Natural History of Salivary Gland Dysfunction and Sjogren's Syndrome
Data Collection
Arthritis+21
+ Arthritis, Rheumatoid
+ Autoimmune Diseases
Summary
Study start date: April 9, 1999
Actual date on which the first participant was enrolled.Saliva plays a major role in maintaining oral health and comfort. Saliva is needed to moisten the mouth, to lubricate food for easier swallowing, to protect oral hard and soft tissues, to modulate oral microbial populations, to provide enzymes necessary to begin food breakdown for digestion, and to promote soft tissue repair and oral cleansing. Therefore, salivary dysfunction may result in numerous clinical conditions affecting oral and systemic health, comfort and quality of life. In particular, we will focus on individuals with Sj(SqrRoot)(Delta)gren's syndrome, an autoimmune exocrinopathy that primarily affects the salivary and lacrimal glands. A number of unanswered questions remain concerning salivary involvement in this disorder. These include the rate of progression of secretory dysfunction, and related oral and systemic complications associated with xerostomia in autoimmune and non-autoimmune diseases, and B-cell dysregulation. Also, more precise estimates of the incidence of the lymphoma development are needed. The purpose of this study is :1) to allow careful follow-up of patients with defined salivary gland alterations so that the long term course and effects of Sj(SqrRoot)(Delta)gren's syndrome (SS) on the oral cavity and systemic health in SS may be delineated; 2) to follow the development and progression of B-cell dysregulation in SS; 3) to follow subjects to establish whether those initially manifesting incomplete criteria for SS progress toward fully meeting the criteria.; 4) to refine diagnostic tests for SS, and to determine whether those subjects who meet the criteria for SS continue to do so; and 5) to develop intermediary outcome measures for SS based on long term outcomes (loss of tears and loss of stimulated salivary flow). Patients will return every two years from the baseline visit for a full oral examination, salivary function assessment, clinical laboratory studies, and questionnaires concerning signs and symptoms of salivary gland dysfunction. These individuals will be patients with Sj(SqrRoot)(Delta)gren's syndrome (SS), incomplete SS (patients who have some, but not all of the criteria for SS) or radiation-induced salivary gland hypofunction. We anticipate that many of these patients will also participate in therapeutic trials conducted within the branch.
Protocol
This section provides details of the study plan, including how the study is designed and what the study is measuring.560 patients to be enrolled
Total number of participants that the clinical trial aims to recruit.Eligibility
Researchers look for people who fit a certain description, called eligibility criteria: person's general health condition or prior treatments.Any sex
Biological sex of participants that are eligible to enroll.Over 4 Years
Range of ages for which participants are eligible to join.Healthy volunteers allowed
If individuals who are healthy and do not have the condition being studied can participate.Conditions
Pathology
Criteria
INCLUSION CRITERIA: Male and female subjects. All subjects will have first participated in screening protocol 84-D-0056 to confirm their diagnosis and assess salivary function. Subjects must have dry mouth symptoms (xerostomia) and a diagnosis of primary or secondary SS, incomplete SS, or radiation-induced salivary gland dysfunction (as determined in protocol 84-D-0056). Diagnostic criteria for SS are the American-European Consensus Group Classification Criteria: For primary Sjogren s syndrome, any 4 of the 6 criteria, must include item IV (Histopathology) or VI (Auto-antibodies) or any 3 of the 4 objective criteria (III, IV, V, VI). For secondary Sjogren s syndrome, must have established connective tissue disease, one symptom (I or II) plus 2 of the 3 objective criteria (III, IV, VI). Ocular symptoms (at least one) Daily persistent dry eyes for greater than 3 months? Recurrent sensation of sand or gravel in the eyes? Use of tear substitutes greater than 3 x/day? Oral symptoms (at least one) Daily feeling of dry Mouth greater than 3 months Recurrent or persistently swollen salivary glands as an adult? Frequently drink liquids to aid in swallowing dry foods? Ocular signs (at least one) Schirmer s test, (without anesthesia) greater than or equal to 5mm/5 minutes Positive vital dye staining (van Bijsterveld greater than or equal to 4) Histopathology: Lip biopsy showing focal lymphocytic sialadenitis (focus score greater or equal to 1 per 4mm squared) Oral signs (at least one) Unstimulated whole salivary flow (greater or equal to 1.5 ml in 15 minutes) Abnormal parotid sialography Abnormal salivary scintigraphy Auto-antibodies (at least one) Anti-SSA (Ro) or Anti-SSB (La) EXCLUSION CRITERIA FOR DIAGNOSIS OF SS: Past head and neck radiation treatment Hepatitis C infection Acquired Immunodeficiency Syndrome (AIDS) Pre-existing lymphoma Sarcoidosis Graft versus host disease Current use of anticholinergic drugs EXCLUSION CRITERIA: Failure to complete evaluation procedures as specified in 84-D-0056. Diagnosis of drug-related xerostomia. Age less than 4 years. There are no exclusions based on gender, race, or ethnicity. Salivary gland dysfunction is uncommon in children and exceedingly rare in those less than 4 years old. Additionally, the evaluation method described in the protocol cannot be used in this age group. ULTRASOUND GUIDED CORE NEEDLE BIOPSY OF THE PAROTID GLAND Adult subjects already enrolled in this protocol and healthy volunteers will be eligible for this research procedure if they fulfill the criteria below and sign the parotid biopsy consent form. Healthy volunteers will be recruited only for the ultrasound guided core needle biospy of the parotid gland. They will sign the parotid biopsy consent form only. INCLUSION CRITERIA: Age 18 years or older. Ability to give informed consent. EXCLUSION CRITERIA: History of bleeding diathesis or the current use of anticoagulants. Any uncontrolled or severe chronic disease.
Study Centers
These are the hospitals, clinics, or research facilities where the trial is being conducted. You can find the location closest to you and its status.This study has 1 location
National Institutes of Health Clinical Center, 9000 Rockville Pike
Bethesda, United StatesOpen National Institutes of Health Clinical Center, 9000 Rockville Pike in Google Maps