Most patients don't come to the doctor complaining of dry eyes or dry mouth. Most take it for granted that these may be normal symptoms. Dry eyes may first become apparent to those who use contact lens when these become more difficult to use and the patient requires more eye drops. Others may note an increased amount of matter in their eyes in the morning or their eyes may become more irritable and even teary due to the irritation. Symptoms of dry mouth may be even more subtle. The patient may become accustomed to carrying a water bottle with them or keeping water at their bedside. Generally a good question to elicit the presence of dry mouth is whether one can eat two dry crackers without the use of water. In moderate to advanced stages, most if not all will not be able to perform this task. Other symptoms associated with SS may be joint pain. This will need the expertise of a physician to determine if this is arthritis or another entity.

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Complete blood count may be normal or show evidence of low grade anemia. The white blood cell count as well as the platelet count may be low as well.

The antinuclear antibody (ANA) is generally positive in a high titer with a speckled pattern. The blood chemistries including kidney and liver function are generally normal but occasionally the liver transaminase enzymes are elevated. Renal function which includes three tests, BUN (blood urea nitrogen), creatinine, and urine analysis is generally normal, except in cases when SS is seen with a lupus patient who has renal disease. In these patients one or more of these tests are abnormal. Some patients have a type of kidney involvement referred to as renal tubular acidosis, and a high potassium and a low bicarbonate may be found by blood tests.

SSA(Ro)/SSB(La) antibodies (Sjogren's antibodies) are found to be positive in as many as 60-70% of patients with Sjogren's syndrome; however they need not be positive in order to establish a diagnosis. Thyroid studies may be abnormal in as many as 30% of patients with SS usually with an elevated thyroid stimulation hormone (TSH) consistent with hypothyroidism.

X-rays of joints in patients with primarily SS are normal. If lung involvement is present a chest x-ray may show interstitial lung disease.

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The diagnosis of SS can generally be made on the basis of tests described below.

1. A Schirmer's test is performed by placing a pre-measured strip of filter paper and then measuring the amount of wetting that occurs over a five minute period. Less than 5 mm of wetting generally indicates the presence of dry eyes (xerophthalmia). This test can have as much as a 15% false negative or positive rate.
2. Rose-Bengal Dye test is applied topically and then the eye is examined under a special slit lamp. The damaged epithelium of the cornea and conjunctiva stains positively and is indicative of dry eyes. There is a 5% error in testing.
3. Minor salivary gland biopsy is performed to detect the presence of dry mouth (xerostomia). Generally a biopsy of the minor salivary glands taken from the inner side of the lower lip is performed under local anesthesia. The biopsy then is prepared and stained and examined for inflammatory infiltrates. These inflammatory infiltrates contain T cells, B cells, and macrophages. The aggregates of cells are characteristic of SS and are graded by a standard scale known as the Greenspan Scale. A trained oral pathologist can determine if the minor salivary gland findings fulfill criteria for the diagnosis of SS.

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Education is the first step in the treatment of Sjogren's syndrome. Care of the eyes and oral cavity are very important. Dry mouth can be treated with Salagen (pilocarpine) which stimulates the flow of saliva to the mouth. The most common drug-related adverse events with Salagen include sweating, urinary frequency, chills and flushing. Topical eye drops and gel preparations are often necessary for the treatment of dry eyes. Some patients require the use of tear duct plugs to help preserve those few tears produced. Temporary plugs can be tried first before a permanent closure of the ducts is surgically performed. The associated complications of Sjogren's syndrome require individualized treatment. Plaquenil is often used to treat arthritis and fatigue as well as certain skin rashes. Vasculitis which can be limited to the skin can also be successfully treated with Plaquenil in some patients. In severe cases steroids and sometimes cytotoxic agents are used (such as Cytoxan). Every case must be individually assessed since the disease may vary from mild symptoms to more severe forms.

Rodolfo Molina, M.D.

Joel E. Rutstein, M. D.