Pharmacotherapy of Xerostomia in Primary Sjogren's Syndrome

Geoffrey C. Wall, Pharm.D., Michelle L. Magarity, Pharm.D., Jeffrey W. Jundt, M.D.

Disclosures

Pharmacotherapy. 2002;22(5) 

In This Article

Diagnosis

Primary SJS is most prevalent in women, with a gender ratio of 9:1. It may occur at any age but is most common in the fourth and fifth decades of life. The incidence varies greatly from 1/100 to 1/1000 people, depending on diagnostic criteria. The San Diego (SD) and European Epidemiology Center (EEC, also called European Community) criteria are used most often to make a diagnosis of primary or secondary SJS.[1,4]

  1. Symptoms and objective signs of ocular dryness

    1. Schirmer's test less than 8 mm wetting/5 minutes and

    2. Positive rose bengal or staining of cornea or conjunctiva to indicate keratoconjunctivitis sicca

  2. Symptoms and objective signs of dry mouth

    1. Decreased parotid flow rate using Lashley cups or other methods and

    2. Abnormal biopsy of minor salivary gland (focus score of ≥ 2 based on average of four evaluable lobules)

  3. Evidence of a systemic autoimmune disorder

    1. Elevated rheumatoid factor ≥ 1:320 or

    2. Elevated antinuclear antibody (ANA) ≥ 1:320 or

    3. Presence of anti-SS-A (Ro) or anti-SS-B (La) antibodies

  4. Secondary Sjögren's syndrome requires signs and symptoms above plus clinical features sufficient to allow diagnosis of rheumatoid arthritis, systemic lupus erythematosus, polymyositis, systemic sclerosis, or biliary cirrhosis

  5. Exclusion criteria: sarcoidosis, preexistent lymphoma, human immunodeficiency virus (HIV) infection, or other known causes of keratitis sicca or salivary gland enlargement

  1. Ocular symptoms (at least one)

    1. Daily, persistent, troublesome dry eyes for more than 3 months

    2. Recurrent sensation of sand or gravel in the eyes

    3. Use of a tear substitute more than 3 times/day

  2. Oral symptoms (at least one)

    1. Daily feeling of dry mouth for at least 3 months

    2. Recurrent feeling of swollen salivary glands as an adult

    3. Drinking liquids to help wash down dry foods

  3. Objective evidence of dry eyes (at least one)

    1. Schirmer I test 5 mm or below/5 minutes

    2. Rose bengal score of 4 or greater according to van Bijsterveld system

  4. Histopathologic signs

    1. Minor salivary gland biopsy with focus score of 1 or greater

  5. Objective evidence of salivary gland involvement (at least one)

    1. Salivary gland scintigraphy

    2. Parotid sialography

    3. Unstimulated whole sialometry 1.5 ml/15 minutes or below

  6. Laboratory abnormality (at least one)

    1. Anti-SS-A or anti-SS-B antibody

    2. ANA

    3. IgM rheumatoid factor

  7. Exclusion criteria: preexisting lymphoma, acquired immunodeficiency syndrome, sarcoidosis, graft-versus-host disease

Not all patients fulfill both sets of criteria. It is estimated that only 10-15% of those who meet SD criteria also satisfy EEC requirements.

[3]

A third joint classification system is being developed to combine elements of both sets to allow for greater sensitivity while maintaining a moderate degree of specificity.

[5]

Evaluation of xerostomia in a patient suspected of having SJS begins with a thorough history and physical examination. Cofactors that may cause or aggravate symptoms include smoking, dehydration, and low-humidity environment. Many drugs with and without anticholinergic properties may induce xerostomia.[1] Product information from many drugs lists this as a possible adverse effect. Difficulty eating and talking may be the initial complaints, followed by recurrent oral infections and progressive dental caries. Persistent parotid swelling, especially when asymmetric, may be an indicator of an underlying bacterial infection or lymphoma. Serologic studies may point to the diagnosis and also help differentiate between primary and secondary SJS and other causes of xerostomia. Several autoantibodies may be present, such as ANA, single-stranded anti-DNA antibodies SS-A(Ro) and SS-B(La), and rheumatoid factor. Salivary flow rates and minor salivary gland biopsies usually are done in the research setting. They also may be of benefit in difficult-to-diagnose cases and to measure symptomatic and immune responses to therapy.

Xerostomia may occur in disorders such as HIV infection, sarcoidosis, amyloidosis, and depression, and after radiation therapy of the head and neck area. This further underscores the need for a thorough history and physical evaluation of these patients.[6,7]

Saliva is a vital body fluid. Without it, continued healthy functioning of the oral cavity would not be possible; for example, teeth would be subject to demineralization.[8] Saliva's primary components are the digestive enzymes lingual lipase and ptyalin (salivary a-amylase), mucins, immunoglobulin A, and electrolytes (sodium, potassium, chloride).[9] The salivary glands secrete about 1000 ml of saliva/day. Saliva has lubricating, cleansing, and antimicrobial properties. It maintains normal balance of oral flora, protecting mucosa from overgrowth of potentially pathogenic organisms, most notably Candida species. Of course it plays a crucial role in proper disintegration and digestion of food.

The viscosity of saliva is much higher than that of plain water due to the presence of various glycoproteins and immunoglobulins. These and other chemical characteristics give saliva its coating and lubricating properties. Saliva also contains various antimicrobial enzymes, calcium and phosphate ions, and several pH buffers.

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