. These subjects had meibomian gland orifice metaplasia, an increased number of occluded meibomian gland orifices, and a reduced quality of meibomian gland secretions. Further, patients with pSS, sSS + SLE, sSS + RA, and MGD had significant alterations in their tear film, lid margin, cornea, and conjunctiva. Symptoms of DED were increased ∼10-fold in all pSS, sSS, and MGD groups relative to controls. Conclusions: Our findings support our hypothesis and show that individuals with pSS, sSS + SLE, and sSS + RA have MGD. In addition, our study indicates that patients with pSS and sSS have both aqueous-deficient and evaporative DED.

Characteristics of germinal center-like structures in patients with Sjögren's syndrome.

To analyze the relationship between ectopic germinal centers (GCs) in the salivary glands and the clinical/laboratory characteristics of patients with Sjögren's syndrome (SS). Retrospectively, 126 patients with primary SS (pSS) and 16 patients with secondary SS (sSS) were analyzed. Minor salivary gland biopsies were evaluated for the presence of GC-like morphology by hematoxylin and eosin (H&E) and immunohistochemical (IHC) staining for CD21. Clinical and serological data were obtained from medical records. GC-like structures were observed in 36/126 (28.6%) pSS patients and 4/16 (25.0%) sSS patients. The mean inflammatory focus score of the gland was significantly higher in GC-positive samples than in GC-negative ones in both pSS and sSS patients (P = 0.007 and 0.024, respectively). In pSS, significantly elevated titers of rheumatoid factor (RF)-IgM (P = 0.023) and antinuclear antibodies (ANA) (P = 0.036), increased levels of IgA (P = 0.012) and IgG (P = 0.017) were encountered in GC-positive patients. The GC-positive group also presented higher prevalence of anti-SSA antibodies, lower levels of white blood cells, higher levels of erythrocyte sedimentation rate and γ-globulin, although not statistically significant. In sSS patients with ectopic GC formation, ANA titers were remarkably elevated. The anticyclic citrullinated peptide (anti-CCP)-IgG titers and the prevalence of antikeratin antibody (AKA)-IgG, antiperinuclear factor (APF)-IgG were also increased, yet not significantly. GCs were found to be associated with antibody and immunoglobulin production. This study indicates that SS patients with ectopic GCs have distinct features. Ectopic GC structures were particularly noted in patients with higher focus scores, and might play an essential role in sustaining antibody production as well as B cell activation.

Growth Arrest-Specific 6 Protein in Patients with Sjögren Syndrome: Determination of the Plasma Level and Expression in the Labial Salivary Gland.

AimsGrowth arrest-specific protein 6 (Gas6) is a vitamin K-dependent protein expressed by endothelial cells and leukocytes that are involved in cell survival, migration, and proliferation in response to inflammatory processes. The aim of this study was to assess the implications of Gas6 in Sjögren syndrome (SS) and its expression in the labial salivary gland.Methods and ResultsA total of 254 adults, including 159 with primary Sjögren syndrome (pSS), 34 with secondary Sjögren syndrome (sSS), and 61 normal controls, were recruited. Plasma Gas6 concentrations were determined, and Gas6 expressions in labial salivary gland (LSG) tissues from controls and pSS and sSS patients were also evaluated. Plasma Gas6 concentrations were significantly lower among patients with pSS than normal controls (13.5 ± 8.6 vs. 19.9 ± 13.4 ng/ml, p < 0.001). There were, however, no significant differences in plasma Gas6 levels between pSS and sSS patients (13.5 ± 8.6 vs. 16.9 ± 11.2 ng/ml, p = 0.068). In multivariate logistic regression analysis, after adjustment for white blood cell count, hemoglobin level, platelet count, lymphocyte count, and C3 and C4 levels, lower plasma Gas6 concentrations were significantly associated with an increased risk of SS. Moreover, by using a semi-quantitative scale to evaluate Gas6 expression in LSG tissues, Gas6 expression was found to be markedly lower in LSG tissues from pSS patients than in tissues from normal controls.ConclusionsDecreased plasma Gas6 concentration and LSG expression were associated with pSS. As such, Gas6 may represent a novel independent risk factor for pSS, with a potential role in salivary gland inflammation and dysfunction.

Clinical characteristics of RA patients with secondary SS and association with joint damage.

Secondary SS (sSS) is a common extra-articular manifestation of RA. There are conflicting data regarding the association of sSS with worse joint damage. This study aims to characterize sSS patients in an RA cohort and study the association between sSS and joint damage. We conducted a cross-sectional study of RA patients with ≥1 year of follow-up at a large academic centre. Subjects with co-morbid diseases that can also result in sicca symptoms were excluded from the analysis. Subjects were considered to have sSS if they were reported as having sSS by their rheumatologist at recruitment into the cohort and had the diagnosis confirmed by chart review. The primary outcome was Sharp score using bilateral hand radiographs at recruitment. We constructed a linear regression model to determine the association of sSS status and Sharp score adjusted by age, gender, disease duration and ACPA and RF status. We studied 829 RA subjects, mean age 57 years, 83% female, mean RA duration 13 years, 74% seropositive; 85 subjects (10.3%) had sSS. We observed a female predominance (95.3%), longer mean disease duration (16.9 years) and higher frequency of RF or ACPA positive among patients with sSS and RA. Having sSS at baseline was associated with higher Sharp scores (P = 0.03), independent of age, gender, RA disease duration and seropositive disease. In our RA cohort, RA subjects with sSS had worse joint damage, suggesting that sSS is a marker of more aggressive disease.

Significant Association Between Snapping Scapula Syndrome and Anterior Angulation of the Superomedial Scapular Angle

Objectives:Significant Association Between Snapping Scapula Syndrome and Anterior Angulation of the Superomedial Scapular Angle.Methods:In this Institutional Review Board approved study, bony morphologies of the scapula were evaluated on the MRIs of 28 patients (28 scapulae) with SSS and 20 patients (22 scapulae) with non-SSS pathologies. Patients with SSS were identified from a prospective surgical registry that had undergone a preoperative shoulder MRI along with scapular bursectomy and/or superomedial angle resection. The non-SSS patients underwent shoulder MRIs for other reasons that subsequently allowed for evaluation of the scapula. The medial scapula corpus angle (MSCA), was measured on axial STIR or PD FSE sequences cranial to the spine of the scapula (Figure 1). Scapula length angled towards rib cage was documented. Scapulae were categorized as straight, s-shaped or concave. Two blinded observers, one radiologist and one orthopedic surgeon, reviewed all MRIs. MSCAs were measured using Stryker OfficePACS Power 4.1 Express Edition (Kalamazoo, MI). Positive MSCAs were defined as anterior scapular angulation towards the thorax in the axial plane whereas negative MSCAs were defined as posterior scapular angulation away from the thorax in the axial plane.Results:Axial scapula corpus configurations were identified: 31 scapulae were of the straight type, 14 were s-shaped and 5 were concave. All five concave scapulae had surgically confirmed SSS. The measurement of MSCA had excellent inter-observer agreement of 0.81 [95% CI, 0.68 to 0.89] and a fair to good intra-observer agreement of 0.68 [95% CI, 0.50 to 0.80]. There were significant differences in the mean MSCAs between those with SSS (14.4° ± 19.3°) and those with other pathologies (-3.3° ± 15.3°; p = 0.001). After excluding all concave scapulae (n = 5), the differences in the mean MSCAs between the SSS and non-SSS groups were significant (MSCA for SSS: 15.3° ± 17.5° MSCA for non-SSS: -3.3° ± 15.3°; p < 0.0001). Twenty of 28 (71.4%) scapulae with a positive MSCA had SSS, whereas only 3 of 17 (25%) scapulae with a negative MSCA had SSS (see Figure 1). The mean length of the medial scapula border angled to towards the rib cage was 14.4 mm (± 4.6 mm).Conclusion:Anterior angulation of the superomedial angle in the axial plane had an association with SSS. Those patients with a concave-shaped scapula and a positive MSCA may be at risk for SSS. This information may have clinical relevance in the treatment of SSS patients since there are no guidelines on the amount of scapular resection may or may not be needed. The MSCA may prove helpful in determining the location and amount of scapula resection needed to reduce SSS symptoms in patients.

Distribution pattern of Sjögren's syndrome: a sialographical study.

The present controlled sialographical study was conducted to learn more from the horizontal and vertical symmetry of the ductal lesions of the major salivary glands in primary (pSS) and secondary (sSS) forms of the disease. A total of 98 patients (38 pSS patients, 38 sSS patients, 22 control subjects) were included in the study. Contrast radiography of both parotid and submandibular glands was performed within the same session. A 6-point scoring system allowed summary indexes for each of the glands to be calculated. Pansialography was accomplished within 30 min each. The sparsity of the branching pattern of the ducts was the most frequent pathological finding. In pSS, horizontal symmetry was more pronounced in the parotid glands, whereas in sSS it was more pronounced in the submandibular glands. The most discriminating features were the width of the peripheral ducts in the parotid and the number of acinar dilatations in the submandibular glands. The most advanced lesions were found in the left parotid gland. The peripheral ducts are more affected by SS than the main excretory duct. There is a tendency for asymmetric involvement of the parotid glands in pSS and of the submandibular glands in sSS. Parotid glands are globally more involved than submandibular glands. Differential diagnosis between pSS and sSS cannot be accomplished by means of pansialography alone. Left parotid sialography is recommended for routine use.

THU0555 Validation of a Portable Device (RE.MO.TE.) for the Hand Functional Assessment in Patients with Chronic Rheumatic Diseases

BackgroundPatients with inflammatory rheumatic diseases characterized by a chronic progressive course, such as Rheumatoid Arthritis (RA) and Systemic Sclerosis (SSc), develop a disability of the hand which requires an adequate...

Effect of managed ventricular pacing on the life quality of SSS patients with pacemakers

Objective To study the effect of managed ventricular pacing (MVP) on the life quality of SSS patients with pacemakers.Methods Pacemakers with MVP algorithm were implanted in 15 patients.Conventional mode of DDDR was programmed in the first month post operation,and then MVP algorithm was programmed in the second month.Variation of the percentage of the accumulated ventricle pacing ( CumVP％ ),change of four kinds of pacing and perception state time percentage were analyzed respectively in DDDR and MVP.The quality of the life were tested by the quality of life questionnaire of Minnesota in heart failure subjects (LiHFe) in two monthes.Results During following-up,CumVP％ of the MVP algorithm was 1.6％ lower than 91.3％ of DDDR algorithm,and the difference of four kinds of pacing and perception state time percentage was statistically significant (U=130.5,P ＜0.01).In MVP algorithm the LiHFe total score (10.4 ±4.2) was significantly lower than that in the conventional DDDR algorithm (34.0 =3.4) ( t =49.4,P ＜ 0.01 ) ; the difference of other dimensions score in DDDR and MVP was statistically significant (P ＜ 0.01 ).Conclusions MVP algorithm for SSS patients implanted with cardiac pacemaker is a safe,effective and reliable intervention,which can improve their quality of life. Key words: Managed ventricular pacing; DDDR mode; Life quality; SSS

Permanent Left Atrium Pacing via Coronary Sinus

Atrial pacing is effective in sick sinus patients. In high threshold right atrium, however, atrial pacing is difficult. Left atrium muscle exists around coronary sinus. Left atrium pacing is enabling via coronary sinus. Patients: From 2008 to 2010, four SSS patients and two A-V block patients received permanent left atrium pacing via coronary sinus. There were three men and three women. Patient’s mean age was 67 years old (43–83). One patient was post MVR. Other patient became reoperation since atrial lead placed upper atrial septum was floated. Methods and Results: Pacing lead was placed via left subclavian vein with cut down technique. Screw-in lead was used. First right atrium upper septum was attempted, next right atrium appendage, finally lower septum or coronary sinus. Lead placed site was proximal position in 5 patients and distal in one patient. Atrial threshold is 0.5–1.25 V at proximal and 3 mV at distal. Atrial sensing is 0.5–4.3 mV. Lead impedance is 464–583 Ω. Post operative chest lateral Xp showed atrial lead in coronary sinus. Negative P wave was showed in ECG 2 lead. There were no complications of infection, coronary sinus thrombosis, hemorrhage, or trauma. All patients alive and DDD pacing was maintained at last examination. Conclusion: Left atrium pacing via coronary sinus is a safe and feasible technique.

Proteomic analysis of saliva: a unique tool to distinguish primary Sjögren's syndrome from secondary Sjögren's syndrome and other sicca syndromes

IntroductionA growing interest has arisen in salivary proteomics as a tool for the identification of biomarkers for primary Sjögren's syndrome (pSS). Nonetheless, only a limited number of preclinical validation studies have been performed, limiting the possibility of translating proteomic results into clinical practice. The primary aim of this study was to refine the diagnostic power of a panel of candidate salivary biomarkers described in pSS with respect to both healthy volunteers and pathological controls. We also explored the pathogenetic function of the detected putative biomarkers both in the local exocrinopathy and in the systemic inflammatory processes of SS.MethodsOne hundred and eighty patients were included in the study overall. In the first "exploratory phase", we enrolled 40 females with pSS, 40 sex- and age-matched healthy volunteers, 10 patients with sicca non-SS and 15 secondary SS (sSS) patients. The testing cohort of the second "challenge phase" of the study was represented by 75 unselected, consecutive subjects: 19 pSS, 21 healthy volunteers, 10 sicca non-SS and 25 sSS patients. Salivary proteomic analysis was performed combining two-dimensional electrophoresis (2DE) and matrix-assisted laser desorption/ionisation time-of-flight mass spectrometry (MALDI-TOF-MS). Western blot (WB) analysis and enzyme-linked immunosorbent assay (ELISA) were employed to validate 2DE results. Ingenuity Pathway Analysis (IPA) Knowledge base was adopted to associate candidate biomarkers in a signalling pathogenetic network.ResultsA total of 28, 6, 7 and 12 protein spots were found to be significantly different in pSS samples with respect to healthy volunteers, non-SS sicca syndrome, SSc-sSS and rheumatoid arthritis-sSS, leading to the identification of 15 differently expressed proteins. Among them, α-amylases precursor, carbonic anhydrase VI, β-2 microglobulin, glyceraldehydes-3-phosphate dehydrogenase (G3PDH), epidermal fatty acid binding protein (E-FABP) and immunoglobulin k light chain (IGK-light chain) apparently showed the most significant differences in pSS when compared to healthy volunteers and non-SS pathological controls. On the other hand, as expected, pSS and sSS salivary profiles shared a great number of similarities.ConclusionsThis study demonstrated that salivary fluid might represent a novel ideal milieu for the detection of a diagnostic panel of candidate biomarkers for pSS, and to gain an insight into the pathogenetic processes underlying glandular and systemic autoimmune disorders.

E0649 Long-term effects of RA-URIS pacing on left ventricular remodelling in patients with chronic heart dysfunction

ObjectiveTo assess the effects of RA-URIS dual chamber sequential pacing on left ventricular remodelling and cardiac function in SSS or AVB patients with chronic heart dysfunction.Methods32 patients with SSS or...

Health-related quality of life, employment and disability in patients with Sjögren's syndrome

To compare health-related quality of life (HR-QOL), employment and disability of primary and secondary SS (pSS and sSS, respectively) patients with the general Dutch population. HR-QOL, employment and disability were assessed in SS patients regularly attending the University Medical Center Groningen (n = 235). HR-QOL, employment and disability were evaluated with the Short Form-36 questionnaire (SF-36) and an employment and disability questionnaire. Results were compared with Dutch population data (matched for sex and age). Demographical and clinical data associated with HR-QOL, employment and disability were assessed. Response rate was 83%. SS patients scored lower on HR-QOL than the general Dutch population. sSS patients scored lower on physical functioning, bodily pain and general health than pSS patients. Predictors for reduced HR-QOL were fatigue, tendomyalgia, articular involvement, use of artificial saliva, use of anti-depressants, comorbidity, male sex and eligibility for disability compensation (DC). Employment was lower and DC rates were higher in SS patients compared with the Dutch population. SS has a large impact on HR-QOL, employment and disability.