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Objective Parotid swelling (PSW) is a major predictor of non-Hodgkin's lymphoma (NHL) in primary SS (pSS). However, since detailed information on the time of onset and duration of PSW is scarce, this was investigated to verify whether it may lead to further improved prediction. NHL localization was concomitantly studied to evaluate the role of the parotid gland microenvironment in pSS-related lymphomagenesis. Methods A multicentre study was conducted among patients with pSS who developed B cell NHL during follow-up and matched controls that did not develop NHL. The study focused on the history of salivary gland and lachrymal gland swelling, evaluated in detail at different times and for different durations, and on the localization of NHL at onset. Results PSW was significantly more frequent among the cases: at the time of first referred pSS symptoms before diagnosis, at diagnosis and from pSS diagnosis to NHL. The duration of PSW was evaluated starting from pSS diagnosis, and the NHL risk increased from PSW of 2–12 months to >12 months. NHL was prevalently localized in the parotid glands of the cases. Conclusion A more precise clinical recording of PSW can improve lymphoma prediction in pSS. PSW as a very early symptom is a predictor, and a longer duration of PSW is associated with a higher risk of NHL. Since lymphoma usually localizes in the parotid glands, and not in the other salivary or lachrymal glands, the parotid microenvironment appears to be involved in the whole history of pSS and related lymphomagenesis. [ABSTRACT FROM AUTHOR]

We present the case of a 28-year-old African man with multiple hospitalizations during the past two years due to worsening shortness of breath. The initial work-up revealed recurrent pulmonary embolism and interstitial lung disease (ILD). The diagnosis of primary Sjogren syndrome was established and a lung biopsy demonstrated cellular non-specific interstitial pneumonia (NSIP). Proper treatment of NSIP was partially effective and respiratory failure persisted, implying additional pathology to NSIP. Further evaluation revealed pulmonary hypertension that required treatment with pulmonary vasodilators. [ABSTRACT FROM AUTHOR]

Background: We investigated the safety and efficacy of short-term treatment with topical low-dose hydrocortisone sodium phosphate 0.335% (PFH) in patients with moderate to severe primary Sjögren syndrome (SS)-related dry eye disease (DED). Methods: A retrospective single-centre interventional study. All patients received PFH for 6 days with a pulsed posology: three times daily for 2 days, twice daily for 2 days, and once daily for 2 days. This scheme was repeated for 3 consecutive months and then alternated for 3 months. Data were collected at baseline, 3 months, and 6 months of follow-up. Results: A total of 40 SS patients were enrolled. Conjunctival hyperaemia and corneal-conjunctival stain significantly improved (p < 0.001). Ocular Surface Disease Index score reduced significantly between baseline and 3 months and between baseline and 6 months (p < 0.001). The tear film osmolarity lowered significantly in each eye from baseline to 3 months and from baseline to 6 months (p = 0.002 and p = 0.037, respectively). Comparing results at 3 and 6 months, the Ocular Surface Disease Index score (p = 1.000), the frequency of lacrimal substitutes installation (p = 0.632), and tear film osmolarity (right eye p = 0.518, left eye p = 1.000) did not change significantly. Intraocular pressure did not change during the study period. Conclusion: PFH eye drops with a pulsed posology improve signs and symptoms, not affecting the intraocular pressure in SS-related DED. Therefore, this pulsed treatment is safe and efficacious. [ABSTRACT FROM AUTHOR]

Background: We aimed to explore the underlying mechanism of the total glucoside of peony (TGP) in treating Sjogren syndrome (SS) using the network pharmacology approach. Methods: The protein targets of TGP and SS were identified by database search. Then, the intersection of the two groups was studied. The drug--target network between TGP and the overlapping genes was constructed, visualized, and analyzed by Cytoscape software. Gene Ontology and Kyoto Encyclopedia of Genes and Genomes enrichment were performed to analyze these genes. Finally, the predictions of potential targets were evaluated by docking study. Results: Forty-six overlapping genes were discovered. The results suggested that TGP used in the treatment of SS is associated with cellular tumor antigen p53, neurotrophic tyrosine kinase receptor type 1, and epidermal growth factor receptor, as well as their related 3372 protein networks, which regulate intrinsic apoptotic signaling pathway, cellular response to oxidative stress, rhythmic process, and other processes. Molecular docking analysis proved that hydrogen bonding is the main form of interaction. Conclusion: Our research provided the protein targets affected by TGP in SS treatment. The key targets (caspase 3, vascular endothelial growth factor A, glyceraldehyde-3-phosphate dehydrogenase, etc.), which involve 3372 proteins, are the multitarget mechanism of TGP in SS treatment. [ABSTRACT FROM AUTHOR]

Purpose: The objective of the study was to compare the efficacy and safety of two concentration of autologous serum (AS) 20% vs 50% in recalcitrant moderate‑to‑severe dry eye patients. Methods: A double‑blind prospective, interventional, and randomized study was done on 44 patients (80 eyes) clinically diagnosed with moderate‑to‑severe dry eye disease (DED) that was refractory to conventional treatment, and all patients were treated with AS20% or AS50% for 12 weeks. We documented Ocular Surface Disease Index (OSDI), tear film breakup time (TBUT), OXFORD corneal staining score (OSS), and Schirmer test (ST) at baseline, 2,4,8, and 12 weeks. These parameters were compared in both groups and between the groups by using Student’s t‑test. The study included 11 males and 33 females. Results: Out of 80 eyes, 33 eyes had moderate and 47 had severe DED. The age of patients in AS20% was 44.73 ± 14.37 years, and in AS50% was 46.41 ± 14.47 years. The most common etiology associated with DED was secondary Sjogren syndrome. In moderate DED, both the groups showed significant improvement in both subjective and objective parameters. But in severe DED, the AS20% group failed to show any significant improvement objectively, though subjective improvement was present. Conclusion: In refractory severe DED patients, AS50% is better option for treatment and in moderate DED both concentrations of autologous serum are effective. [ABSTRACT FROM AUTHOR]

Interstitial lung disease associated with Sjögren syndrome belongs to the category of “arthralgia caused by dryness pathogen ” in traditional Chinese medicine, and it is generally believed that its pathogenesis is centered on internal dryness, which is caused by internal heat and burning of body fluid due to the lack of innate endowment or external dryness. The metabolism of water and grain essence is slow, and excessive accumulation of grain essence or abnormal transportation and transformation can lead to turbidity, and the accumulation of turbidity is the basis for the development of turbid poison. Turbid poison has the characteristics of poisonous evil, its nature is harsh, it is more likely to consume qi and blood, and it has the characteristics of agglutination, stagnation and filth. Turbid poison can be classified as a class of original poison. The original base of its transformation is turbidity from the grain essence. If the grain essence is transformed into a mellow and moist essence, alienation is brewed into the turbidity of corruption. The formation of turbid poison caused by spleen deficiency is a process of gradual accumulation, which can damage the internal organs and is complex and easily recurring. We believe that deficiency of the spleen and stomach is the source of dryness and that turbid poison with phlegm and blood stasis in the middle jiao and lung channels is the key factor aggravating the dryness. The theory of turbid poison of spleen and stomach covers the whole disease process. In the early stage, the spleen and stomach are deficient and the turbidity gathers at the beginning, which hinders qi and injures body fluid. In the middle stage, the turbidity breeds turbid poison, which leads to heat with phlegm and blood stasis. In the late stage, the turbid poison accumulates to form a mass, which can damage various organs. Clinical treatment should be based on the degree of turbid poison, blocking the source of turbid poison in the early stage, paying attention to the protection of body fluid, dredging the meridians by detoxification in the middle and late stage and taking in energy to restore the vital qi. The symptoms and signs of interstitial lung disease associated with Sjögren syndrome are consistent with the characteristics of turbid poison invading the human body. The theory of turbid poison of spleen and stomach can provide a new idea for the differentiation and treatment of this disease in traditional Chinese medicine. [ABSTRACT FROM AUTHOR]

Sjögren syndrome, a systemic autoimmune disease that is characterized by dry mouth and eyes, on which treatment decision remain challenging, while traditional Chinese medicine ( TCM) has advantages and is widely used in China. However, the lack of consensus on standardized TCM patterns diagnosis and treatment based on evidence-based evidence, limits the further improvement of clinical efficacy and scientific research level. Rheumatology Branch, Chinese Academy of Chinese Medicine organized more than 50 experts from 48 hospitals across the country to form Expert Consensus for TCM Patterns of Sjögren Syndrome: (ⅰ) pattern of yin deficiency and depletion of fluid; (ⅱ) pattern of deficiency of both qi and yin; (ⅲ) pattern of endogenous heat due to yin deficiency ( pattern of yin deficiency and heat-poison, included); (ⅳ) pattern of intermingled dryness and blood stasis; (ⅴ) pattern of intermingled dryness and dampness. According to the patterns, principles of treatment and prescriptions are also on the same page. The formation and promotion of this consensus is of importance in the TCM course of diagnosis and treatment of Sjögren syndrome. [ABSTRACT FROM AUTHOR]

Qi Tang, Jiafen Liao, Xi Xie Department of Rheumatology and Immunology, the Second Xiangya Hospital, Central South University, Changsha, People’s Republic of ChinaCorrespondence: Xi Xie, Department of Rheumatology and Immunology, the Second Xiangya Hospital, Central South University, Changsha, 410011, People’s Republic of China, Tel/Fax +86 0731 8529 5255, Email Hsixie1997@csu.edu.cnAbstract: To strengthen the understanding of rheumatic diseases (RDs) as the most common underlying conditions associated with acquired hemophilia (AH), a potentially fatal bleeding condition due to the development of autoantibodies or inhibitors to coagulation factor VIII, and rarely to factor IX, here we presented two cases of RDs associated AH to elucidate the disease progression, treatment, and prognosis. The presented 2 cases showed good responses to glucocorticoid (GC) and immunosuppressive agents. And then, a case-based systematic review was conducted to better understand the clinically practiced diagnosis and treatment of RDs associated AH. A total of 14 articles were included in the final literature review. All the identified 14 patients with underlying RDs and AH presented with bleeding symptoms, increased APTT, decreased FVIII activity, and positive FVIII inhibitors. Twelve of the 14 patients (85.7%) started an eradication of autoantibodies treatment with GC and immunosuppressive agents. Among which six patients achieved partial or complete remission, and four patients (28.6%) switched to Rituximab and responded well. Nine of the 14 patients received hemostasis therapy, including recombinant human FVIIa (rFVIIa). Two patients (14.3%) died due to mass bleeding and key organ failure. AH should be highly suspected in patients with RDs presenting spontaneous mucocutaneous or internal bleeding and an isolated prolonged APTT. Given the high morbidity of AH, it is important to facilitate efficient and proper management.Keywords: acquired hemophilia, systemic lupus erythematosus, Sjogren syndrome, rheumatoid arthritis, connective tissue disease

Abstract Background We aimed to assess the efficacy of the injections of platelet rich plasma (PRP) for the treatment of severe dry eye disease (DED). Results In this retrospecitve interventional clinincal study, we included 28 eyes of 14 patients with severe DED who were diagnosed with Sjogren syndrome. Each patient received unilateral lacrimal gland injection of PRP at days 0, 30, 60 and 90 days while the other eye served as control group who received preservative free eye drops. We objectively assessed parameters at baseline, 1 month, 2 months and 3 months including ocular surface analyzer (OSA) namely; noninvasive tear breakup times (NIBUT), tear meniscus height (TMH), lipid layer thickness (LLT) in addition to the Schirmer test I, corneal fluorescein staining (CFS) and meiboscore. The mean age was 43.4 ± .7.85 years. Comparing different parameters, baseline data showed non-significant difference between injected eye group and control group. After 1 and 3 months of follow up, there were significant differences between both groups regarding NITBUT, TMH, LLT, CFS and Schirmer test, with p

Objective: To explore the effects of Huoxue Jiedu Formula on the expression of the apoptosis-related factors B-cell lymphoma-2 (Bcl-2) and survivin in submaxillary gland cells in mice with Sjögren syndrome (SS). Methods: Using 15 healthy C57BL/6J female mice as the blank control group. Seventy-five of non-obese diabetes mellitus female mice were classified as SS model. They were randomly assigned to the model group, total glucosides of paeony capsule group (TGP group, 0.233 g/kg), Huoxue Jiedu Formula low- (15.925 g/kg), middle- (31.850 g/kg), and high-dose (62.335 g/kg) groups, with 15 mice in each group. The blank control and model groups received distilled water by gavage, and the other groups received respective drug doses by gavage once a day for 8 consecutive weeks. After 1, 2, 4, 6, and 8 weeks of gavage, the salivary secretion volume of the mice was measured. The submaxillary gland index was calculated. Pathological changes in the submaxillary gland of mice were observed by hematoxylin-eosin staining. The expression of apoptosis-related factors Bcl-2 and survivin was detected by Western blotting and real-time fluorescence PCR. Results: There was statistical significance in salivary secretion volume from aspects of between-group, time-point, and interaction effects (P<0.05). After 8 weeks of treatment, compared to the blank control group, salivary secretion volume, submaxillary gland index, and protein and mRNA expressions of Bcl-2 and survivin in the model group were decreased, while the pathological score grading increased (P<0.05). Compared to the model group, the TGP group and the Huoxue Jiedu Formula low-, medium-, and high-dose groups showed an increase in saliva secretion after 8 weeks of treatment and an increase in submaxillary gland index, Bcl-2, and survivin mRNA expression levels, and a decrease in pathological score levels. The protein expressions of Bcl-2 and survivin in the Huoxue Jiedu Formula medium- and high-dose groups were increased (P<0.05). Compared to the TGP group, salivary secretion volume and submaxillary gland index in the Huoxue Jiedu Formula low-dose group were lower at weeks 4 and 6, while the pathological score grading was higher (P<0.05). The pathological score grading in the Huoxue Jiedu Formula high-dose group was lower. The protein and mRNA expressions of Bcl-2 in the Huoxue Jiedu Formula low- and medium-dose groups were lower (P<0.05). Conclusion: Huoxue Jiedu Formula may relieve the submaxillary gland lesions and increase the salivary secretion volume of the submaxillary gland in SS model mice by regulating the expressions of apoptosis-related factors such as Bcl-2 and survivin in the submaxillary gland. [ABSTRACT FROM AUTHOR]

Yu-Hsiang Chiu,1,2 Chen-Chih Chu,1 Chun-Chi Lu,1 Feng-Cheng Liu,1 Shin-En Tang,3 Shi-Jye Chu,1 San-Yuan Kuo,1 Hsiang-Cheng Chen1 1Division of Rheumatology/Immunology/Allergies, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan; 2Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, Utrecht, the Netherlands; 3Division of Thoracic Medicine, Department of Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, TaiwanCorrespondence: Hsiang-Cheng Chen, Division of Rheumatology/Immunology/Allergy, Department of Medicine, Tri-Service General Hospital, National Defense Medical Center, No. 325, Sec, 2, Cheng-Gong Road, Neihu 114, Taipei, Taiwan, R.O.C, Tel +886 2 87927135, Fax +886 2 87927136, Email hccheng@mail.ndmctsgh.edu.twObjective: Krebs von den Lungen-6 (KL-6) is expressed on regenerating type II pneumocytes and has been recognized as biomarkers in interstitial lung disease (ILD). We aim to identify the role of the serum KL-6 level in patients with newly diagnosed Sjögren syndrome (SS), as well as the correlation between the immunoassays.Methods: Patients with newly diagnosed SS and receiving HRCT for clinical reason during follow-up were included. Baseline KL-6 level was measured via enzyme-linked immunosorbent assay (ELISA) and latex particle-enhanced turbidimetric immunoassay (LETIA).Results: Of the 39 patients, 21 (53.85%) developed interstitial lung disease (ILD) by the conclusion of the follow-up period. The median time to diagnosis of ILD was 2.24 years (IQR 1.15– 4.34) in the ILD group. The median serum KL-6 level, measured using ELISA, was 1232 U/mL (IQR 937– 2242) and 764.5 U/mL (IQR 503.25– 1035.75) in the ILD group and the non-ILD group, respectively (p = 0.001). The median LETIA for serum KL-6 was 329 U/mL (IQR 235– 619) and 245 U/mL (IQR 215.25– 291) in the ILD group and the non-ILD group, respectively (p = 0.074).Conclusion: Serum KL-6 levels were higher in newly diagnosed SS patients with ILD diagnosis during follow-up. Thus, the serum KL-6 level can serve as a valuable biomarker to identify hidden ILD in patients with newly diagnosed SS patients. However, the immunoassay procedure may influence the efficacy of the prediction and its clinical association.Keywords: Sjögren syndrome, interstitial lung disease, KL-6

Primary Sjögren syndrome (pSS) is a systemic autoimmune inflammatory disease. Up to now, the role of regulatory T cells (Tregs) and their subgroups in pSS is still in controversial. In this study we tried to elucidate the roles of Tregs and its subgroups in pSS. Total 43 pSS patients and 23 health persons as control were enrolled in this study. We grouped the pSS patients according to the anti-SSa/SSb and the EULAR Sjögren's syndrome disease activity index (ESSDAI). Among the 43 pSS patients, 14 patients were followed after treatment. The percentage of rTregs (resting Treg cells) among Tregs was increased in the pSS group, and decreased after treatment. In the high disease activity subpopulation (ESSDAI ≥ 5), the percentage of rTregs among Tregs decreased after treatment. On the contrary, the percentage of aTregs (activated Treg cells) increased after treatment. It was in an inverse correlation between the percentage of aTreg and rTreg in pSS patients. The Tregs are co-cultured with responder T cells. Tregs from pSS patients showed poorer proliferation inhibitory function. Our results show that the percentages of Tregs and their subgroups altered in pSS patients. The percentage of aTreg and the percentage of rTreg have an inverse correlation in pSS patients. Compared to the control group, the percentage of rTregs among Tregs was increased in the pSS patients and decreased after the treatment. Our study also showed that The Tregs from pSS patients may have poorer inhibitory functions.

In sicca syndrome patients the xerostomia, xerophthalmia and the serological findings may strongly suggest the autoimmune Sjögren’s syndrome, but the histological findings in the labial salivary gland biopsies do not always justify the suspected diagnosis. The aim of this study was to compare the histomorphological changes and the clinical findings in patients with pathologically established Sjögren’s syndrome and in cases with negative histology. A total of 133 labial biopsies have been retrospectively evaluated from 2015 to May 2022, and the characteristic Sjögren’s lesions were found in 67 cases. According to the clinical data, 34 cases proved to be primary, and 33 were associated (“secondary”) forms. In 66 cases, the histology did not justify Sjögren’s syndrome; a significant acinar loss, fibrolipomatous infiltration, and mild sialadenitis had led to the clinical symptoms. In Sjögren’s histologies, the acinar loss was detected in just 31.8% of cases, which might indicate that the diminished saliva production represents immune-mediated hypofunction rather than direct damage of the acini. This is the first systemic study in Hungary investigating the correlation between pathological alterations and clinical findings.

Introduction: Sjögren syndrome (SS) is an autoimmune disease characterized by salivary gland (SG) destruction leading to loss of secretory function. A hallmark of the disease is the presence of focal lymphocyte infiltration in SGs, which is predominantly composed of T cells. Currently, there are no effective therapies for SS. Recently, we demonstrated that a newly developed therapy using effective-mononuclear cells (E-MNCs) improved the function of radiation-injured SGs due to anti-inflammatory and regenerative effects. In this study, we investigated whether E-MNCs could ameliorate disease development in non-obese diabetic (NOD) mice as a model for primary SS.Methods: E-MNCs were obtained from peripheral blood mononuclear cells (PBMNCs) cultured for 7 days in serum-free medium supplemented with five specific recombinant proteins (5G culture). The anti-inflammatory characteristics of E-MNCs were then analyzed using a co-culture system with CD3/CD28-stimulated PBMNCs. To evaluate the therapeutic efficacy of E-MNCs against SS onset, E-MNCs were transplanted into SGs of NOD mice. Subsequently, saliva secretion, histological, and gene expression analyses of harvested SG were performed to investigate if E-MNCs therapy delays disease development.Results: First, we characterized that both human and mouse E-MNCs exhibited induction of CD11b/CD206-positive cells (M2 macrophages) and that human E-MNCs could inhibit inflammatory gene expressions in CD3/CD28- stimulated PBMNCs. Further analyses revealed that Msr1-and galectin3-positive macrophages (immunomodulatory M2c phenotype) were specifically induced in E-MNCs of both NOD and MHC class I-matched mice. Transplanted E-MNCs induced M2 macrophages and reduced the expression of T cell-derived chemokine-related and inflammatory genes in SG tissue of NOD mice at SS-onset. Then, E-MNCs suppressed the infiltration of CD4-positive T cells and facilitated the maintenance of saliva secretion for up to 12 weeks after E-MNC administration.Discussion: Thus, the immunomodulatory actions of E-MNCs could be part of a therapeutic strategy targeting the early stage of primary SS.

PurposeTo 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.MethodsParticipants 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).ResultsEleven 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).ConclusionsData 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.

Sjögren syndrome (SS) is a multisystem autoimmune disease, primarily targeting salivary and lacrimal glands; skin, nasal and vaginal dryness, along with musculoskeletal pain and fatigue are the most commonly reported symptoms. Hearing loss is hypothesized to be frequent as well. The purpose of this systematic review was to estimate the prevalence of Hearing loss and its different subtypes in patients with Sjögren syndrome. PRISMA guidelines were followed to ensure highest quality for our systematic review. A random effects model meta-analysis and meta-regression was conducted using I2 as heterogeneity indicator. Eleven observational studies were included in this systematic review. Ten of them were cross-sectional, while one study was case–control. Studies were assessed for risk of bias: all were rated to a moderate level, except for two rated to a low level. Pooled prevalence of any type of hearing loss was 52.2%. After excluding studies rated to moderate bias, the pooled prevalence of hearing loss was 36.7%. We also conducted a subgroup analysis depending on type of hearing loss. Pooled prevalence of sensorineural hearing loss was 42.6%., while pooled prevalence of conductive hearing loss and mixed hearing loss were 5% and 2.3%, respectively. Meta-regression was conducted in an effort to identify possible variables capable to explain high heterogeneity between studies. Sample size and year of study were separately found to account for a portion of heterogeneity between studies of sensorineural hearing loss. Year of study was also found to account for a portion of heterogeneity between studies of conductive hearing loss. In conclusion, sensorineural hearing loss, is highly prevalent in patients with Sjögren syndrome. On this basis, early screening and follow-up of patients with Sjögren syndrome by pure tone audiometry is important. [ABSTRACT FROM AUTHOR]

10% of rheumatoid arthritis (RA) cases are associated to so-called secondary Sjögren's syndrome (SS). These RA cases have higher DAS, fewer remissions. Is this linked to a poor response to DMARDs (disease-modifying anti-rheumatic drugs)? No study has addressed this question to date. Does the association between secondary Sjögren's syndrome (SS) and rheumatoid arthritis (RA) affect the therapeutic response to DMARDs and long-term prognosis? We conducted a retrospective case–control study: 39 RA associated with SS was (anti-SSA antibodies and/or Chisolm stage III or IV) were compared to 39 isolated cases of erosive RA matched by age, duration of progression and gender. The DAS CRP was higher in the RA + SS group in patients with disease progression of 16 years: 2.6 (1.5–4.5) compared to the RA group: 1.6 (1.3–2.8) (p = 0.0001) while fewer patients were in remission: 61 vs. 92% (p = 0.002). A higher number of B DMARDs have been prescribed: RA + SS = 3.04 (1–7); RA = 1.7 (1–5) (p = 0.004). Anti-TNFs are less effective when RA is associated with SS: 30 vs. 70%. Conversely, Rituximab is more effective when RA is associated with SS: 80 vs. 30%. Erosive RA-related SS exacerbates the clinical course of the condition: higher DAS, fewer remissions. This is linked to reduced treatment efficacy: higher number of DMARDs prescribed, reduced efficacy of anti-TNF drugs. RA-related SS could modify sensitivity to biotherapies: lower percentage of remissions and resistance to anti-TNF drugs. [ABSTRACT FROM AUTHOR]

Objectives: Sjögren syndrome (SS) features salivary gland architectural changes such as ductal deformities and fat deposition secondary to inflammatory cell infiltrates; however, the anatomical and pathophysiological correlations of SS remain to be determined. This study aimed to determine the correlations of imaging findings based on the magnetic resonance (MR) sialography with the clinical and laboratory aspects of SS patients. Methods: We evaluated the MR sialographic findings of two pairs of parotid glands (PGs) and submandibular glands (SMGs) from 41 SS patients. The distinct MR sialographic features were then compared with the clinical symptoms, biological (anti‐SSA/Ro antibody positivity), histological (labial gland focus score), and functional (salivary flow and scintigraphy) data. Results: Ductal deformities such as sialectasis in the PG ducts were exclusively observed in SS, and fat replacement in SMGs was more distinct in patients with SS than in non‐SS sicca patients. Of the 82 PGs and SMGs from 41 SS patients, the grade of sialectasis in PGs on MR sialographic images and fat stage in SMGs showed strong correlations with anti‐SSA/Ro‐positivity. PG sialectasis was found more frequently in patients with dry mouth and decreased salivary gland function. A significant correlation was found between fat deposition in PGs and SMGs and decreased salivary gland function. Conclusion: These results suggest that MR sialography is a reliable, adjunctive anatomical and functional study to assess SS. Detail aspects of sialectasis and fat accumulation encountered on MR sialography in SS patients can help physicians assume the disease status in SS. Level of Evidence: 3 Laryngoscope, 133:307–316, 2023 [ABSTRACT FROM AUTHOR]

Sjögren's syndrome is a chronic and insidious autoimmune disease characterized by lymphocyte infiltration of exocrine glands. Patients typically present with dry eye, dry mouth, and other systemic manifestations. Currently, the available molecules and drug-delivery systems for the treatment of Sjögren's syndrome dry eye (SSDE) have limited efficacy since they are not specific to SSDE but to dry eye disease (DED) in general. The current treatment modalities are based on a trial-and-error approach using primarily topical agents. However, this approach gives time for the vicious cycle of DED to develop which eventually causes permanent damage to the lacrimal functional unit. Thus, there is a need for more individualized, specific, and effective treatment modalities for SSDE. The purpose of this article is to describe the current conventional SSDE treatment modalities and to expose new advances in ocular drug delivery for treating SSDE. A literature review of the pre-clinical and clinical studies published between 2016 and 2022 was conducted. Our current understanding of SSDE pathophysiology combined with advances in ocular drug delivery and novel therapeutics will allow the translation of innovative molecular therapeutics from the bench to the bedside. [ABSTRACT FROM AUTHOR]

Dry eye disease (DED) is one of the most common eye diseases caused by multiple factors. Rebamipide, which is currently used to treat peptic ulcer disease, was shown to enhance secretory function and modulate inflammation in animal disease models. Considering the pathophysiology of DED, SA001 was developed expecting enhanced systemic exposure of rebamipide. Clinical trials to evaluate the safety, tolerability and pharmacokinetic (PK) characteristics of SA001 and its active metabolite rebamipide were conducted. After oral administration of SA001, blood and urine samples were collected for PK analysis of SA001 and rebamipide. PK parameters were compared between SA001 and conventional rebamipide (Bamedin®) and also between fasted and fed. Safety and tolerability were evaluated throughout the study based on adverse events (AEs), physical examinations, vital signs, 12-lead electrocardiography and clinical laboratory tests. SA001 was rapidly absorbed and quickly converted to rebamipide. The systemic exposure of rebamipide was dose-proportional after single and multiple doses. The plasma concentration of rebamipide after administration of SA001 was higher with a dose adjusted AUClast and Cmax 2.20 and 5.45 times higher in the 240 mg dose group and 4.73 and 11.94 times higher in the 600 mg dose group compared to conventional rebamipide. The favorable PK and tolerability profiles support further clinical development. [ABSTRACT FROM AUTHOR]

BACKGROUND: Amyloidosis is an uncommon disorder characterized by the extracellular deposition of amorphous and insoluble proteins in an abnormal fibrillary configuration. Mammary amyloidosis is an unusual and easily overlooked diagnosis with an ambivalent presentation that could mimic breast cancer. CASE REPORT: We here report the case of 60-year-old Caucasian woman who presented to our surgical oncology department for clinically and radiologically suspicious breast mass. A fine needle biopsy was irrelevant, so we performed a lumpectomy. Final histology revealed an amyloid deposit and further workup was consistent with nodular cutaneous and breast AL amyloidosis associated with Sjögren's syndrome. CONCLUSION: Although rare, an awareness of the clinicopathologic characteristics of this easily overlooked entity is of great importance for breast surgeons. [ABSTRACT FROM AUTHOR]

Background and aims: RFC1-CANVAS and primary Sjögren syndrome (pSS) are among the most frequent causes of sensory ganglionopathy (SG) and can present simultaneously in a given patient, sharing confounding signs and symptoms. We describe the clinical characteristics of patients with SG due to CANVAS who were suspected of having or had received a previous diagnosis of pSS. Methods: Patients with SG and a genetically confirmed RFC1-CANVAS followed in our centre were ascertained and their personal history of pSS was collected. Results: Among the 71 patients with CANVAS, six patients (all females) had been diagnosed with pSS by their clinicians. In these six patients, the mean age at onset of the SG was 61 years, four patients describing gait instability and positive sensory symptoms, one only gait instability and one only positive sensory symptoms. Five patients had a history of chronic cough; signs at examination included a cerebellar syndrome (n = 3), vestibulopathy (n = 2), pes cavus (n = 3) and dysautonomia (n = 2). On neurophysiological examination, SG was generally severe and symmetrical. All patients presented a sicca syndrome, but only four patients had a confirmed pSS as per 2016 ACR-EULAR classification criteria. Four patients were treated but did not respond to immunosuppressive therapy, one suffering from side effects. Interpretation: The suspicion or diagnosis of pSS in a patient with SG should not exclude RFC1 expansion analysis, as a CANVAS diagnosis may dissuade the clinician from initiating or continuing immunosuppressive therapy, especially in unconfirmed pSS patients. Importantly, a sicca syndrome may lead to pSS misdiagnosis. [ABSTRACT FROM AUTHOR]

Sjogren syndrome is a slowly progressive autoimmune disease with lymphocytic infiltration of exocrine glands, peri-epithelial and extra-epithelial tissues. In light of better diagnostic modalities and improved treatment options, patients with Sjogren syndrome are now commonly encountered by anaesthetist for various surgeries. Here, we would like to describe an anaesthetic management of a parturient with bad obstetric history who was diagnosed with Sjogren syndrome and was planned for caesarean delivery. This article also reviewed anaesthetic problems faced due to inherent disease pathology and concurrent alterations in treatment modalities. [ABSTRACT FROM AUTHOR]

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The diagnosis and monitoring of Sjögren syndrome (SS) is often difficult, requiring a multidisciplinary approach with invasive procedures. Our aim is to elucidate the tear protein alterations of dry eye disease (DED) with primary SS (pSS) and secondary SS (sSS) with the long-term instillation of eyedrops. We collected clinical demographics and tear fluid (TF) samples from DED patients with no autoimmune diseases (non-SS-DED), pSS-DED, and sSS-DED patients, followed by TF screening with tandem mass tagging-labeling gel-free proteomics assay. Bioinformatic analysis via Ingenuity Pathway Analysis was used to identify functional pathways and interacting networks. Validation of candidate proteins with enzyme-linked immunosorbent assay on the tear samples was done. The top functional pathways of the two comparisons (sSS-DED vs. pSS-DED and sSS-DED vs. non-SS-DED) were both associated with inflammation and stress-related signaling. After constructing an interaction network model with the selected candidate proteins, five proteins were identified. A Disintegrin and Metalloproteinase domain-containing protein 10 (ADAM10) was found to be an important candidate biomarker in all groups, followed by epidermal growth factor (EGF) in TF. This study revealed novel DED markers, ADAM10 and EGF, in differentiating between primary and secondary SS patients from tears by in-depth proteomic analysis. [ABSTRACT FROM AUTHOR]

Hydroxychloroquine (HCQ) therapy decreased immunoglobulin (Ig) levels in patients with Sjögren syndrome (SS) and rheumatoid arthritis (RA) in previous studies. We found no report of Ig levels of women with IgG subclass deficiency (IgGSD) and systemic lupus erythematosus (SLE), SS, or RA treated with HCQ. We retrospectively evaluated IgG, IgG subclass, IgA, and IgM levels and other characteristics of women at IgGSD diagnosis who did and did not take HCQ for SLE, SS, or RA. There were 132 women (48 subnormal IgG1 only, 49 combined subnormal IgG1/IgG3, and 35 subnormal IgG3 only). Mean age was 49 ± 13 years. Twenty-two women with SLE, SS, RA, or combination thereof reported HCQ ≥ 200 mg/day ≥ 6 months. In each IgGSD subtype, median Ig levels of women who took HCQ were not significantly lower than those of women who did not take HCQ. Women with combined subnormal IgG1/IgG3 who took HCQ had greater median IgG2 than women who did not take HCQ (4.89 g/L (range 4.43, 4.94) vs. 2.57 g/L (1.21, 6.44), respectively; p = 0.0123). Regressions on IgG1, IgG2, and IgG3 revealed positive associations with HCQ therapy (p = 0.0043, 0.0037, and 0.0139, respectively). There were no significant Ig associations with age, SLE, SS, or RA as independent variables. HCQ therapy of SLE, SS, or RA in women with IgGSD was not associated with significantly lower IgG, IgG subclass, IgA, or IgM levels. IgG1, IgG2, and IgG3 were positively associated with HCQ therapy, after adjustment for other variables. [ABSTRACT FROM AUTHOR]

Objective: We examined the epidemiology of parotid gland lymphomas (PGL), the incidence, survival rates, clinical features, and association with primary Sjögren's syndrome (pSS)., Methods: This retrospective nationwide cohort study analyzed data from Danish patients diagnosed with PGL between 2000 and 2020. Data were collected from medical records, the National Pathology Register, and the Danish lymphoma database. Statistical analyses included Kaplan-Meier curves, log-rank tests, and Cox proportional hazards models., Results: A total of 433 patients were included. The incidence rate was 0.39 per 100,000 person-years, with PGL constituting 1.9% of all non-Hodgkins lymphoma in Denmark. The average annual incidence was 2.7% (incidence rate ratio = 1.027, p < 0.01). Follicular lymphoma (FL) was the most common subtype with 154 cases (35.6%), followed by large B-cell lymphoma (LBCL) with 119 cases (27.5%), and extranodal marginal zone lymphoma (EMZL) with 84 cases (19.4%). The median overall survival (OS) for FL was 9.5 years (95% CI 6.9-10.2), with 5-year and 10-year OS rates of 70% and 44%, respectively. For LBCL, the median OS was 7.8 years (95% CI 5.0-8.8), with 5-year and 10-year OS rates of 59% and 33%. EMZL had a median OS of 12.8 years (95% CI 9.0-16.3), with 5-year and 10-year OS rate of 83% and 55%. EMZL was significantly associated with pSS, relative risk 21.97 (95% CI 2.81-171.53). Advanced age, B symptoms, and elevated LDH levels were significantly linked to poorer overall survival., Conclusion: This study offers new epidemiological, clinical, and prognostic insights, with a focus on their association with pSS., Level of Evidence: 3 Laryngoscope, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

Sjögren Syndrome (SS) is a chronic inflammatory autoimmune disease characterized by lymphocytic infiltration of exocrine glands. This study, based on bioinformatics predictions, investigates the biological functions of ubiquitin specific peptidase 18 (USP18) and notch receptor 1 (NOTCH1) in T helper 17 (Th17) and regulatory T (Treg) cell imbalance and B cell activity in SS. USP18 and NOTCH1 were highly expressed in peripheral blood mononuclear cells (PBMCs) of SS patients and the PBMCs of NOD mice compared to the controls. Adenovirus-mediated knockdown of USP18 significantly enhanced the salivary flow rate of NOD mice while reducing lymphocyte infiltration in mouse salivary ligand tissues. In addition, it decreased the proportions of Th17 cells while increasing the proportions of Treg cells. USP18 enhanced NOTCH1 protein stability through de-ubiquitination modification. In the presence of USP18 knockdown, the NOTCH1 upregulation restored the predominance of Th17 cells in mice. In B cells isolated from PBMCs, the production of B cell autoantibodies was decreased by USP18 silencing but enhanced by NOTCH1 upregulation. In summary, this study demonstrates that USP18-mediated protein stabilization of NOTCH1 is correlated with Th17/Treg cell imbalance and B cell activity during SS development., Competing Interests: Declarations. Ethics approval: The use of human samples was approved by the Institutional Review Board of the Seventh Medical Center of Chinese PLA General Hospital and adhered to the Declaration of Helsinki. Each respondent signed an informed consent. The use of animals was approved by the Animal Ethics Committee of Seventh Medical Center of Chinese PLA General Hospital and was performed in line with the Guide for the Care and Use of Laboratory. Conflict of interests: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Dr. Irwin M. Braverman has advanced our understanding of cutaneous manifestations of autoimmune connective tissue diseases (AI-CTD). Prior to the era of molecular diagnostics, Dr. Braverman's careful clinical observations and detailed descriptions of patients with AI-CTD in his textbook Skin Signs of Systemic Disease provided insights into disease characterization, progression, and evolution. Patients from Dr. Braverman's career who did not fulfill criteria for a single clinical entity, but rather were diagnosed with undifferentiated connective tissue disease or mixed connective tissue disease, are discussed. In addition, key observations of Dr. Braverman into the cutaneous manifestations of Sjögren syndrome and the systemic complications of urticarial vasculitis are highlighted., Competing Interests: Declaration of competing interest Matthew D. Vesely, MD, PhD – none, (Copyright © 2024. Published by Elsevier Inc.)

Objectives: To evaluate the impact of thyroid diseases (TD) on the comorbidities incidence and immune system of patients with primary Sjögren's syndrome (pSS)., Methods: A total of 329 patients diagnosed with pSS who were admitted between January 2018 and September 2023 were evaluated. The patients were divided into two groups: those with and without TD. Clinical data at the onset of SS were recorded. Kaplan-Meier method and Cox proportional hazards model were utilized for survival analysis. Flow cytometry and immunofluorescence measurements were used to analyse the lymphocyte subtypes., Results: Of the 329 patients, 109 had TD. The most common types of TD were thyroid nodules (40.4%) and autoimmune thyroid diseases (AITD) (28.4%). Patients with TD exhibited a significantly elevated incidence risk of malignancy (14.7% vs 6.4%, p= 0.01) and osteoporosis (OP) (23.9% vs 14.1%, p= 0.03). TD comorbidity was an independent risk factor of malignancy (HR 4.7, 95% CI 1.1-19.3, p= 0.03) and OP (HR 3.7, 95% CI 1.3-10.2, p= 0.01). Patients with SS and TD exhibited a higher ratio of PD1+ subsets of total CD3+ T cells, Th and Tc-cells (all p= 0.03), as well as a lower ratio of unswitched memory B-cells (p= 0.01) in peripheral blood. The ratio of PD1+ subsets of Th-cells in salivary glands also exhibited a significantly increase in these patients compared with those without TD (p= 0.03) and the controls (p< 0.01)., Conclusion: The incidence risk of malignancy and OP was significantly elevated in pSS patients with TD. The significant elevated proportions of PD1+ T cells may influence the occurrence of this process., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Introduction: Evidence has demonstrated that autoimmune diseases tend to coexist at a higher rate than expected, reflecting a common pathogenic pathway. In this study, we investigate the co-occurrence of systemic sclerosis, systemic lupus erythematosus, and Sjogren syndrome in patients with type 1 and type 2 diabetes mellitus., Methods: Our data were obtained using the 2019 Healthcare Cost and Utilization Project, and International Classification of Diseases, 10th Revision diagnosis codes were used to identify patients with systemic sclerosis, systemic lupus erythematosus, lupus nephritis, and Sjogren syndrome, as well as patients with type 1 and type 2 diabetes. We utilized Statistical Analysis System 9.4 for all analyses and included designated weight values to produce nationally representative estimates., Results: The prevalence of systemic sclerosis among patients with type 1 diabetes mellitus and type 2 diabetes mellitus was significantly lower than that for the non-diabetes mellitus control group (0.0007% vs 0.09%, p-value = 0.0064 and 0.01% vs 0.07%, p-value < 0.0001), respectively. Similarly, there was a significant decrease in the prevalence of systemic sclerosis with lung involvement in patients with type 1 and type 2 diabetes mellitus, with a statically significant difference in type 2 diabetes mellitus versus nondiabetic group (0.001% vs 0.006%, p-value < 0.0001). We noted a similar pattern regarding the prevalence of systemic lupus erythematosus and lupus nephritis in patients with type 1 and 2 diabetes. Similarly, there was a significant decrease in the prevalence of Sjogren syndrome in patients with type 1 diabetes and type 2 diabetes., Conclusion: The collected data demonstrates an inverse relation between some autoimmune connective tissue diseases and diabetes. This suggests that these diseases and diabetes mellitus may have different immune pathogenesis. There was also a significantly lower incidence of organ complications such as lupus nephritis and systemic sclerosis lung disease among patients with diabetes, suggesting that diabetes and treatment of diabetes may alter the clinical expression of these disorders., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Inflammatory sensory neuronopathies are rare disorders mediated by dysimmune mechanisms targeting sensory neurons in the dorsal root ganglia. They constitute a heterogeneous group of disorders with acute, subacute, or chronic courses, and occur with cancer, systemic autoimmune diseases, notably Sjögren syndrome, and viral infections but a noticeable proportion of them remains isolated. Identifying inflammatory sensory neuronopathies is crucial because they have the potential to be stabilized or even to improve with immunomodulatory or immunosuppressant treatments provided that the treatment is applied at an early stage of the disease, before a definitive degeneration of neurons. Biomarkers, and notably antibodies, are crucial for this early identification, which is the first step to develop therapeutic trials., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Sjögren's Syndrome (SS) is an autoimmune inflammatory disease, characterized by lymphocytic infiltration of exocrine glands. Approximately 10% of patients with SS have pulmonary involvement as the first manifestation of their disease, the most common being non-specific interstitial pneumonia.We present the case of a 51-year-old man with organizing pneumonia as the presenting feature of primary SS.Pulmonary involvement as the presenting feature of SS is uncommon, especially when the pattern on CT-scan is that of organized pneumonia. Initial management includes steroids and other immunosuppressants agents, with a better response in organized pneumonia secondary SS cases.

IntroductionPrimary Sjögren's syndrome (pSS) is associated with an increased prevalence of traditional risk factors and cardiovascular diseases (CVDs). The study aimed to identify specific risk factors for CVD in pSS patients.MethodsPSS patients with and without CVD were compared. All patients fulfilled the EULAR/ACR classification criteria. Patients with CVD presented at least one of the following manifestations: myocardial infarction, transient ischemic attacks, ischemic or hemorrhagic stroke, peripheral artery disease, coronary artery disease, and carotid plaques. Data were collected by a standardized protocol and review of medical records.Results61/312 (19.6%) pSS patients presented with CVD. Traditional risk factors such as hypertension, hypercholesterinemia and diabetes (p < 0.05), pSS manifestations, in particular vasculitis (p = 0.033) and Raynaud's phenomenon (p = 0.018) were associated with CVD. Among patients with ischemic events (28/312, 9%), particularly cerebrovascular disease (n = 12/28, 42.9%), correlations with increased EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) (p = 0.039) and EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) (p = 0.048) were observed. Age at first cerebrovascular event was 55.2 [48.9–69.6] years. Multivariate analysis confirmed hypertension [odds ratio (OR) 3.7, 95% confidence interval (CI) 1.87–7.18, p < 0.001], hypercholesterinemia (OR 3.1, 95% CI 1.63–5.72, p < 0.001), male gender (OR 0.4, 95% CI 0.17–0.78, p = 0.009), Raynaud's phenomenon (OR 2.5, 95% CI 1.28–4.82, p = 0.007), and CNS involvement (OR 2.7, 95% CI 1.00–7.15, p = 0.048) as independent CVD predictors.ConclusionRaynaud's phenomen as well as vasculitis and high ESSDAI have shown a significant association to CVD. PSS patients with cerebrovascular events were younger than expected. Knowledge about risk factors may help clinicians to identify pSS patients at risk for CVD. After diagnosis of pSS, patients should be screened for risk factors such as hypertension and receive appropriate therapy to prevent or at least reduce sequelae such as infarction. However, further investigations are necessary in order to achieve a reliable risk stratification for these patients.

The relationship between Sjögren syndrome (SS) and T-cell large granular lymphocytic (T-LGL) leukemia remains unclear. In this paper, we report for the first time a large case series of 21 patients with primary and secondary SS associated with T-LGL leukemia. Our results suggest the importance of considering T-LGL leukemia in the diagnostic evaluation of SS patients, particularly when neutropenia occurs. We also postulate that elevated antinuclear antibody titers in patients with T-LGL leukemia indicate the need for the clinical assessment of SS. To assess whether SS affects the frequency of the signal transducer and activator of transcription 3 (STAT3) gene mutations in T-LGL leukemia, we examined STAT3 mutations by next-generation sequencing in two cohorts of patients: with SS-associated T-LGL leukemia and T-LGL leukemia in the setting of rheumatic diseases but without SS. While our results suggest that SS, per se, is not associated with an increased frequency of STAT3 mutations in T-LGL leukemia, further studies are needed to better assess the role of the STAT pathway in the development of concomitant SS and T-LGL leukemia. [ABSTRACT FROM AUTHOR]

Objective: To investigate cardiovascular risk among diabetic patients with Sjögren syndrome. Methods: This study was a nationwide population‐based case‐control study from 1997 to 2013, in which the association between autoimmune diseases and diabetes was investigated. The study population consisted of individuals with newly diagnosed type 2 diabetes with macrovascular or microvascular complications with at least two outpatient visits or one hospitalization as the outcome variables, and the exposure variables included traditional risk factors, medications, and autoimmune diseases. The odds ratio of cardiovascular events among each prevalent autoimmune disease and hydroxychloroquine's effect on cardiovascular risk were analyzed. Results: The study included a total of 7026 individuals with diabetes with microvascular and macrovascular complications and the same number of patients in the control group. Sjögren syndrome was significantly higher in the diabetes complication group than in the non‐complication group (0.8% vs 0.5%, P = 0.036). By using multivariate analysis, we found hypertension, hyperlipidemia, and Sjögren syndrome to be three independent risk factors for diabetes vascular complications (odds ratio [OR] 1.96, 95% confidence interval [CI] 1.82‐2.10; OR 1.53, 95% CI 1.42‐1.64; and OR 1.67, 95% CI 1.06‐2.65; respectively, all P < 0.05). Treatment with traditional statins and aspirin might be able to overcome the increased risk of developing cardiovascular events while comparing between diabetes patients with and without Sjögren syndrome. Conclusion: Sjögren syndrome is an unrecognized independent risk factor for cardiovascular events among diabetes patients, which indicates that patients with diabetes combined with Sjögren syndrome require closer follow up regarding cardiovascular complications in clinical settings. Treatment with hydroxychloroquine might not be enough to lower the cardiovascular risk significantly in diabetes patients with Sjögren syndrome. [ABSTRACT FROM AUTHOR]

Background: Primary Sjögren Syndrome is a rare autoimmune systemic disease characterized by impaired secretory functions of the exocrine gland. One of the main clinical features is dry mouth and subsequent oral diseases, which are also found in patients with Sicca. This leads to a marked deterioration in the quality of life and the patient's search for information and solutions. Many patients turn to patients' associations that offer moments of sharing to their members, especially through online discussion forums. Today, these forums represent quality material for a sociological or biomedical analysis of patients' concerns, as close as possible to their daily lives. Our objective is to analyze the concerns of patients with SS or Sicca regarding their dry mouth especially dental care. Methods: In this cross-sectional observation study, a quantitative analysis of the Mouth-Nose online forum discussion of the French Association of Patients with Gougerot-Sjögren's Syndromes and Dryness have been performed. After reading and re-reading, initial request themes, topics, and subtopics were established and coding was performed. Then, the 885 threads were classified depending the initial request, pragma-linguistic indices and the main topic discussed in the thread. After identifying the threads dealing with dental care, we looked at which types of care were most discussed and classified the discussions according to whether or not the patient was satisfied with their care at the dentist. Results: The majority of the initial requests are posts for experiences sharing and/or advice. The topic of "dental care" is one of the main concerns of the forum users. Among the threads that concern dental care, requests to share experience with implants are in the majority. Finally, the majority of the posts on dental care relate to care in private dental practice, deals with dental implants and prevention and resulted mainly in patient satisfaction. Conclusions: Analysis of the forum reveals importance of patient concerns about prevention, and care costs due to implant treatment, which add to disease burden. Most of messages relate favorable experiences with their dentists, which is in line with the approach of sharing experiences and support characteristic of a forum. [ABSTRACT FROM AUTHOR]

In the last years, new insights into the molecular basis of rheumatic conditions have been described, which have generated particular interest in understanding the pathophysiology of these diseases, in which lies the explanation of the diversity of clinical presentation and the difficulty in diagnostic and therapeutic approaches. In this review, we focus on the new pathophysiological findings for Sjögren syndrome and on the derived new SPECT and PET radiopharmaceuticals to detect inflammation of immunological origin, focusing on their role in diagnosis, prognosis, and the evaluation of therapeutic efficacy. [ABSTRACT FROM AUTHOR]

Dry mouth is the main manifestation of Sjögren syndrome (SS). Quercetin has been reported to alleviate radiation‐induced salivary gland damage, yet the effect of quercetin on SS‐caused salivary gland damage remains unclear. This study aimed to investigate the effects of quercetin on SS‐induced salivary gland damage and the mechanism underlying its therapeutic potential in SS. Here, NOD/Ltj mice were used to spontaneously mimic SS‐induced salivary gland inflammation in vivo and salivary gland epithelial cells (SGECs) were stimulated by interferon‐γ (IFN‐γ) to mimic cell inflammation in vitro. Results showed that quercetin significantly reduced loss of saliva flow, salivary gland damage, cell apoptosis, and inflammatory response in NOD/Ltj mice. Quercetin treatment also significantly reduced the increased serum leptin (LP) levels in NOD/Ltj mice. Furthermore, quercetin blocked the increases in the expression of obesity receptor (OB‐R) and its downstream Janus kinase 2/signal transducer and activator of transcription 3 (JAK2/STAT3) signaling in the salivary glands. In vitro experiments confirmed that quercetin could protect SGECs from IFN‐γ‐induced cell apoptosis and inflammation through the LP/OB‐R‐activated JAK2/STAT3 signaling. Hence, quercetin might protect against SS‐induced salivary gland damage by relieving cell apoptosis and inflammation by inhibiting the LP/OB‐R signaling, providing a new perspective for treating SS‐induced dry mouth. [ABSTRACT FROM AUTHOR]

Sjögren syndrome (SS) is diagnosed based on invasive tissue biopsies and blood sampling. Therefore, a novel non-invasive and simple inspection diagnostic marker of SS is required. Here, we identified exosome-derived microRNAs (miRNAs) as biomarkers for SS using non-invasive mouthrinse samples collected from patients with SS and healthy volunteers. We compared miRNAs derived from exosomes in mouthrinse samples from the two groups using microarrays and real-time polymerase chain reaction (PCR) and identified 12 miRNAs as biomarker candidates. The expression ratios of four miRNAs were significantly increased in the SS group compared to the control group. Logistic regression analysis revealed a more significant influence of miR-1290 and let-7b-5p in the SS group than that in the control group. We combined these miRNAs to create a diagnostic prediction formula using logistic regression analysis. The combination of miR-1290 and let-7b-5p distinguished SS from the control samples with an AUC, sensitivity, specificity, positive predictive value, and negative predictive value of 0.856, 91.7%, 83.3%, 84.6%, and 90.9%, respectively. These results indicated that an increased ratio of these miRNAs could serve as a novel and non-invasive diagnostic marker for SS. This is the first report of diagnosis and screening of SS by adopting a non-invasive method using mouthrinse. [ABSTRACT FROM AUTHOR]

Objectives: The Xerostomia Inventory (XI) was developed to assess the severity of dryness in patients with xerostomia. It has a long and a short form with three- and five-point Guttman-type response options. In this study, we aimed to translate the XI into Turkish, to assess the validity and reliability of both response options in patients with head & neck cancer (HNC) or Sjögren syndrome (SS), and to select the optimal version for Turkish patients. Patients and methods: Between January 2019 and June 2019, the XI was translated into Turkish (XI-T) and applied to patients aged ≥18 years with HNC and SS. All patients were applied two tests including both the three- and five-point options. The internal consistency was assessed by Cronbach alpha and test-retest reliability by intraclass correlation coefficients (ICCs). Content validity was based on expert opinion and patient reviews. Results: A total of 186 patients (109 males, 77 females; median age: 54 years; range, 19 to 78 years) answered the XI-T. The number of patients with HNC and SS was 143 (77%) and 43 (23%), respectively. Median XI-T score was 17 for the three-point, and 24 for the five-point option, respectively. Overall internal consistency was satisfactory for both options (α=0.81 and α=0.89, respectively). Overall test-retest reliability was satisfactory and ICCs ranged between 0.71 and 0.92 for the three-point, and 0.36 and 0.94 for the five-point option, respectively. Assessments based on expert opinions and patient reviews also favored the content validity of the scale. Conclusion: The XI-T with both three- and five-point options is a valid and reliable tool to evaluate the presence and severity of dryness in patients with HNC and SS who experience xerostomia. The three-point option is more comprehensible and can be preferred over the five-point option in the Turkish population. [ABSTRACT FROM AUTHOR]

Distal renal tubular acidosis (DRTA) has been reported in association with autoimmune diseases, such as Sjögren's syndrome, systemic lupus erythematosus (SLE), autoimmune hepatitis, primary biliary cirrhosis, rheumatoid arthritis and autoimmune thyroiditis. Whether we talk about the complete or incomplete form of DRTA associated with autoimmune diseases, the real incidence is unknown because asymptomatic patients usually are not identified, and most of the reported cases are diagnosed due to severe symptoms secondary to hypokalemia, a frequent finding in these cases. The mechanisms involved in DRTA in patients with autoimmune diseases are far from being fully elucidated and most of the data has come from patients with Sjögren's syndrome. This review will present different hypotheses raised to explain this association. Also, aiming for a better understanding of the association between autoimmune diseases and DRTA, our review summarizes data from 37 case reports published in the last five years. We will emphasize data regarding clinical presentation, biological alterations, treatment and outcome. A very important question is whether immunosuppressive therapy is helpful in DRTA associated with autoimmune diseases. Because the pathology is rather rare, treatment is not standardized, and reported results are often contradictory. Corticosteroids are frequently used, but multiple other immunosuppressive drugs have been proposed and will be approached in this review. [ABSTRACT FROM AUTHOR]