Sarcoidosis, preexisting lymphoma, HIV infection, hepatitis virus B or C infection, primary fibromyalgia, and other known causes of autonomic neuropathy, keratitis sicca, or salivary gland enlargement

*Definite Sjogren's syndrome requires objective evidence of dryness of eyes and mouth and autoimmunity, including a characteristic minor salivary gland biopsy (criteria IA, IB, and IC). Probable Sjogren's syndrome does not require a minor salivary gland biopsy but can be diagnosed by demonstrating decreased salivary function (criteria IA, IB-1, and IC).

From Fox RI, Saito I. Criteria for diagnosis of Sjogren's syndrome. Rheum Dis Clin North Am 1994;20:391.

A diagnosis of definite SS requires a minor salivary gland biopsy; a probable SS diagnosis can be based on demonstrating decreased salivary function. Exclusions to the diagnosis based on the San Diego Criteria include patients with HIV, primary fibromyalgia, sarcoidosis, preexisting lymphoma, keratitis sicca, hepatitis B or C, or salivary gland enlargement (Box 32-4). Objective evidence of ocular dryness can be obtained by the Schirmer II test, which involves stimulating the nasolacrimal reflex by inserting a cotton swab into the nostril; the increase in tear flow is then measured for both eyes (Tsubota, 1991). Rose bengal staining of the corneal or conjunctiva epithelial layer is another objective test. Serologic evidence of SS includes an elevated RF (>1:320), elevated ANA (>1:320), or presence of anti-SS-A (Ro) or anti-SS-B (La) antibodies.

Salivary secretions can be quantified by asking the patient to suck on a sugarless piece of candy for 3 minutes and then expectorate. If there is very little or no expectorant, a probable diagnosis of SS can be made; a follow-up minor salivary gland biopsy is definitively diagnostic. Patients not fitting these objective criteria can be reassured that their dry eyes or mouth is most likely not caused by an autoimmune disorder. Other manifestations of SS include constitutional symptoms such as fatigue, dry skin, vaginal dryness, upper respiratory tract dry-ness, and difficulty swallowing (caused by decreased saliva).

SS is not the only entity that can cause enlarged lacrimal and salivary glands and glandular dysfunction. SS needs to be differentiated from infiltrative processes (lymphoma, sarcoidosis, fatty infiltrates, hemochromatosis); infectious diseases (blepharitis, HIV, hepatitis B and C, tuberculosis, syphilis); neuropathic dysfunction of the glands caused by multiple sclerosis or Bell's palsy; autonomic neuropathy; and drug side effects.

Treatment of keratoconjunctivitis sicca consists of the use of artificial tears while monitoring for blepharitis caused by preservatives used in these preparations. Punctal plugs can be used to increase eye moisture, with either collagen plugs that dissolve after 2 days or silicone plugs, which are more durable but can be removed if excessive tearing occurs. If this is helpful, permanent punctal occlusion surgery might be considered. Humidifiers may also be helpful. Cyclosporine ophthalmic preparations are FDA approved for treatment of keratoconjunctivitis sicca, with objective and subjective improvement in dry eyes (Sall et al., 2000).

Dry mouth can be relieved by sugarless mints or gum. Frequent sips of water are also helpful. Those with continued symptoms may be candidates to try artificial saliva solutions (Salivart, Mouth Kote). Meticulous dental care should be encouraged because caries can occur more frequently with reduced salivary flow. Secretagogues such as pilocarpine or cevimeline can be used, but adverse effects such as flushing, increased perspiration, and increased bowel or bladder motility might outweigh their benefits. Topical antifungal troches are useful for low-grade oral infections.

Systemic treatment of SS is similar to that of SLE. NSAIDs and antimalarials, in particular, are useful for concomitant arthralgias and other systemic symptoms, but corticosteroids and immunosuppressants might be needed for refractory severe systemic disease.

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