Sjogren's Syndrome

Background: \- Salivary glands in and around the mouth and throat make saliva. Salivary gland disorders can affect a person s quality of life. Studying people who have a disease that affects their salivary gland(s) may teach researchers about the disorders and their genetics. Objectives: \- To study salivary gland diseases and disorders. To collect data and samples from people with salivary gland problems and their relatives. Eligibility: * People more than 4 years old who have or are suspected to have a disease involving salivary glands. * Their relatives more than 4 years old. * Healthy volunteers 18 years or older. Design: * Participants may be screened with: * Medical history * Physical exam * Blood and urine tests * General oral and dental history and exam * Saliva collection * Eye exam and test for dry eyes * Health questionnaires (adults) * Biopsy of some minor salivary glands. A small incision will be made on the inside of the lower lip and several tiny salivary glands will be removed. * Participants will have 2-3 visits. These may include: * Repeats of some screening tests * Ultrasounds of some glands. Researchers will put some gel on the face, then press on it with a smooth wand. * Adults may have other biopsies * A small catheter inserted into the opening of the parotid gland duct on the inside of the cheek. A saline solution (in a syringe) will fill the duct. * Swishing a saltwater solution in the mouth for 10 seconds and then spitting into a cup * Scrapings collected from teeth, tongue, and cheeks

Background: Sjogren disease is an autoimmune disease - that is, a disease that causes the body's immune system to attack its own organs and tissues. Sjogren disease can affect the kidneys, lungs, or other organs. It can also cause dry mouth and eyes, fever, joint pain, rashes, and other symptoms. Researchers want to know if a drug approved to treat rheumatoid arthritis and other autoimmune diseases can help people with Sjogren disease. Objective: To test a drug (tofacitinib) in people with Sjogren disease. Eligibility: People aged 18 to 75 years with Sjogren disease. They must be enrolled in protocol 15-D-0051. Design: * Participants will be screened. They will have a physical exam with blood and urine tests. They will give samples of saliva; a small sample of tissue will be taken from a salivary gland. They will have a test of their heart function. They will have an eye exam, including a test for dry eyes. * Tofacitinib is a tablet taken by mouth. Participants will take the drug twice a day at home. * Participants will have 9 clinic visits over 28 weeks. Each visit will take up to 5 hours. In addition to repeated tests, they will have tests of the speed and pressure of blood flow through their body. They will complete health questionnaires throughout the study. * Participants will also have 5 phone visits during the study. They will review their health and study treatments. * They will have 1 final visit after they stop taking the drug.

The objectif is to study the diagnosis performance of the different classification criteria in reference to the gold standard consisting of the diagnosis made by expert doctors after standardized assessment, of pSS (primary Sjogren syndrome)

Background: -Sjogren s Disease (formerly: Sjogrens Syndrome, Sj(SqrRoot)(Delta)gren s syndrome) is a disease that affects about 1-4 million Americans. It is more common in women. It mainly affects the glands that produce saliva and tears, leading to dry eyes and dry mouth. The cause of Sjogren s Disease is unknown, but inflammation plays an important role. The purpose of this study is to learn more about Sjogren s Disease. Objectives: -To better understand how Sjogren s Disease begins and how it affects patients so that we can develop better ways to treat them. Eligibility: * Participants must be 16 years of age or older. * They must have a diagnosis of Sjogren s Disease or have at least two symptoms of Sjogren s Disease. Design: * People taking part in the study will come to the NIH Clinical Center for at least three visits. * During these visits, participants will have a medical history and physical exam. They will have oral and dental assessments, and saliva collection. Lab tests (blood and urine) and dry eye exams will be done. Participants will answer questionnaires and have salivary scintigraphy (adults only unless required for diagnosis). * Other optional tests may also be done. Participants may have to come in for additional visits if they have these optional tests or if their disease changes. * The only treatment provided as part of this study is for medical emergencies or complications that occur while you are at NIH for evaluation.

A phase 1b, open-label study of CLN-978 administered subcutaneously in patients with active, moderate to severe Sjogren's Disease.

This is an open clinical pharmacological translational Research Study, aiming to evaluate the safety, tolerability, pharmacokinetics, pharmacodynamics and efficacy of CD19 or CD19-BCMA CAR-T in patients with active SLE, SSc, AAV, IIM and pSS.

This study evaluates the safety and efficacy of YTS109 cells in adults with relapsed/refractory autoimmune diseases, such as Systemic Lupus Erythematosus (SLE), including LN and SLE-ITP, Sjogren's Syndrome, etc. Aproximately 18 patients aged 18-65 will receive a single infusion of YTS109 cells. The dose groups are set to commence at 3×10⁶ STAR -T cells/kg, employing a 3+3 escalation principle for dose titration. The primary objective of this study is to evaluate the safety of YTS109 cells therapy in treating recurrent/refractory autoimmune diseases, while the secondary objectives are to assess the efficacy of YTS109 cells as well as their pharmacokinetic and pharmacodynamic characteristics. The primary endpoint is observations of types, severity, and frequency of adverse events (AEs) and efficacy assessment. This single-arm, open-label trial will enroll patients across Bengbu Third People's Hospital.

This study will involve the collection of follow-up data for patients who previously participated in the Sjogren's International Clinical Collaborative Alliance (SICCA) study at the University of Pennsylvania. Clinical data and specimens will be collected from subjects with objective evidence of dry eye who were or were not diagnosed with Sjogren's syndrome at the time of their initial participation in the SICCA study. Specimens will be collected from participants which will include tears, saliva, whole blood, serum, DNA and possible labial minor salivary gland biopsies when indicated. All individuals will participate in a standard evaluation protocol including an oral, ocular and physical examination, objective tests for dry eyes and dry mouth and, whenever necessary, a labial minor salivary gland biopsy. The biopsy requirement is waived for those who have already had positive lip biopsies in the past.

To facilitate clinical, basic science, and translational research projects involving the study of rheumatic diseases.

The goal of this project is to critically evaluate the effectiveness of an online health program designed to improve diet and self-care in patients with rheumatological conditions, including rheumatoid arthritis (RA), Sjogren's syndrome (SS), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), psoriatic arthritis (PsA), Additionally, investigators will assess the program's effectiveness, as well as the challenges and facilitators involved in using an online wellness program to reduce fatigue and enhance the quality of life in patients suffering from these conditions.

This multi-site registry, centered at Duke University, will enroll pregnant women with autoimmune and rheumatologic diseases. The main goal of MADRA is to identify ways to improve the health of women with rheumatic diseases and their babies during pregnancy. Prior studies demonstrate the importance of increase inflammation prior to and during pregnancy on these outcomes. The future research will seek to better define these risk factors and to identify ways to may improve them.

Generative AI (GenAI) based on large language models (LLMs) is expected to improve the diagnosis and treatment of autoimmune diseases. We are studying how GenAI may affect the diagnosis of various complications of rheumatoid arthritis (RA). In a retrospective study using RA patients' EHR records, we will quantify physician adoption of GenAI predictions for RA complications and co-existing diseases. In a prospective observational study, we will assess the feasibility of using GenAI predictions as additional clinical information to help physicians make more complete diagnoses of RA complications and co-existing diseases, including complex, uncommon, or rare conditions.

Cannabis, in addition to its psychotropic properties, could have anti-inflammatory and immunomodulatory effects. Phytocannabinoids (pCBs) are a group of molecules naturally secreted by the cannabis plant. The major pCBs are cannabidiol (CBD) and Δ9-tetrahydrocannabinol Δ9 (THC). Only THC has psychotropic effects, which CBD does not have. Alongside these two main components, there is a wide variety of other molecules, such as other pCBs and terpenes which could increase the effects on immune system through synergistic interactions between these different compounds ("entourage effect").In vivo, pCBs essentially interfere with the endocannabinoid system, acting on many ubiquitous receptors, present on a significant number of different cell types. Numerous published studies show that pCBs have immunomodulatory and anti-inflammatory properties by acting on several of these receptors, whether through modulation of the immune response of different cell types, effects on cytokine networks, reduction of innate and adaptive responses and/or impact on cell survival or death (autophagy, proliferation/ apoptosis). The Immune-Mediated Inflammatory Diseases (IMIDs) affect 5 to 7% of the general population in Western countries, involve different organs (joints, skin, digestive tract) but share the same inflammatory mechanisms resulting from a dysregulation of the immune response. Our research focuses on the identification of the most effective phytochemical profile of pCBs, allowing an optimal effect on chronic inflammatory pathologies of interest among immune-mediated chronic inflammatory diseases (IMIDs). The pCB-IMIDs project is therefore part of an innovative translational project, around new therapeutic applications of medical cannabis (CannAppIMIDs). In our study, we will include 100 patients with one of IMIDs among Rheumatoid Arthritis, spondylarthritis, psoriatic arthritis, Sjogren disease and systemic lupus, at different stage and with different treatments. After patient's consent we will collect for research purposes an additional 40 ml of blood during a routine care blood test. Mononuclear and polynucleated blood cells will be exposed in vitro to different full-spectrum pCB extracts (full spectrum extract) including a CBD dominant and low THC extract (\<0.2%), 1 dominant THC extract, 1 balanced THC/CBD extract and 1 dominant CBG extract. In this cross-sectional study, our objective will be to assess the biological effects of different pCB compositions on inflammatory profiles (concentrations of pro and anti-inflammatory cytokines and chemokines) and modulations of expression profiles (autophagy, apoptosis, and cannabinoid receptor expression profile).

Sjögren's syndrome (SS) is a chronic autoimmune disease primarily affecting the salivary and lacrimal glands, leading to symptoms of dryness. Assessing the inflammatory activity is crucial for guiding treatment. Fibroblast activation protein (FAPI) PET is an emerging imaging technique increasingly used for evaluating various inflammations and tumors. Thus this prospective study is going to investigate the uptake characteristics of FAPI PET in Sjögren's syndrome and evaluate its potential in assessing inflammatory activity to guide clinical treatment. A control group of tumor patients undergoing FAPI PET imaging will be included to compare FAPI uptake patterns between autoimmune inflammation and neoplasia.

The goal of this study is to determine the safety and tolerability of orally taken probiotic (R-2487) in patients with Sjogren's Syndrome. Patients will take an oral dosage of probiotic (R-2487) and physicians will assess and measure their Sjogren's Syndrome. Blood and fecal evaluations of inflammation and assessment of probiotic (R-2487) on fecal level will also be measured.

Longitudinal prospective multicenter Armenian registry of systemic autoimmune, autoinflammatory diseases with constitution of bio-banking.

Inflammatory rheumatic diseases affect 1% of the population. Treatment of such diseases should be based on disease activity, safety issues and other patient characteristics such as comorbidities (EULAR, 2022), leading to a higher risk of cardiovascular diseases. To this end, the general treat-to-target approach, as recommended in the EULAR guidance, may require several successive treatment lines based on updates to the patients' profile and close monitoring as the keystone of its implementation. Regular feedback from patients could be used to fuel such strategies. This feedback can be collected using an ePRO (electronic Patient Reported Outcome). The purpose of this study is therefore to assess patient management using the information provided by patients through e-PROs, which will transfer the data provided by the patient to the physician and will notify the investigators via email when a patient has completed a form (no data interpretation or alerts). The hypothesis is that the more physicians are provided with insights into their patients' health, the more they will function in a treat-to-target approach and the more often they will tend to adjust their patients' treatments.

By analyzing the differential proteins in exosomes, this study aims to understand the pathological mechanisms of SJS and DES, identify potential diagnostic and therapeutic methods, and advance the diagnosis and treatment of these diseases.

This study aims to collect information on rheumatology patients' dietary habits, autoimmune disease activity, dietary changes, disease symptom improvements, and perceptions on their dietary habits and how it affects their autoimmune disease. The main objective is to see if rheumatology patients change their dietary habits after their diagnosis of an autoimmune disease and if it subjectively improved their disease symptoms. It will also look at rheumatology patients' expectations for their rheumatologist when it comes to dietary advice and what resources they used to choose their new dietary habits. The study also seeks to measure the interest that rheumatology patients have in pursuing dietary changes as a means of controlling the symptoms of their autoimmune disease. It is expected that patients who changed their eating habits to healthier diets such as a Mediterranean diet would report less severe autoimmune disease symptoms. There are limited dietary recommendations for the management of many rheumatological diseases, so this study seeks to assess rheumatology patients' willingness to try dietary modifications, what improvements they had, and why they decide to make these changes in light of limited information.

This is an open, single-arm, clinical study to evaluate the efficacy and safety of chimeric antigen receptor T cell immunotherapy (CAR-T) targeting BCMA or CD19 or both sequentially in the treatment of Relapsed/ Refractory Autoimmune Disease such as Sjogren's Syndrome or Systemic Lupus Erythematosus and other Autoimmune Disease.

This study is a preliminary investigation, with a single-group design, not randomized and transparent, focusing on treatment. Its purpose is to identify the highest dose of BH002 injection (CD19-BCMA CAR-T cells) that patients suffering from resistant systemic lupus erythematosus can tolerate.

An exploratory clinical study of the safety and efficacy of YTS109 cell injection in subjects with recurrent/refractory autoimmune disease

Sjögren's syndrome is a complex autoimmune disorder. The investigators hypothesize that striated ductal cells may function as non-professional antigen-presenting cells in Sjögren's syndrome. However, there are currently no established methods to assess the functional status of striated ductal cells or their relationship with clinical presentations. To address this knowledge gap, the investigators aim to investigate the potential of sialoscintigraphy, a non-invasive imaging technique, to evaluate the functional status of striated ductal cells. The sodium iodide symporter (NIS), predominantly expressed in striated ductal cells, facilitates the transport of technetium-99m, a radiotracer used in sialoscintigraphy. The investigators hypothesize that the expression level of NIS, as evaluated through sialoscintigraphy, may serve as an indicator of striated ductal cell function and potentially correlate with clinical manifestations in Sjögren's syndrome. However, the literature and our preliminary data lead us to hypothesize that age and the specific major salivary gland being evaluated may introduce confounding factors in the interpretation of sialoscintigraphy results. The investigators therefore proposed to 1) establish a nomogram of sialoscintigraphy stratified by age and specific salivary glands. The investigators will recruit healthy volunteers to receive sialoscintigraphy for the nomogram; 2) associate the expression level of NIS in the striated ductal cells from the major salivary glands with sialoscintigraphy, markers of antigen presentation, and disease manifestations of Sjögren's syndrome. The outcomes will help further applications of sialoscintigraphy in Sjögren's syndrome and formulate hypotheses to explore the pathological mechanisms underlying Sjögren's syndrome.

Xerostomia is a common and very bothersome manifestation that impairs the quality of life in Sjogren's syndrome. Its diagnosis mainly relies on the measurement of salivary flow (SF). Performing this test is unpleasant for the patient, lacks precision, can be influenced by certain conditions, and requires good patient cooperation. Other alternatives such as the buccal Schirmer test can be used. The aim of this study is to demonstrate the non-inferiority of the buccal Schirmer test compared to SF measurement. This is a diagnostic study comparing a group of patients (n=90) with Sjogren's syndrome (SS) and normal SF (≥0.1 ml/minute) to a group of patients with SS and decreased SF (\<0.1 ml/minute).

Evaluations will be made by researchers following the guidance of individuals with primary Sjögren's Syndrome who receive diagnosis, routine medical care, and treatment management. In addition, for the reliability of the Turkish Eating Assessment Tool-10, the Turkish Eating Assessment Tool-10 will be repeated on patients at least one-fifth of the number of individuals included, after one week. For the sensitivity of the scale, an exercise that is routinely applied in the Rheumatological Rehabilitation Unit of Hacettepe University Faculty of Physical Therapy and Rehabilitation will be invited and after 3 months, the same evaluations as applied in the first measurement will be made again on individuals at least one-fifth of the number of individuals included.