The Pathogenesis and Natural History of Sjogren's Disease

June 17, 2026 updated by: National Institute of Dental and Craniofacial Research (NIDCR)

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.

Study Overview

Status

Status

Indicates the current recruitment status or the expanded access status.
Recruiting

Conditions

Conditions

The disease, disorder, syndrome, illness, or injury that is being studied. On ClinicalTrials.gov, conditions may also include other health-related issues, such as lifespan, quality of life, and health risks.

Detailed Description

Background

Sjogren's Disease (SjD; formely Sjogren's Syndrome) is an autoimmune disease characterized by chronic inflammation involving the exocrine glands. Salivary and lacrimal glands are predominantly affected leading to dry mouth and dry eyes but other exocrine organs are also frequently involved. It is one of the most common rheumatic autoimmune diseases, which effects between 1-4 million Americans, predominantly women with a female to male ratio of 9:1. Sjogren s Disease may occur alone (primary SjD), or may coexist with other systemic connective tissue disorders (i.e., secondary SjD). In many cases systemic manifestations, such as fatigue, arthritis, vasculitis, lung disease, peripheral or central neuropathy and autonomic nervous system dysfunction accompany glandular involvement. Patients with systemic manifestations are at higher risk of lymphoma, the incidence of which is increased in SjD. The treatment of sicca symptoms is mainly symptomatic, whereas management of extraglandular manifestations is similar to other autoimmune diseases.

The cause and pathogenesis of Sjogren's Disease is still largely unknown. In a genetically predisposed individual various environmental factors, such as viral infections, may lead to epithelial cell activation and a protracted inflammatory response with features of autoimmunity. Autoreactive lymphocytes and autoantibodies are considered important in this process although the pathogenic role of any particular autoantibody is still undefined. Although inflammation may contribute to the exocrinopathy of SjD, the relationship between inflammation and exocrine dysfunction is poorly understood. Moreover, the model does not explain many of the extraglandular manifestations of SjD patients, such as fatigue. Further studies are needed to better understand the pathogenesis of SjD.

Objectives

The primary objective of this study is to enable the collection of longitudinal clinical and laboratory data and biologic specimens to identify pathogenetic mechanisms of SjD by careful clinical phenotyping of SjD patients and Sjogren's-like conditions over time and collection of biologic samples for concurrent and future laboratory studies related to the pathogenesis of Sjogren's Disease. Another objective of the study is to identify biomarker candidates associated with the diagnosis, severity, prognosis, or organ involvement in SjD. The protocol will enable the study of the genetic basis and the mechanistic aspects of immunologic and non-immunologic abnormalities of SjD and their associations with various clinical phenotypes.

Eligibility Criteria

The study will enroll 300 subjects with Sjogren s Disease or Sjogren's-like conditions. Subjects aged 16-years or older fulfilling European American Consensus Criteria for Primary or Secondary Sjogren s Disease are eligible for the study. Selected subjects with incomplete Sjogren's Disease or who are excluded from the European American criteria may also be eligible. Screening will be done on the Characterization of Diseases with Salivary Gland Involvement protocol (15-D-0051) prospectively or on the previous screening protocol (84-D-0056).

Description of the Study

This is a longitudinal observational study. All subjects will have core evaluations (approximately biannually) during a 10-year period. Additional evaluations may be required if there is a significant change in the clinical condition of subjects likely related to SjD or sicca syndrome or to provide additional research samples or clinical data for the pathogenesis studies. Clinical data will be collected through questionnaires, personal interviews, physical examination, laboratory testing and imaging studies. The core evaluation will include a complete medical history and physical examination and a complete oral and dry eye evaluation. Blood, saliva and biopsy samples will be stored and used for laboratory research studies aimed at the pathogenesis of Sjogren s Disease. Samples labeled with a code without any personal identifiers may be shared with researchers in and outside the NIH. DNA will be collected for genetic studies related to Sjogren's' syndrome and related conditions.

Study Type

Study Type

Describes the nature of a clinical study. Study types include interventional studies (also called clinical trials), observational studies (including patient registries), and expanded access.
Observational

Enrollment (Estimated)

Enrollment

The number of participants in a clinical study. The "anticipated" enrollment is the target number of participants that the researchers need for the study.
300

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact

The name and contact information for the person who can answer enrollment questions for a clinical study. Each location where the study is being conducted may also have a specific contact, who may be better able to answer those questions.

Study Contact Backup

Study Locations

  • United States
    • Maryland
      • Bethesda, Maryland, United States, 20892
        • Recruiting
        • National Institutes of Health Clinical Center

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Eligibility Criteria

The key requirements that people who want to participate in a clinical study must meet or the characteristics they must have. Eligibility criteria consist of both inclusion criteria (which are required for a person to participate in the study) and exclusion criteria (which prevent a person from participating). Types of eligibility criteria include whether a study accepts healthy volunteers, has age or age group requirements, or is limited by sex.

Ages Eligible for Study

16 years and older (Child, Adult, Older Adult)

Accepts Healthy Volunteers

No

Sampling Method

Non-Probability Sample

Study Population

The population consists of patients who have completed our Sjogren's screening protocol (15-D-0051) and are deemed eligible to enroll in this pathogenesis protocol.

Description

  • INCLUSION CRITERIA:

    1. Ability to sign informed consent form

    2. Fulfilling one the definitions below:

      1. Sjogren s defined by European-American (EA) classification criteria for primary or secondary Sjogren s Disease (SjD group)
      2. Excluded from the EA criteria because of a comorbid condition but otherwise fulfilling the European-American classification criteria (EA excluded SjD group)
      3. Incomplete SjD

      i. at least 2 of the EA criteria with a common manifestation of SjD not included in these criteria (e.g., fatigue, vasculitis, arthritis, etc) or

      ii. 2 or more common manifestations of SjD which are not included in the EA criteria (e.g.,: fatigue, vasculitis, arthritis, autonomic dysfunction, etc ) and are not explained by other conditions

      EXCLUSION CRITERIA:

    1. Age <16 years
    2. inability or unwillingness to comply with follow up requirements
    3. Any medical or psychological/psychiatric condition or treatment that, in the opinion of the Principal Investigator, would exclude the subjects from the research studies (e.g., alternative explanation for subjects signs and symptoms)
    4. NIH employees who report directly to the principal investigator or who are a co-worker or relative of the principal investigator.

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Observational Models: Cohort
  • Time Perspectives: Prospective

Cohorts and Interventions

Group / Cohort

Group / Cohort

A group or subgroup of participants in an observational study that is assessed for biomedical or health outcomes.
incomplete sjogren's
patients who meet criteria as incomplete sjogren's
primary sjogren's
patients who meet criteria for classification for primary sjogren's
secondary sjogren's
patients who meet classification criteria for secondary sjogren's

What is the study measuring?

Primary Outcome Measures

Primary Outcome Measures

In a clinical study's protocol, the planned outcome measure that is the most important for evaluating the effect of an intervention/treatment. Most clinical studies have one primary outcome measure, but some have more than one.
Outcome Measure
Measure Description
Time Frame
Clinical phenotyping of SjD patients and controls over time Collection of samples for concurrent and future laboratory studies related to the pathogenesis Identification of biomarker candidates in SjD
Time Frame: patients are followed up to 10 years
following the natural history of Sjogren's disease progression.
patients are followed up to 10 years

Secondary Outcome Measures

Secondary Outcome Measures

In a clinical study's protocol, a planned outcome measure that is not as important as the primary outcome measure for evaluating the effect of an intervention but is still of interest. Most clinical studies have more than one secondary outcome measure.
Outcome Measure
Time Frame
1. To establish a teaching cohort of patients with SjD or SjD-like conditions treated with conventional treatments 2. To identify patients for other protocols related to Sjogren's Disease
Time Frame: Ongoing
Ongoing

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Sponsor

The organization or person who initiates the study and who has authority and control over the study.
National Institute of Dental and Craniofacial Research (NIDCR)

Investigators

Investigators

A researcher involved in a clinical study. Related terms include site principal investigator, site sub-investigator, study chair, study director, and study principal investigator.
  • Principal Investigator: Zohreh Khavandgar, D.D.S., National Institute of Dental and Craniofacial Research (NIDCR)

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Helpful Links

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

Study Start

The actual date on which the first participant was enrolled in a clinical study. The "anticipated" study start date is the date that the researchers think will be the study start date.
January 18, 2012

Study Registration Dates

First Submitted

First Submitted

The date on which the study sponsor or investigator first submitted a study record to ClinicalTrials.gov. There is typically a delay of a few days between the first submitted date and the record's availability on ClinicalTrials.gov (the first posted date).
August 27, 2011

First Submitted That Met QC Criteria

First Submitted That Met QC Criteria

The date on which the study sponsor or investigator first submits a study record that is consistent with National Library of Medicine (NLM) quality control (QC) review criteria. The sponsor or investigator may need to revise and submit a study record one or more times before NLM's QC review criteria are met. It is the responsibility of the sponsor or investigator to ensure that the study record is consistent with the NLM QC review criteria.
August 27, 2011

First Posted (Estimated)

First Posted

The date on which the study record was first available on ClinicalTrials.gov after National Library of Medicine (NLM) quality control (QC) review has concluded. There is typically a delay of a few days between the date the study sponsor or investigator submitted the study record and the first posted date.
August 30, 2011

Study Record Updates

Last Update Posted (Actual)

Last Update Posted

The most recent date on which changes to a study record were made available on ClinicalTrials.gov. There may be a delay between when the changes were submitted to ClinicalTrials.gov by the study's sponsor or investigator (the last update submitted date) and the last update posted date.
June 18, 2026

Last Update Submitted That Met QC Criteria

Last Update Submitted That Met QC Criteria

The most recent date on which the study sponsor or investigator submitted changes to a study record that are consistent with National Library of Medicine (NLM) quality control (QC) review criteria. It is the responsibility of the sponsor or investigator to ensure that the study record is consistent with the NLM QC review criteria.
June 17, 2026

Last Verified

Last Verified

The most recent date on which the study sponsor or investigator confirmed the information about a clinical study on ClinicalTrials.gov as accurate and current. If a study with a recruitment status of recruiting; not yet recruiting; or active, not recruiting has not been confirmed within the past 2 years, the study's recruitment status is shown as unknown.
March 17, 2026

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

Other Study ID Numbers

Identifiers or ID numbers other than the NCT number that are assigned to a clinical study by the study's sponsor, funders, or others. These numbers may include unique identifiers from other trial registries and National Institutes of Health grant numbers.
  • 110172
  • 11-D-0172

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

NO

IPD Plan Description

This is a natural history study that began enrolling in 2011. De-identified data will be made public consistent with the policies of the NIH.

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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