A relatively rare traditional Chinese medicine pattern of primary Sjögren syndrome: A case report.

RATIONALE: This report presents a unique case of a patient diagnosed with Primary Sjögren's syndrome and a relatively rare traditional Chinese medicine pattern, known as the combined cold and heat pattern and cold-dampness syndrome. The patient's condition was successfully managed using Chinese herbal medicine, specifically the modified Da-Chai-Hu decoction and Linggui Zhugan decoction. PATIENT CONCERNS: A 56-year-old woman had chronic dry eye and mouth for over 10 years. She was initially managed with traditional Chinese herbal medicine (TCHM) prescriptions, including the Zengye decoction, but the therapeutic effects were unsatisfactory. As the disease progressed, she was diagnosed with an anxiety disorder due to symptoms of vexation and insomnia. Treatment with alprazolam and venlafaxine failed to alleviate these symptoms. Recently, her general condition gradually worsened, with symptoms including a bitter taste in her mouth, dizziness, hot flashes, chills, poor appetite, chest discomfort, and constipation. DIAGNOSES: After a series of examinations, including a Schirmer test and labial gland biopsy, she was diagnosed with Sjögren's syndrome. INTERVENTIONS: Despite regular treatment with pilocarpine, sodium hyaluronate eye drops, venlafaxine, and alprazolam, the dry mouth symptoms intensified. Consequently, she sought further intervention through the TCHM. OUTCOMES: After 8 weeks of treatment with the modified Da-Chai-Hu decoction and Linggui Zhugan decoction, she reported a significant improvement in her dryness-related symptoms and sleep quality. LESSONS: This case report demonstrates that TCHM can effectively treat Primary Sjögren's syndrome, and should be considered for broader applications. Furthermore, this underscores the importance of tailoring treatment formulas to patients by identifying their specific syndrome differentiation in a clinical setting.

Clinical significance of the expression levels of serum transforming growth factor-ß and CXC type chemokine ligand 13 in primary Sjogren's syndrome patients.

OBJECTIVE: The aim of this study was to investigate the expression levels of the serum transforming growth factor-ß1 (TGF-ß1) CXC type chemokine ligand 13 (CXCL13) in primary Sjogren's syndrome (pSS) patients and its correlation with disease severity. METHOD: Thirty patients with pSS admitted to Nanjing Traditional Chinese Medicine Affiliated Hospital of Nanjing University of Traditional Chinese Medicine from January 2021 to December 2022 were included as the pSS group, while 30 patients who underwent physical examination during the same period were included as the control group. The levels of TGF-ß1 and CXCL13 were detected. The diagnostic value of TGF-ß1 and CXCL13 for pSS was analyzed. Detection of serum TGF-ß1 and CXCL13 levels in pSS patients with different disease activities and lip gland pathological grading of pSS was done. We compared the correlation between TGF-ß1 and CXCL13 levels and disease activity and labial gland pathological grading in pSS patients. RESULT: The TGF-ß1 and CXCL13 levels in the pSS group were higher than those in the control group. The area under the receiver operating characteristic (ROC) curve (AUC) for TGF-ß1 and CXCL13 diagnosis of pSS was 0.790 (95% confidence interval (CI): 0.720~0.861) and 0.838 (95% CI: 0.778~0.898), respectively. The serum TGF-ß1 and CXCL13 levels of pSS patients significantly increase with the increase of disease activity and lip gland pathological grading. The TGF-ß1 and CXCL13 levels in pSS patients were positively correlated with disease activity and lip gland pathological grading. CONCLUSION: The levels of TGF-ß1 and CXCL13 in pSS patients were increased, and it was closely related to disease activity and lip gland pathological grading, which can be used as an effective indicator for the diagnosis of pSS. Key Points • The TGF-ß1 and CXCL13 levels in the pSS group were higher than those in the control group. • The TGF-ß1 and CXCL13 levels in pSS patients were positively correlated with disease activity and lip gland pathological grading. • TGF-ß1 and CXCL13 can be used as an effective indicator for the diagnosis of pSS.

Safety and Efficacy of Oral Hydroxychloroquine in the Treatment of Ophthalmic Disease Associated with Sjögren's Syndrome.

Background: Dry eye disease is common among patients with primary Sjögren's syndrome (pSS). Hydroxychloroquine (HCQ), known for its immunomodulatory effects and minimal adverse effects, has emerged as a pivotal treatment option for pSS. Nonetheless, conflicting evidence exists regarding the therapeutic efficacy of HCQ in managing dry eye disease associated with pSS. Objectives: To evaluate the safety and efficacy of oral hydroxychloroquine in treating dry eye disease associated with pSS. Methods: A prospective randomized controlled study was conducted, enrolling pSS patients with moderate to severe dry eye disease. Participants were randomly assigned to an oral HCQ group and an observation group. Various scales (ESSDAI, ESSPRI, OSDI, and SPEED questionnaire score), dry eye-related tests (OSS score, TBUT, and Schirmer test I), ophthalmology-specific tests (BCVA, SD-OCT RT, field of view, latency and amplitudes for multifocal ERG ring 1 and ring 2), whole body protein levels (serum IgA, IgG, and IgM), and blood glucose were assessed before and after 12 months of treatment. Results: Pairwise comparison of the observed indicator baseline revealed no statistical significance (P > .05). After 12 months, the HCQ group exhibited notable improvements in ESSPRI, serum IgA, and Schirmer test I results compared to the control group (P < .05). Both groups demonstrated significant improvements in BCVA, OSDI, SPEED scores, and dry eye-associated examinations compared to baseline (P < .05). Serum IgG and IgM levels decreased in the HCQ group after 12 months of treatment, but without statistical significance (P > .05). None cases of HCQ retinopathy were reported during follow-up. Conclusions: Oral HCQ was demonstrated safety and efficacy in managing pSS-related dry eye disease. Treatment with Oral HCQ markedly reduced the ESSPRI score, improve patients' systemic dryness symptoms, and greatly decreased blood IgA levels. Combined with topical cyclosporin, HCQ improved Schirmer test I scores and alleviated ocular surface inflammation and dry eye signs and symptoms.

Current evidence on diagnosis and treatment of parotid gland lymphomas: a systematic review.

BACKGROUND AND PURPOSE: Parotid gland lymphoma (PGL) is a rare and challenging diagnosis. Different lymphomas can develop in the parotid gland, with the most common being the mucosa-associated lymphoid tissue (MALT) lymphoma, which originates directly from the glandular parenchyma. Other histologic subtypes arise from both intraglandular and extraglandular parotid lymph nodes. A consensus on diagnosis and treatment of PGL is still lacking, and published data is scarce and heterogeneous. METHODS: We performed a systematic review of the literature, including studies published after 2001, when the WHO classification of lymphoid tumours was introduced. RESULTS: Twenty retrospective studies were included in the analyses, eight of which focused exclusively on MALT lymphomas. Final analysis included 612 cases of PGL, with a 1.68:1 F/M ratio. MALT lymphoma was the most common histology, followed by follicular and diffuse large B-cell lymphoma. Most cases were low stages (IE/IIE acc. Ann Arbour, 76.5%) and only 10% of patients presented with symptoms, most commonly pain (4.8%) and B symptoms (2.2%). A high prevalence of associated autoimmune diseases was found, particularly Sjögren's syndrome, that affected up to 70% of patients with MALT lymphoma. In most cases diagnosis was achieved through parotidectomy (57.5%), or open biopsy (31.2%). Treatment strategies were either surgical, non-surgical or a combination of modalities. Surgery as a single-modality treatment was reported in about 20% of patients, supposing it might be a valuable option for selected patients. CONCLUSIONS: Our review showed that the diagnosis and treatment of PGLs is far from being standardized and needs further, more homogeneous reports to reach consensus.

Biological therapy in systemic lupus erythematosus, antiphospholipid syndrome, and Sjögren's syndrome: evidence- and practice-based guidance.

Systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), and Sjögren's syndrome (SS) are heterogeneous autoimmune diseases. Severe manifestations and refractory/intolerance to conventional immunosuppressants demand other options, namely biological drugs, and small molecules. We aimed to define evidence and practice-based guidance for the off-label use of biologics in SLE, APS, and SS. Recommendations were made by an independent expert panel, following a comprehensive literature review and two consensus rounds. The panel included 17 internal medicine experts with recognized practice in autoimmune disease management. The literature review was systematic from 2014 until 2019 and later updated by cross-reference checking and experts' input until 2021. Preliminary recommendations were drafted by working groups for each disease. A revision meeting with all experts anticipated the consensus meeting held in June 2021. All experts voted (agree, disagree, neither agree nor disagree) during two rounds, and recommendations with at least 75% agreement were approved. A total of 32 final recommendations (20 for SLE treatment, 5 for APS, and 7 for SS) were approved by the experts. These recommendations consider organ involvement, manifestations, severity, and response to previous treatments. In these three autoimmune diseases, most recommendations refer to rituximab, which aligns with the higher number of studies and clinical experience with this biological agent. Belimumab sequential treatment after rituximab may also be used in severe cases of SLE and SS. Second-line therapy with baricitinib, bortezomib, eculizumab, secukinumab, or tocilizumab can be considered in SLE-specific manifestations. These evidence and practice-based recommendations may support treatment decision and, ultimately, improve the outcome of patients living with SLE, APS, or SS.

Serum vitamin D levels and Sjogren's syndrome: bi-directional Mendelian randomization analysis.

BACKGROUND: Based on the results of existing observational studies, it can be found that the association between serum vitamin D levels and the risk of Sjogren's syndrome (SS) in humans is still controversial. Based on this situation, this study aimed to assess the causal relationship between serum vitamin D levels and SS by using the Mendelian randomization (MR) approach. METHODS: In this study, genome-wide association studies (GWAS) summary statistics on serum vitamin D levels [sample size = 417,580 (UK Biobank)] and SS [sample size = 416,757 (cases = 2495, controls = 414,262) (FinnGen)] were used. The bi-directional MR analysis was then used to assess possible causal relationships. The major analysis method of MR was performed using inverse-variance weighted (IVW), supplemented by MR-Egger and the weighted median approaches. In addition, sensitivity analyses were used to ensure the stability of the results, including Cochran's Q test, MR-PRESSO, MR-Egger intercept test, and the leave-one-out test. RESULTS: The MR suggested that no significant causal effects of serum 25(OH)D levels on SS risks were observed [odds ratio (OR) = 0.9824; 95% confidence interval (CI) = 0.7130 to 1.3538; P = 0.9137]. Similarly, no evidence supported the causal effects of SS on serum vitamin D levels (ß: 0.0076, 95% CI: - 0.0031 to 0.0183; P = 0.1640). CONCLUSION: This study found no obvious evidence that serum vitamin D level is causally associated with SS risks or vice versa. We call for larger sample size studies to further unravel the potential causal relationship and the exact mechanism.

Hypokalemia Related to Distal Renal Tubular Acidosis as an Initial Presentation of Primary Sjogren's Syndrome.

Hypokalemia due to loss of potassium through the kidneys can be caused by distal Renal Tubular Acidosis (dRTA). The etiology of dRTA can be primary due to genetic defects or secondary to autoimmune diseases, especially Sjogren's syndrome (SS). The occurrence of dRTA in SS patients is low, at only 5% of cases. This case was interesting because dRTA was the initial clinical manifestation that led to the diagnosis of SS in the patient. A 48-year-old woman came with complaints of recurrent weakness. The patient was routinely hospitalized with severe hypokalemia and received potassium supplementation. The diagnosis of dRTA was based on repeated weakness, normal blood pressure, severe and recurrent hypokalemia, high urinary potassium, alkaline urine, low plasma bicarbonate, and standard anion gap metabolic acidosis. The diagnosis of SS in this patient was confirmed based on dry eyes, dry mouth, positive Schirmer's test, and positive autoantibodies to SS-A and Ro-52. There was a delay in the diagnosis of SS for two years in this patient because the complaints were initially subtle and non-specific. The hypokalemia in this patient was secondary to dRTA associated with primary SS. The possibility of an underlying autoimmune disorder should be considered in a patient presenting with recurrent severe hypokalemia. dRTA, as the etiology of hypokalemia, can be a gateway to the diagnosis of SS. In this patient, complaints related to dRTA appeared before the onset of sicca symptoms, and the diagnosis of SS was established.

Multiparametric autoantibody analysis: a new paradigm for the diagnosis of connective tissue diseases.

BACKGROUND: In patients affected by connective tissue diseases (CTDs), the identification of wide autoantibody profiles may prove useful in early diagnosis, in the evaluation of prognosis (risk stratification), and in predicting response to therapy. The aim of the present study was to evaluate the utility of multiparametric autoantibody analysis performed by a new fully automated particle-based multi-analyte technology (PMAT) digital system in a large multicenter cohort of CTD patients and controls. METHODS: Serum samples from 787 patients with CTD (166 systemic lupus erythematosus; 133 systemic sclerosis; 279 Sjögren's syndrome; 106 idiopathic inflammatory myopathies; 103 undifferentiated CTD), 339 patients with other disorders (disease controls) (118 infectious diseases, 110 organ-specific autoimmune diseases, 111 other rheumatic diseases), and 121 healthy subjects were collected in 13 rheumatologic centers of the FIRMA group. Sera were analyzed with the Aptiva-PMAT instrument (Inova Diagnostics) for a panel of 29 autoantibodies. RESULTS: Multiparametric logistic regression showed that enlarged antibody profiles have a higher diagnostic efficiency than that of individual antibodies or of antibodies that constitute classification criteria for a given disease and that probability of disease increases with multiple positive autoantibodies. CONCLUSIONS: This is the first study that analyzes the clinical and diagnostic impact of autoantibody profiling in CTD. The results obtained with the new Aptiva-PMAT method may open interesting perspectives in the diagnosis and sub-classification of patients with autoimmune rheumatic diseases.

Sjögren's syndrome: shedding light on emerging and key drug targets.

INTRODUCTION: Sjögren's syndrome (SS) is an immune-mediated inflammatory condition characterized by sicca syndrome, musculoskeletal pain, and fatigue. Extra-glandular manifestations are common and there is a markedly increased risk of lymphoma development. SS is associated with high health-economic burden driven largely by the symptom burden on patients. Currently, there is no approved disease-modifying treatment and management is based on empirical evidence. Progress in the understanding of SS pathogenesis has led to an expanding portfolio of more targeted therapies under development. AREAS COVERED: This review summarizes the key development in targeted biological therapies in SS including emerging targets. It also highlights the challenges in therapeutic development in SS such as disease heterogeneity and defining appropriate disease assessment tools to evaluate therapeutic efficacy. EXPERT OPINION: Early trials in SS failed to meet their primary outcomes which may in part due to the use of inappropriate or insensitive study endpoints. Recent trials targeting B-cells, B-T cell co-stimulation and IFN signaling have shown promising results. Development of composite endpoints including patient reported outcomes and objective disease measure may provide a more holistic approach to disease assessment. The impact of these new tools on therapeutic development that benefit patients remains to be fully evaluated.

Sjögren syndrome successfully treated with oxygen-ozone auto-hemotherapy (O2-O3-AHT). a case report.

OBJECTIVE: Sjögren syndrome (SS) is an autoimmune disorder, affecting about 16,000 individuals in Italy, yet lacking a standardized therapy protocol and a plain inclusion in the reimbursed healthcare services. This raises many controversial issues about how managing the SS patient, to relief pain and discomfort and improve patients' health and social life. The ozone therapy resulted successful in previous reports, and therefore, it was used in this case report. CASE PRESENTATION: A 69-years old female outpatient, showing positivity to Schirmer's test, was previously diagnosed as a primary Sjögren syndrome, who later developed an autoimmune thyroiditis and showed the presence of rheumatoid factors. The patient suffered from a marked ocular dryness, subsequently to a purported endothelitis, alongside with fatigue and pain. Laboratory tests showed a positive ANA 1:320 in a speckled pattern with negative anti-SSA and anti-SSB tests. From December 2020 to January 2021 she underwent 2 routes of three sessions of oxygen-ozone autohemotherapy (O2-O3 AHT), as described below and improved, with only 2 sessions, her symptomatology and clinical outcome, as ocular dryness, fatigue and pain, rapidly disappeared. CONCLUSIONS: The use of ozone in the therapy of SS is a straightforward, affordable and feasible approach to treat primary Sjögren syndrome without significant side effects.

Hypokalemic Paralysis Due to Primary Sjögren's Syndrome: Case Series.

BACKGROUND: Sjögren's syndrome (SS) is autoimmune disorder charaterized by exocrine glandular involvement and extra-glandular manifestations. Associations between hypokalemic paralysis and SS have not been emphasized enough. Present study evaluates hypokalemic paralysis as presenting feature in PSS. METHODS: A retrospective cross-sectional study from 2015 to 2020 was conducted to evaluate the clinical phenotype of primary Sjögren's syndrome (PSS) who presented to us with hypokalemic paralysis. RESULTS: Data of 13 patients were evaluated. All were female patients and mean age was 38 years. 61.5% (n= 8) had more than one episode of hypokalemic paralysis; 61.5% (n= 8) patients had oral dryness and 69% (n= 9) had dryness of eyes. 23% (n= 3) patients had inflammatory arthritis and 1 patient had Raynaud's phenomenon, myopathy respectively. 1 patient had chronic constipation and hypothyroidism was present in 61.5% (n= 8) patients. Other co-morbidity included hypertension, renal calculi and situs inversus present in 15%, 15% and 7% respectively. The mean ESR at presentation was 64 mm/hr; average serum potassium level was 2.04meq/dl and distal renal tubular acidosis was present in all patients. Paralysis was completely recovered in all patients after supplementation with potassium. CONCLUSION: The renal involvement in PSS can uncommonly present as hypokalemic paralysis in the absence of significant sicca symptoms or may precede sicca symptoms. A high index of suspicion for PSS should be kept in all patients with hypokalemic paralysis. This phenotype may represent a distinct subset. Serum electrolytes should be regularly monitored in all patients with SS.

A comprehensive overview of living with Sjögren's: results of a National Sjögren's Foundation survey.

To gain insight into the Sjögren's disease (SjD) patient experience using a survey generated by patients and providers. We evaluated the results of the 2016 Sjögren's Foundation survey, with 25 questions designed in a collaborative effort between the Foundation, patients with SjD, SjD provider experts, and a marketing research company. We used descriptive statistics to provide a thorough understanding of SjD demographics, symptoms, quality of life (QoL), cost, and treatments. Analyses revealed high symptoms, QoL, and financial burdens in SjD. Dry mouth and eye were the most commonly reported symptoms (94 and 93%, respectively). The most frequent extra-glandular symptoms included fatigue, dry or itchy skin, and morning stiffness. The top three aspects of QoL most impaired included (i) sex life (53%), (ii) participating in hobbies/social activities/extracurricular activities (52%), and (iii) job/career or ability to work (49%). SjD respondents commonly reported taking health food supplements/remedies, vitamin D, and exercising, in addition to taking treatments for symptomatic dryness. SjD costs were high, including a total yearly cost, on average, of $2026 for dental care. SjD respondents reported that dryness and risk factors for lymphoma and fatigue are essential to address with new therapies. In this comprehensive overview of the SjD experience, we demonstrated a high burden of disease to SjD respondents, including symptoms, QoL, and financial burden. We also identify the top goals of therapy for new systemic SjD therapies. Key Points • The top three symptoms or signs that patients with Sjögren's hope new treatments will address are dryness, fatigue, and reduction in lymphoma or blood cancer risk • The top aspects of quality of life reported to be impaired by Sjögren's are sex life, hobbies, social activities and extracurricular activities, job/career or ability to work, and finding the correct word during conversations • Patients with Sjögren's have a yearly mean dental cost of $2026 but also have high costs associated with prescription medications, healthcare appointments, over-the-counter medications, alternative therapies, and medical equipment.

Sjogren's syndrome-An interesting case.

OBJECTIVE: To present a case of Sjogren syndrome with pulmonary manifestations in an adult female and discuss its assessment and management. DESIGN: Case Report. SETTING: Tertiary care hospital. PATIENT: One. RESULTS: A 50 yrs female admitted with complaints of dryness of eyes with decreased salivation causing difficulty in swallowing since last 3 years, with persistent dry cough since 10-15 days and progressive dyspnea since 4-5 days. Anti-nuclear antibody (ANA) profile revealed Anti- Ro/SS-A and Anti- La/SS-B Positive. Also, sub-lingual excisional biopsy was done which was consistent with findings of Sjogren's syndrome. Patient showed significant improvement after starting oral glucocorticoids, systemic anti inflammatory agents (Tab. HCQS), artificial tear drops, oral iron supplements and other supportive treatment. CONCLUSION: Sjögren syndrome (SS) is a chronic inflammatory disorder characterized by diminished lacrimal and salivary gland function and associated with lymphocytic infiltration of exocrine glands, and can affect extraglandular organ systems including the skin, lung, heart, kidney, neural, and hematopoietic systems. We present a case of Sjogren syndrome in an adult female presenting with xerostomia and dyspnea and was diagnosed upon detection of anti-Ro and anti-La antibodies and confirmed by histopathological examination of lip biopsy. Patient was started on oral steroids and other supportive treatment, General condition improved significantly and is doing very well on regular follow-up.

Comparative analysis of the efficacy and safety of herbal decoction CheReCunJin alone and combined with hydroxychloroquine for treating primary Sjögren's syndrome: A randomized controlled trial.

INTRODUCTION: There is currently no established effective treatment for primary Sjögren's syndrome (pSS). Traditional Chinese Medicine (TCM) is widely used in China and is reported to improve patient symptoms. This study compare the clinical efficacy and safety of herbal decoction CheReCunJin alone and combined with hydroxychloroquine for the treatment of pSS. METHODS: Seventy pSS patients without visceral involvement were randomly assigned in equal numbers to oral administration of CheReCunJin decoction only (group 1) or CheReCunJin decoction combined with hydroxychloroquine (group 2), Efficacy was evaluated after 3 months of treatment by the TCM syndrome and total effectiveness scores, European League Against Rheumatism (EULAR) Sjögren's Syndrome Patient Reported Index (ESSPRI), and Sjögren's Syndrome Disease Activity Index (ESSDAI), Schirmer's test, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and immunoglobulin G (IgG) levels. Safety was assessed. RESULTS: There were no differences in the baseline characteristics of the two groups. Compared with baseline values, the TCM syndrome, ESSPRI and ESSDAI scores, ESR, CRP, and Schirmer's test results improved significantly in both groups after treatment (p < 0.05). There was no significant difference in the TCM syndrome total effectiveness rate between the two groups (p = 0.31). Between-group differences in the changes in ESSPRI, ESSDAI, ESR, CRP, Schirmer's test, and IgG after treatment were not significant (all p> 0.05). Adverse reactions were reported in 5.88% of group 1 and 3.33% of group 2 participants (p = 0.83). CONCLUSION: CheReCunJin decoction alone was effective and safe for the treatment of pSS. TRIAL REGISTRATION: Chinese Clinical Trial Registry: ChiCTR1800016471.

Plummer-Vinson syndrome in primary Sjögren syndrome: a case-based review.

This study aimed to describe a patient with Sjögren syndrome who developed Plummer-Vinson syndrome, and to review the literature and describe shared aspects of this rare association. A systematic screening of articles was conducted in PubMed/MEDLINE, LILACS, SciELO, Scopus, Web of Science, and Cochrane, dating 1940 to 2020. All the articles included the association between Sjögren syndrome and Plummer-Vinson syndrome. No language restriction was applied. The following terms were used: "Sjögren syndrome" or "sicca syndrome" and "Plummer-Vinson syndrome" or "Paterson-Kelly syndrome." We performed our analysis by adding our present case, with a total of 4 cases. Three out of four were female (75%), age varied from 56 to 58 years old. In 2 cases, Sjögren syndrome preceded Plummer-Vinson syndrome diagnosis, and in 1 report, Plummer-Vinson syndrome appeared before Sjögren syndrome. Disease duration varied from 7 to 20 years. In two cases, autoantibodies were available, and antinuclear antibodies and anti-Ro/SS-A were positive in both, and anti-La/SS-B in one of them was associated with anti-dsDNA; however, no data regarding lupus was available in the article. Treatment involved iron supplementation in 3/3. Two out of three received parenteral iron supplementation, and in these two cases, mechanical esophageal dilatation was needless. In the other case, an additional endoscopic esophageal dilatation was necessary to receive the oral iron supplement. All 3 cases had a good outcome. This case illustrates a patient with Sjögren syndrome who developed the rare Plummer-Vinson syndrome. In Sjögren syndrome, the presence of iron-deficiency anemia, dysphagia, and weight loss should alert the physician to search for associated Plummer-Vinson syndrome.

Clinical Features and Laboratory Examination Results of Sjogren's Syndrome Complicated with Thyroid Disorders: A Retrospective Analysis.

Objective: To analyze the clinical incidence, clinical manifestations, laboratory examination, and complications of Sjogren's syndrome complicated with thyroid disorders in patients and to explore the clinical significance of its occurrence and concurrence relationship. Methods: The clinical manifestations, thyroid function, antithyroid antibodies, immunology indicators, autoantibodies, and routine laboratory examination items of 201 patients with Sjogren's syndrome in Chongqing Hospital of Traditional Chinese Medicine were reviewed and analyzed. According to whether the thyroid function was abnormal or not, the patients were divided into the group of Sjogren's syndrome complicated with abnormal thyroid function (n = 36) and the group of Sjogren's syndrome without abnormal thyroid function (n = 165). The clinical symptoms and test indicators of the two groups were compared. Results: Among 201 patients with Sjogren's syndrome, 36 patients had abnormal thyroid function (17.9%) and 36 patients with abnormal thyroid function had hypothyroidism. The abnormal renal function, decreased Hb, decreased WBC, increased ESR, and decreased C4 were more significant in the group with Sjogren's syndrome complicated with abnormal thyroid function, which had significant differences compared with the group with normal thyroid function (P < 0.05). The positive rates of aTG and aTPO in patients with Sjogren's syndrome complicated with thyroid disorders were higher than that in the normal group, and the difference between the two groups was statistically significant (P < 0.05). Conclusion: Patients with Sjogren's syndrome are often associated with hypothyroidism, and these patients may have more severe immune disorders, anemia, leukopenia, and renal involvement. The results show that paying attention to the detection of thyroid function in patients with Sjogren's syndrome may be of positive significance to judge the condition and prognosis.

Sjogren's syndrome in clinical trials of traditional Chinese medicine: protocol for the development of a core outcome set.

BACKGROUND: Sjogren's syndrome (SS) is a chronic autoimmune rheumatic disease with an incidence of 0.03 to 0.3%. In recent years, there are an increasing number of randomized controlled trials of traditional Chinese medicine (TCM) for SS. However, there are generally some problems in these published clinical trials: lack of reporting primary or long-term outcomes and the heterogeneous in different clinical trials' outcome. Our study aims to determine the priority outcomes and standard TCM syndromes for all stakeholders and reach agreement on the COS and syndromes to be measured and reported in all future TCM trials in patients with SS. METHODS AND ANALYSIS: A phase-wise refinement approach will be used, consisting of three phases, yet complementary, sub-work phases, whereby each phase will inform the next coming phases. The following are the three phases: (I-a) identifying of a long initial list of outcomes through systematic literature review and semi-structured qualitative interviews and (I-b) identifying an initial list of TCM syndromes through (1) systematic literature review, (2) referencing ancient Chinese medical books, and (3) retrospective studies of medical records; (II) prioritization of outcomes using Delphi survey with different stakeholders, such as health professionals and patients; and (III) through consensus meetings with patients and professionals to agree on the final COS and TCM syndromes. DISCUSSION: We summarized the actions of COS into three points: direct action, indirect action, and final action. After the final COSs is completed, we will publish this research in a journal to promote communication. TRIAL REGISTRATION: Core Outcome Measures in Effectiveness Trials Initiative (COMET) number 1429 . Registered on 01 December 2019.

Distal Renal Tubular Acidosis due to Primary Sjögren's Syndrome: Presents as Hypoakalemic Paralysis with Hypokalemia-Induced Nephrogenic Diabetes Insipidus.

Classic distal renal tubular acidosis (dRTA) is characterized by inability to acidify the urine maximally (to

Distal renal tubular acidosis and nephrocalcinosis as initial manifestation of primary sjögren's syndrome.

There is a well-established association between primary Sjögren's syndrome and distal renal tubular acidosis (dRTA). dRTA is a relatively infrequent manifestation of primary Sjögren's syndrome which can present with life-threatening electrolyte abnormalities while, in some patients, it could be the first manifestation of the syndrome. We report the case of a 35-year-old woman who presented with unexplained episodes of generalized weakness, severe hypokalemia, nephrocalcinosis, and normal anion gap metabolic acidosis. Subsequent evaluation revealed primary Sjögren's syndrome as her underlying condition. The patient responded well to potassium supplementation, sodium bicarbonate, and oral prednisolone. After four years of follow-up, there were no other extraglandular manifestations, the renal function remained stable and the acidosis was partially improved without the need for oral bicarbonate. This case demonstrates that dRTA could be the initial manifestation of primary Sjögren's syndrome and highlights the necessity for increased vigilance for patients presenting with persistent hypokalemia or nephrocalcinosis so that an early diagnosis can be made allowing for better control and prevention of disease progression.

Recent Advances in the Use of Exosomes in Sjögren's Syndrome.

Sjögren's syndrome (SS) is a chronic autoimmune disorder of the exocrine glands mediated by lymphocytic infiltrates damaging the body tissues and affecting the life quality of patients. Although traditional methods of diagnosis and treatment for SS are effective, in the time of personalized medicine, new biomarkers, and novel approaches are required for the detection and treatment of SS. Exosomes represent an emerging field in the discovery of biomarkers and the management of SS. Exosomes, a subtype of extracellular vesicles, are secreted by various cell types and can be found in most bodily fluids. Exosomes are packed with cytokines and other proteins, bioactive lipids, and nucleic acids (mRNA, circular RNA, non-coding RNA, tRNA, microRNA, genomic DNA, and ssDNA), and transport such cargo between cells. Evidence has indicated that exosomes may play roles in processes such as the modulation of the immune response and activation of inflammation. Moreover, due to features such as stability, low immunogenicity and toxicity, long half-life, and the capacity to penetrate the blood-brain barrier, exosomes have also emerged as therapeutic tools for SS. In this review, we summarize existing literature regarding the biogenesis, isolation, and function of exosomes, specifically focusing on exosomes as novel biomarkers and their potential therapeutic uses in SS.