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Natural History of Salivary Gland Dysfunction and Sjogren's Syndrome

4 Years
Not Enrolling
Lymphoma, Salivary Gland Disease, Sjogren's Syndrome, Xerostomia

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Trial Information

Natural History of Salivary Gland Dysfunction and Sjogren's Syndrome

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
Sjogren'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 Sjogren'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
Sjogren'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.

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)


Past head and neck radiation treatment

Hepatitis C infection

Acquired Immunodeficiency Syndrome (AIDS)

Pre-existing lymphoma


Graft versus host disease

Current use of anticholinergic drugs


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.


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.


Age 18 years or older.

Ability to give informed consent.


History of bleeding diathesis or the current use of anticoagulants.

Any uncontrolled or severe chronic disease.

Type of Study:


Study Design:


Principal Investigator

Ilias G Alevizos, D.M.D.

Investigator Role:

Principal Investigator

Investigator Affiliation:

National Institute of Dental and Craniofacial Research (NIDCR)


United States: Federal Government

Study ID:




Start Date:

April 1999

Completion Date:

Related Keywords:

  • Lymphoma
  • Salivary Gland Disease
  • Sjogren's Syndrome
  • Xerostomia
  • Xerostomia
  • Oral Dryness
  • Salivary Function
  • Lymphoma
  • Salivary Gland Diseases
  • Xerostomia
  • Sjogren's Syndrome



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