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ID PMID Title PublicationDate abstract
23346917 Primary Sjögren syndrome: an update on current pharmacotherapy options and future directi 2013 Feb INTRODUCTION: Primary Sjögren syndrome (SS) is a chronic systemic autoimmune disease characterized by sicca features and systemic manifestations, and requires a multidisciplinary therapeutic approach. AREAS COVERED: Treatment of sicca manifestations is symptomatic and is based on the administration of topical therapies (saliva substitutes and preservative-free artificial tears). In severe cases of keratoconjunctivitis sicca, topical cyclosporine A may be used. For patients with residual salivary gland function, stimulation of salivary flow with a sialogogue (pilocarpine or cevimeline) is the treatment of choice. The management of extraglandular features must be tailored to the specific organ(s) involved. Hydroxychloroquine may be appropriate for patients with fatigue, arthralgia and myalgia, while glucocorticoids and immunosuppressive agents should be reserved for severe systemic involvement (although no controlled trials in primary SS guide their use). RCTs have demonstrated the lack of efficacy of antitumor necrosis factor agents and promising results for B-cell depleting agents. EXPERT OPINION: The overall low level of evidence in therapeutic studies in primary SS suggests that much larger trials of the most promising therapies are necessary. The use of drugs targeting molecules and receptors involved in the etiopathogenesis of primary SS may open up a new era in the therapeutic management of the disease, but the potential risks and benefits of these agents must be weighed carefully.
23135882 Anti-M3 muscarinic acetylcholine receptor antibodies in patients with Sjögren's syndrome. 2013 Sep Sjögren's syndrome (SS) is an autoimmune disease that affects exocrine glands including salivary and lacrimal glands. Recently, autoantibodies against muscarinic acetylcholine receptor M3 (M3R) have been detected in serum from 9 to 100 % of patients with SS in addition to anti-SS-A and anti-SS-B antibodies. These observations suggest the possibility that anti-M3R antibodies could serve as a new diagnostic test in patients with SS. Some anti-M3R antibodies are directly responsible for salivary underproduction in patients with SS. Thus, strategies designed to eliminate such pathogenic antibodies could help cure SS sufferers. In this review, we summarize the current state of knowledge of anti-M3R autoantibodies in patients with SS and the correlation between B cell epitopes and the function of anti-M3R antibodies.
25343702 Detection of early markers for Sjögren syndrome in dry eye patients. 2014 Dec PURPOSE: The purpose of this case series was to report the detection of early markers for Sjögren syndrome (SS) in 3 patients with chronic dry eye who previously tested negative for classic SS autoantibodies. METHODS: Cases of 3 patients with a history of dry eye are described. Current assessments in a cornea/ocular surface specialist clinical setting and an ocular medical history review are presented in conjunction with laboratory testing for the presence of novel autoantibody markers linked to the early stages of SS. RESULTS: All 3 dry eye patients described in this case series tested negative for the classic autoantibody markers [Sjögren syndrome type A (SS-A) and Sjögren syndrome type B (SS-B)]. However, autoantibodies were detected in these patients indicating a likely diagnosis of early-stage SS. All patients were referred to a rheumatologist for further evaluation and treatment. CONCLUSIONS: Patients with a history of dry eye signs/symptoms that persist despite treatment (male and female patients) may benefit from a serological evaluation for SS that is capable of detecting not only the traditional SS-A and SS-B markers, but also the recently identified autoantibodies.
25197359 Anti-inflammatory effect of peroxisome proliferator-activated receptor-γ (PPAR-γ) on non 2014 OBJECTIVE: To investigate the anti-inflammatory effect of peroxisome proliferator-activated receptor-γ (PPAR-γ) on non-obese diabetic mice (NOD mice) with Sjogren's syndrome. METHODS: 22 eight-week-old female NOD mice were randomly divided into 2 groups. Rosiglitazone and normal saline were administered in the PPAR-γ group and the control group respectively. At the age of 9, 12 and 15 weeks, one mouse in each group was sacrificed respectively, and the remaining mice were sacrificed at the age of 18 weeks. Blood were obtained by cardiac puncture, and salivary glands were resected. The degree of salivary gland damage and infiltration of lymphocytes were examined by H&E staining. The level of IL-1β, IL-4, IL-6 and TNF-α in serum were measured by ELISA. The mRNA expression level of IL-1β, IL-4, IL-6 and TNF-α in MSG were detected by Real-time PCR. Expression of PPAR-γ in the salivary glands was detected by Immunohistochemistry. RESULTS: Compared with the control group, mice in the PPAR-γ group showed that (1) histopathologic changes in the salivary glands were significantly ameliorated; (2) at the age of 18 weeks, IL-6 [(25.86 ± 7.32) vs (37.41 ± 11.34)] and TNF-α [(56.88 ± 22.19) vs (78.61 ± 20.76)] were expressed significantly lower and IL-4 [(25.76 ± 12.65) vs (12.11 ± 3.70)] was expressed significantly higher in serum (P < 0.05); (3) the expression of TNF-α was significantly decreased and the expression of IL-4 was significantly increased in MSG (P < 0.05). CONCLUSION: PPAR-γ ameliorates Sjogren's syndrome on NOD mice effectively. The mechanism may be related to the reduction of Th1 cytokines and change of T helper cell balance from Th1 to Th2.
24706989 Salivary gland ultrasonography improves the diagnostic performance of the 2012 American Co 2014 Sep OBJECTIVE: The aim of this study was to evaluate whether salivary gland ultrasonography (SGUS) improves the diagnostic performance of the 2012 ACR classification criteria for SS. METHODS: We studied a cohort of 101 patients with suspected SS seen at a single centre in Brittany, France. An SGUS echostructure score ≥2 was considered abnormal. The reference standard was a clinical diagnosis of SS made by a group of experts blinded to SGUS findings. RESULTS: SS was diagnosed in 45 patients. Similar proportions of patients with and without SS had an ocular staining score ≥3. Adding RF positivity and ANA titre ≥1:320 as an alternative to anti-SSA/SSB positivity increased the sensitivity of the serological item without modifying specificity compared with using anti-SSA/SSB alone. SGUS was 60.0% sensitive and 87.5% specific for SS. Adding the SGUS score to the ACR criteria increased sensitivity from 64.4% to 84.4% and only slightly decreased specificity, from 91.1% to 89.3%. CONCLUSION: The diagnostic performance of the ACR classification criteria for SS is notably improved by adding the SGUS score. SGUS should be included in future classification criteria for SS.
24439915 A simple test for salivary gland function measuring resting and stimulated submandibular a 2014 Feb OBJECTIVE: This study examined the application of a simple screening test for salivary gland function by measuring resting and stimulated submandibular and sublingual secretions. STUDY DESIGN: An assay system was designed to use filter paper incorporating the chromophore of melanoidin or stimuli such as capsaicin and citric acid. We investigated the relationship between resting and stimulated secretions and melanoidin migration at 2 minutes for healthy and dry mouth groups. RESULTS: The healthy group showed a significant increase in the migration of melanoidin in the paper after citric acid or capsaicin stimulation. In contrast, patients with Sjögren syndrome showed no significant migration in spite of the stimulation. However, some participants with Sjögren syndrome or dry mouth showed a significant increase in the migration of melanoidin after stimulation. CONCLUSIONS: These results show that the newly developed method should be useful for evaluation of residual salivary gland function and screening for hyposalivation with dry mouth.
24347569 Efficacy and safety of belimumab in primary Sjögren's syndrome: results of the BELISS ope 2015 Mar BACKGROUND: Increased expression of B cell activating factor (BAFF or B lymphocyte stimulator) may explain the B cell activation characteristic of primary Sjögren's syndrome (pSS). OBJECTIVES: To evaluate the efficacy and safety of belimumab, targeting BAFF, in patients with pSS. METHODS: Patients were included in this bi-centric prospective 1-year open-label trial if they fulfilled American European Consensus group criteria, were anti-Sjögren's syndrome A-positive and had current systemic complications or salivary gland enlargement, or early disease (<5 years), or biomarkers of B cell activation. They received belimumab, 10 mg/kg, at weeks 0, 2 and 4 and then every 4 weeks to week 24. The primary end-point, assessed at week 28, was improvement in two of five items: reduction in ≥30% in dryness score on a visual analogue scale (VAS), ≥30% in fatigue VAS score, ≥30% in VAS pain score, ≥30% in systemic activity VAS assessed by the physician and/or >25% improvement in any B cell activation biomarker values. RESULTS: Among 30 patients included, the primary end-point was achieved in 18 (60%). The mean (SD) European League Against Rheumatism (EULAR) Sjögren's Syndrome Disease Activity Index decreased from 8.8 (7.4) to 6.3 (6.6) (p=0.0015) and EULAR) Sjögren's Syndrome Patient Reported Index from 6.4 (1.1) to 5.6 (2.0) (p=0.0174). The mean dryness, fatigue and pain VAS varied from 7.8 (1.8) to 6.2 (2.9) (p=0.0021), 6.9 (1.8) to 6.0 (2.2) (p=0.0606) and 4.6 (2.6) to 4.7 (2.4) (p=0.89), respectively. Salivary flow and Schirmer's test did not change. CONCLUSIONS: These encouraging results justify future randomised controlled trials of belimumab in a selected target population of pSS patients most likely to benefit from treatment.
24138706 The association among leukocyte apoptosis, autoantibodies and disease severity in systemic 2013 Oct 19 BACKGROUND: Both apoptosis and autoantibodies are important factors associated with disease activity in the pathogenesis of systemic lupus erythematosus (SLE). This study tested the hypothesis that increased leukocyte apoptosis is associated with elevated levels of autoantibodies and the disease activity of SLE. METHODS: Leukocyte apoptosis was determined by flow cytometry, including annexin V, APO2.7, and 7-amino-actinomycin D (7-AAD) on each subtype of leukocyte in 23 patients with SLE. Leukocyte apoptosis was also evaluated in nine patients with Sjogren's syndrome (SJS) and in 20 volunteer subjects. Titers of common autoantibodies and the disease activity index (SLEDAI-2 k) of the SLE patients were also determined. RESULTS: Except for annexin V and APO 2.7 of monocytes and late apoptosis (annexin V+7-ADD) of lymphocytes, apoptosis in the total and in subsets of leukocytes were significantly higher in SLE patients than in controls (all p<0.05, post hoc analysis). The mean percentage of late apoptosis of leukocytes (annexin V+7-AAD) positively correlated with levels of anti-Ro52/60 (r=0.513, p<0.01), anti-La (r=0.439, p=0.04), and anti-Mi-2 (r=0.492, p=0.02), and inversely correlated with both C3 and C4 levels, although not statistically significant. The percentage of APO2.7 of CD19+ cells positively correlated with SLEDAI-2 K score (p=0.01). CONCLUSIONS: Leukocyte apoptosis is significantly higher in patients with SLE and correlates well with the levels of several autoantibodies. The APO2.7 of B-lymphocyte (CD19+) cells positively correlates with the disease activity of SLE.
23096558 Potential role of adenosine deaminase in the diagnosis of adult-onset Still's disease. 2013 May Adult-onset Still's disease (AOSD) is a systemic inflammatory autoimmune disorder of unknown etiology and pathogenesis. There are no specific laboratory tests for AOSD. To investigate the potential role of adenosine deaminase (ADA) in the diagnosis of AOSD and analyze the correlation among ADA, LDH and WBC (white blood cell count), the serum levels of ADA and LDH in 26 patients with active untreated AOSD, 40 patients with active systemic lupus erythematosus (SLE) as disease control and 48 healthy volunteers as healthy control were determined using automatic biochemical analyzer (Olympus AU2700, Japan). WBC was examined by automatic blood cell analyzer (Beckman Coulter Hmx, America). Significantly higher levels of serum ADA, LDH and WBC were found in active untreated AOSD patients than in active SLE patients and healthy volunteers (F = 27.823; P = 0.000; F = 28.458, P = 0.000; F = 51.929, P = 0.000). Serum ADA were related to LDH level in patients with AOSD patients (r = 0.786, P = 0.000 < 0.01). Both ADA and LDH were not related to WBC (r = 0.244, P = 0.229 > 0.01; r = 0.054, P = 0.794 > 0.01). This is the first study to show that serum ADA could play an important role in AOSD and may be an important biomarker for the diagnosis of AOSD. Serum ADA could be another diagnostic marker independent from whole blood WBC.
22490059 Urban legends series: Sjögren's syndrome. 2013 Jan Sjögren's syndrome (SjS) is one of the most common autoimmune rheumatic diseases, clinically characterized by xerostomia and keratoconjunctivitis sicca. We investigated the following controversial topics: (i) Do we have reliable ways of assessing saliva production? (ii) How important are the quantity and quality of saliva? (iii) Are only anti-SSA/Ro and anti-SSB/La relevant for the diagnosis of SjS? (iv) Are the American-European Consensus criteria (AECC) the best way to diagnose SjS? Results from literature searches suggested the following: (i) Despite the fact that numerous tests are available to assess salivation rates, direct comparisons among them are scarce with little evidence to suggest one best test. (ii) Recent developments highlight the importance of investigating the composition of saliva. However, more research is needed to standardize the methods of analysis and collection and refine the quality of the accumulating data. (iii) In addition to anti-Ro/La autoantibodies, anti α-fodrin IgA and anti-MR3 autoantibodies seem to be promising diagnostic markers of SjS, but more studies are warranted to test their sensitivity and specificity. (iv) AECC are classification, not diagnostic criteria. Moreover, recent innovations have not been incorporated into these criteria. Consequently, treatment directed to patients diagnosed using the AECC might exclude a significant proportion of patients with SjS.
25274901 Serum S100A12 may be a useful biomarker of disease activity in adult-onset Still's disease 2014 Dec OBJECTIVE: S100A12 and soluble receptor for advanced glycation endproducts (sRAGE) have been suggested as biomarkers of disease activity in patients with systemic juvenile idiopathic arthritis. We investigated the clinical significance of these markers in adult-onset Still's disease (AOSD). METHODS: Blood samples were collected from 37 patients with active AOSD and 38 healthy controls (HC). Of the patients with AOSD, followup samples were collected from 19 patients after resolution of disease activity. RESULTS: Serum S100A12 (547.9 ± 148.4 ng/ml) in patients with AOSD was higher than those of HC (272.3 ± 133 ng/ml, p < 0.001). The sRAGE levels of AOSD (514.1 ± 273.6 pg/ml) were lower than those of HC (850.3 ± 405.8 pg/ml, p < 0.001). Serum S100A12 correlated with serum sRAGE (r = -0.228, p = 0.049). Serum S100A12 correlated with erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), ferritin, and systemic score, whereas sRAGE did not correlate with any disease activity markers. In addition, the level of S100A12 was decreased after disease activity was resolved in followed-up patients with AOSD (505.7 ± 161.3 ng/ml vs 361.3 ± 162.5 ng/ml, p = 0.01). Further, the change of S100A12 was well correlated with that of ESR, CRP, and systemic score. CONCLUSION: S100A12 levels showed strong correlations with known disease activity markers such as ESR, CRP, ferritin, and systemic score. In the followup patients with AOSD, most patients showed decreased S100A12 levels after resolution of disease activity. These results suggest that serum S100A12 can be a reliable clinical marker for monitoring disease activity and treatment response.
24990690 Temporal gene expression analysis of Sjögren's syndrome in C57BL/6.NOD-Aec1Aec2 mice base 2014 Sep The purpose of this study was to analyze the temporal gene expression in salivary and lacrimal glands of Sjögren's syndrome (SS) based on time-series microarray data. We downloaded gene expression data GSE15640 and GSE48139 from gene expression omnibus and identified differentially expressed genes (DEGs) at varying time points using a modified Bayes analysis. Gene clustering was applied to analyze the expression differences in time series of the DEGs. Protein-protein interaction networks were used for searching the hub genes, and gene ontology (GO) and KEGG pathways were applied to analyze the DEGs at a functional level. A total of 744 and 1,490 DEGs were screened out from the salivary glands and lacrimal glands, respectively. Among these genes, 194 were overlapped between salivary glands and lacrimal glands, and these genes were compartmentalized into six clusters with different expression profiles. The GO terms of intracellular transport, protein transport and protein localization were significantly enriched by DEGs in salivary glands; while in the lacrimal glands, DEGs were significantly enriched in protein localization, establishment of protein localization and protein transport. Our results suggest that the SS pathogenesis was significantly different in time series in the salivary and lacrimal glands. The DEGs whose expressions may correlate with molecular mechanisms of SS in our study might provide new insight into the underlying cause or regulation of this disease.
24691584 Serum nerve grow factor and brain-derived neurotrophic factor profiles in Sjögren's syndr 2014 Aug The aim of this study is to investigate the serum levels and clinical significance of nerve grow factor (NGF) and brain-derived neurotrophic factor (BDNF) in Sjogren's syndrome (SS) with interstitial lung disease (ILD). Fifty two untreated patients with SS were enrolled in the study. Of them, 25 patients only displayed salivary glands damage and/or lacrimal gland injury (simple SS group). The other 27 patients were lacrimal and/or salivary gland involvement as well as being concomitant only with intestinal lung disease (ILD group). Twenty-five serum samples from healthy volunteers were examined as controls. We measure serum NGF and BDNF levels by ELISA and correlate them with clinical data. Serum NGF levels were significantly higher in ILD patients (372 ± 129 pg/ml) and simple SS patients (293 ± 72 pg/ml) when compared with healthy controls (187 ± 47 pg/ml) (both p < 0.01). Significant difference were also found between the two patient groups (p < 0.01). In contrast, BDNF were significantly decreased in ILD patients (1,005 ± 143 pg/ml) when compared with either simple SS patients (1,204 ± 176 pg/ml, p < 0.01) or healthy controls (1,217 ± 155 pg/ml, p < 0.01). Correlation analysis showed NGF levels in ILD patient were positively correlated with serum levels of C-reactive protein and IgG (both p < 0.05). The abnormal NGF and BDNF in sera may be a potential character of ILD secondary to pSS.
24411591 Treatment for salivary gland hypofunction at both initial and advanced stages of Sjögren- 2014 Mar BACKGROUND AIMS: Non-obese diabetic mice (NOD) exhibit autoimmune Sjögren-like disease (SS-like). We reported previously that a combined-therapy consisting of immuno- and cell-based therapy rescued NOD from SS-like. However, therapies tested to date on NOD mice were aimed at the initial phase of SS-like. It is unknown whether therapies are effective in restoring salivary function when given at an advanced phase of SS-like. METHODS: The efficacy of two therapies (bone marrow versus spleen cells) was compared head-to-head for halting/reversing salivary hypofunction at two critical time points of SS-like (7-week-old NOD with normal saliva output and 20-week-old NOD with minimal saliva). NOD mice were divided into four groups: (i) control, (ii) complete Freund's adjuvant (CFA), (iii) bone marrow transplants with CFA or (iv) spleen cell transplants with CFA. Mice were monitored 8-12 months after therapy. RESULTS: Both cell therapies were effective during the initial phase of SS-like; salivary flow rates were maintained between 80-100% of pre-symptomatic levels. Spleen cell therapy was better than bone marrow when administered in the initial phase of SS-like. When cell therapies were given at an advanced phase of SS-like (20 weeks and older), salivary flow rates improved but were at best 50% of pre-symptomatic levels. Both cell therapies decreased tumor necrosis factor-α, transforming growth factor-β1 levels and T and B cells while increasing epidermal growth factor and regulatory T cells. Elevated serum epidermal growth factor levels were measured in spleen-treated mice. CONCLUSIONS: A therapeutic effect in advanced phase disease, albeit in mice, holds promise for humans in which Sjögren syndrome is generally not diagnosed until a late stage.
24330340 Assessment of the relationship between aortic stiffness and left ventricular functions wit 2014 Jul BACKGROUND AND AIM: Capable of multi-organ involvement in Sjogren's syndrome (SS), cardiac findings of pulmonary effusion, left ventricular diastolic dysfunction and pulmonary hypertension are seen in patients with SS. Aortic stiffness (AS) reflects the mechanical tension and elasticity of the aorta. In this study, our aim is to determine if there is any differences in AS and left ventricular function between patients diagnosed as SS and healthy control groups. METHODS AND RESULTS: We enrolled 50 patients with SS and 47 healthy volunteers with similar demographic characteristics. It was found that isovolumetric relaxation time (IVRT) and deceleration time (DT) were significantly longer and early diastolic wave (E) was significantly lower in patients with SS, but there was no difference in the other parameters. When tissue Doppler echocardiography (TDE) findings were compared between the two groups, it was found that myocardial systolic wave (Sm), myocardial early diastolic wave (Em) and Em/Am ratio were significantly lower, and myocardial isovolumetric relaxation time (IVRTm) and myocardial performance index (MPI) values were significantly higher in patients with SS. A significant positive correlations between aortic strain and Sm (r = 0.35, P < 0.001), Em (r = 0.42, P < 0.001) and Em/Am (r = 0.26, P = 0.008) and negative correlations in IVRTm (r = -0.36, P < 0.001) and MPI (r = -0.24, P = 0.01) were detected. A significant positive correlation between aortic distensibility and Sm (r = 0.36, P < 0.001), Em (r = 0.44, P < 0.001), Em/Am (r = 0.26, P = 0.009) and negative correlation of IVRTm (r = -0.22, P = 0.02) were determined. CONCLUSION: There is a significant relationship between AS and left ventricular diastolic dysfunction in patients with SS in this study. The parameters of aortic elasticity measured by 2D echocardiographic methods can be beneficial in predicting early cardiovascular risk in SS.
24224166 Rare variants in the TREX1 gene and susceptibility to autoimmune diseases. 2013 TREX1 (DNase III) is an exonuclease involved in response to oxidative stress and apoptosis. Heterozygous mutations in TREX1 were previously observed in patients with systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). We performed a mutational analysis of the TREX1 gene on three autoimmune diseases: SLE (210 patients) and SS (58 patients), to confirm a TREX1 involvement in the Italian population, and systemic sclerosis (SSc, 150 patients) because it shares similarities with SLE (presence of antinuclear antibodies and connective tissue damage). We observed 7 variations; two of these are novel nonsynonymous variants (p.Glu198Lys and p.Met232Val). They were detected in one SS and in one SSc patient, respectively, and in none of the 200 healthy controls typed in this study and of the 1712 published controls. In silico analysis predicts a possibly damaging role on protein function for both variants. The other 5 variations are synonymous and only one of them is novel (p.Pro48Pro). This study contributes to the demonstration that TREX1 is involved in autoimmune diseases and proposes that the spectrum of involved autoimmune diseases can be broader and includes SSc. We do not confirm a role of TREX1 variants in SLE.
23534242 [Connective tissue diseases: news in therapy, role of biologics agents]. 2013 Mar 13 Systemic lupus erythematosus and primary Sjögren's syndrom are the two major connective tissue diseases. A better knowledge of their physiopathology allows us today to propose an adapted therapy. Moreover progress concerns the oldest treatment, hydroxychloroquine, and biotherapy. Hydroxychloroquine is still an actual treatment for lupus, its positive effects are better understood today. Nevertheless it does not seem to be efficient to treat primitive Sjögren. Biotherapy targeting B lymphocytes seems efficient in these two connective tissue diseases. Anti TNF therapy is not recommended and seems to induce connective tissue diseases. The real news is the recent approval and reimbursement in Switzerland of the new drug belimumab (Benlysta) in case of moderate lupus.
24646085 Genetic, genomic and epigenetic studies as tools for elucidating disease pathogenesis in p 2014 Apr Primary Sjögren's syndrome (pSS) is characterized by lymphoid infiltration of lacrimal and salivary glands leading to xerophthalmia and xerostomia. pSS is a complex disease involving both genetic and environmental risk factors. Technological advances over the past 10 years have revolutionized genetics and genomics research enabling high-throughput characterization and analysis of DNA and RNA in patient samples on a genome-wide scale. Further, application of high-throughput methods for characterization of epigenetic variation, such as DNA methylation status, is increasingly being applied to AID populations and will likely further define additional risk factors for disease risk and outcome. Main results obtain in pSS through these various approaches are reviewed here.
24477727 Transcultural adaptation of the "EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) 2013 Nov INTRODUCTION: The EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) is an index of primary Sjögren's syndrome (PSS) systemic activity. OBJECTIVE: To perform the ESSDAI transcultural adaptation into Brazilian Portuguese. METHOD: This was a cross-sectional study with 62 patients with PSS according to the criteria of the 2002 American-European Consensus. Six stages were conducted: conceptual, item, semantic, operational, functional, and measurement equivalences (interobserver reproducibility and construct validity). For the validity assessment, the ESSDAI was compared with the Physician's Global Assessment (PhGA), the Sjögren's Syndrome Disease Activity Index (SSDAI), and the Sjögren's Systemic Clinical Activity Index (SCAI). Patients were classified by a specialist physician into two groups according to disease activity (active and inactive), and according to the intention-to-treat (increase in therapy and no increase in therapy). The ESSDAI was tested in these groups. The following statistical tests were used: intraclass correlation coefficient (ICC), Bland-Altman plot for reproducibility, and Spearman's correlation coefficient (r s) and Mann-Whitney's test for validity (P < 0.05 and 95% CI). RESULTS: The mean ESSDAI score was 4.95 ± 6.73. The reproducibility obtained a strong ICC of 0.89 and good agreement. When compared with other indices, it showed a strong r s with PhGA (0.83; P < 0.000), a moderate r s with SSDAI (0.658; P < 0.000) and a weak r s with the SCAI (0.411; P = 0.001). The group "active" and the group " increase in therapy" had higher ESSDAI values (P = 0.000). CONCLUSION: The Brazilian Portuguese version of ESSDAI was shown to be adaptable, reproducible, and valid for this language.
24451006 Predicting progression to lymphoma in Sjögren's syndrome patients. 2014 Apr Although the inherent complexity of the multifactorial nature of primary Sjögren's syndrome (pSS) renders the process of disease prognostication and prediction ambiguous, certain clinical and immunological characteristics have been described as lymphoma predictors in several studies. While the association between pSS and mucosa-associated lymphoid tissue lymphomas is indisputable, recent studies report a predominance of diffuse large B-cell lymphomas implying that pSS-lymphoma association is less subtype-specific than previously considered. The considerable differences in both disease severity and prognosis between patients with various types of lymphoma demand the identification of risk factors that can predict the development of the distinct subtypes. Additionally, a recently discovered diverse range of biological variables appears to influence clinical behavior and lymphoma outcome. In this review, we venture into the area of lymphoma prognostication in pSS, outlining long-established predictors, analyzing currently available prognostic models, and exploring the predictive potential of recent biological and molecular advances.