Protocol Number: 99-D-0070

Title:

Natural History of Salivary Gland Dysfunction and Sjogren's Syndrome

Number:

99-D-0070

Summary:

This study will follow patients with salivary gland dysfunction to identify the long-term course of this disorder and its effects on the mouth, oral function, and overall health. Saliva is important in maintaining oral health and comfort. It moistens the mouth, lubricates food for easier swallowing, provides enzymes needed to begin the digestive process and promotes repair and cleansing of soft tissues of the mouth. Decreased salivary production or changes in salivary composition may affect oral and systemic health and cause an increase in tooth decay.

Patients 4 years of age and older with dry mouth symptoms and a diagnosis of primary, secondary or incomplete Sjogren's syndrome or salivary gland dysfunction due to radiation may be eligible for this study. Candidates will be screened with a complete medical and dental history and blood and saliva tests. Some patients will have a biopsy of the minor salivary glands, usually from the lower lip, to confirm or rule out the diagnosis of Sjogren's syndrome and determine the extent of changes in the salivary glands. (A biopsy is the surgical removal of a small piece of tissue for laboratory examination.) The ability to taste and smell may also be evaluated, and patients may have an ultrasound examination of their swallowing function.

Participants will have a general oral examination of the teeth and soft tissues of the mouth, general physical examination, eye examination and blood tests and will fill out a questionnaire on oral health and function. In addition, they will have the following tests and procedures:

- Identification of possible fungal infection - Patients rinse their mouth with 2 teaspoons of a salt-water solution and spit it in a sterile container for laboratory examination. If a fungal infection is detected, treatment will be offered.

- Unstimulated salivary function assessment - Saliva production is measured by collecting saliva samples through small suction cups connected to collection tubes over the salivary gland ducts in the mouth.

- Stimulated salivary function assessment - A sour-tasting liquid (2% citric acid) is applied to the top and sides of the tongue at 30-second intervals to stimulation saliva production while saliva is collected using the procedure described above.

- Identification of markers of precancerous lesions - The salivary gland biopsy done at the screening evaluation (or from outside sources) is examined for markers of precancerous lesions, as about 5 percent of patients with Sjogren's syndrome develop a tumor called Non-Hodgkin's lymphoma. In some cases, the minor salivary glands may be re-biopsied a few years after the screening biopsy.

Patients will be followed once a year with a comprehensive history and physical examination, eye examination, full oral examination, salivary function assessment and questionnaires about signs and symptoms of salivary gland dysfunction.

Sponsoring Institute:

National Institute of Dental And Craniofacial Research (NIDCR)

Recruitment Detail

Type: Active Accrual Of New Subjects

Gender: Male & Female

Referral Letter Required: No

Population Exclusion(s): None

Eligibility 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).

Our diagnostic criteria for SS require the presence of:

Positive focus score greater than 1 (aggregation of greater than 50 lymphocytes in 4 mm biopsy of minor salivary gland); and

Schirmer-I test less than 5 mm wetting in 5 minutes, or Lissamine green staining applying the Oxford score, if at least one of the two components score positive: a) a corneal component score of 1 on a 0-5 score, b) a conjunctival component score of 3 (on a 0-10 scale) for any eye. Cases with punctual plug or punctual occlusion performed for dry eyes will be considered to have dry eyes (KCS) if epiphora are not present; and

Positive immunological findings (Igs, RF, anti-SSA, anti-SSB, or ANA).

The incomplete SS are a group of patients who have some objective evidence of an autoimmune exocrinopathy. This group will be followed for possible development of SS.

Objective findings of autoimmune exocrinopathy include positive keratoconjunctivitis sicca (KCS) or low Schirmer-I test, a labial minor salivary gland demonstrating focal infiltrate of lymphocytes replacing normal salivary acini and ducts, or positive immunological laboratory findings.

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.

Special Instructions: Currently Not Provided

Keywords:

Sjogren's Syndrome

Xerostomia

B-Cell Dysregulation

Lymphoma

Candidiasis

Oral Dryness

Salivary Function

Recruitment Keywords:

None

Conditions:

Lymphoma

Salivary Gland Disease

Sjogren's Syndrome

Xerostomia

Investigational Drug(s):

None

Investigational Device(s):

None

Contacts:

Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Electronic Mail:prpl@mail.cc.nih.gov

Citations:

Sjogren's syndrome in childhood

Sjogren's syndrome in patients with newly diagnosed untreated non-Hodgkin's lymphoma

Serum anti-SS-B/La and IgA rheumatoid factor are markers of salivary gland disease activity in primary Sjogren's syndrome

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