Sjögren's Syndrome

Signs and Symptoms: More than 90 % of Sjögren's syndrome patients are female of any racial or ethnic background.1 Some patients manifest the disorder in their mid- to late 20s, and pregnancy may actually be a contributory factor. One fourth to one half of Sjögren's patients suffer from rheumatoid arthritis.1,2 Other disorders may include systemic lupus, polymyositis, thyroiditis, scleroderma, hypergammaglobulinemia, anemia and chronic fatigue syndrome.

The most common symptoms of Sjögren's syndrome involve dry eye and dry mouth. Often the first clinical manifestation is keratoconjunctivitis sicca (KCS). Patients with KCS present with mild to severe ocular burning and discomfort, and report a sandy or gritty feeling. Examination may reveal a diminished tear meniscus, epithelial corneal stippling, reduced fluorescein tear break up time (FTBUT), and rose bengal or lissamine green staining in the band region of the conjunctiva. Tear volume assessment via Schirmer test or Quick Zone (phenol red thread test) will show significant reduction. Mucous filaments often appear within the tear film. when these become large and adhere to the corneal epithelium, filamentary keratitis may result. Associated ocular findings may include staphylococcal blepharitis, ropy mucous strand accumulation, meibomian gland dysfunction and corneal pannus in extreme cases.

Dry mouth or xerostomia results in complaints of dysphagia, loss of taste and painful lesions of the lips and tongue, and enhances the likelihood of tooth decay. Hoarseness and a dry cough may be signs of dry mouth extending to the oropharynx. In the later stages, parotid gland enlargement may cause a "chipmunk" appearance of the face.

Sjögren's syndrome may impair neurologic, pulmonary, rheumatologic, dermatologic, renal and genitourinary systems. Other symptoms include dry skin or skin rashes, pain or weakness in joints or muscles, numbness or tingling in the extremities, labored breathing, vaginal dryness, difficulties with memory and fatigue.

Pathophysiology: Lymphocytic infiltration of the exocrine glands characterizes this chronic disorder. Sjögren's is said to be the second most common autoimmune rheumatic disease after rheumatoid arthritis. Prevalence in the United States is estimated at between 500,000 and 2 million.3 Researchers speculate that the condition results from a combination of genetic and environmental factors.

There are two forms of Sjögren's. The primary form is generally absent of other autoimmune conditions. Secondary Sjögren's is typically associated with pre-existing systemic disorders such as rheumatoid arthritis or SLE.

Management: While there is no cure for Sjögren's syndrome, early diagnosis is important. Many therapies may significantly reduce the symptoms. Studies show that about 5-8% of patients develop malignant lymphoma as a late complication.4

When suspected, consider Sjögren's syndrome in female patients with dry eye. Refer these patients for a full rheumatologic exam, as well as a consult with a dentist or oral surgeon skilled in managing Sjögren's syndrome. Laboratory testing may also be in order. Consider the antinuclear antibody (ANA), Sjögren's specific antibodies (SS-A and SS-B), rheumatoid factor (RF), erythrocyte sedimentation rate (ESR). Serological testing may reveal hypergammaglobulinemia, cryoglobulinemia and thyroid autoantibodies.

Aim your treatment plan at reducing symptoms to improve the patient's day-to-day functioning. Manage dry eye with tear replacement therapy (non-preserved preparations, 4-6 times daily), and punctal occlusion as a second step. Manage more severe keratitis sicca with low-dose topical corticosteroids, moisture-retaining goggles or tarsorrhaphy. Topical Restasis (cyclosporin A, Allergan) may offer relief for some patients with KCS, though this drug is still under consideration by the FDA.

Oral Salagen (pilocarpine, MGI Pharma) and Evoxac (cevimeline, SnowBrand Pharmaceuticals) may offer relief for both dry mouth and dry eye. Plaquenil (hydroxychloroquine, Sanofi) may delay the progression of Sjögren's and reduce the severity of associated symptoms. Many patients require anti-inflammatory therapies, including both NSAIDs and corticosteroids in some cases.

Clinical pearls:

  • Because of the high association with rheumatoid arthritis, examine the hands of Sjögren's suspects for enlargement of the knuckles and/or a "gnarled" appearance of the fingers.
  • Advise patients to sip fluids (preferably water) and suck on lozenges throughout the day. Caution them to avoid sugar because they are prone to rapid tooth decay.
  • A small "table top" humidifier may be a simple yet substantial way of ameliorating symptoms.
  • Sjögren's syndrome is a multisystem disorder that one must comanage with a variety of medical specialists. Always stress regular dental and rheumatological care.

1. Mahoney BP. Sjögren's syndrome. In Marks ES, Adamcyzk DT, Thomann KH, eds. Primary Eyecare in Systemic Disease. Norwalk, Conn: Appleton & Lange 1995:250-254.
2. Mahoney BP. Rheumatologic Disease. In Blaustein BH, ed. Ocular Manifestations of Systemic Disease. New York: Churchill Livingstone 1994:61-80.
3. Fox RI, Saito I. Criteria for diagnosis of Sjögren's syndrome. Rheum Dis Clin North Am 1994 May;20:391-407.
4. Fox RI: Treatment of the patient with Sjögren's syndrome. Rheum Dis Clin North Am 1992 Aug;18:699-709.

Other reports in this section

Eyelids & Eyelashes | Conjunctiva & Sclera | Cornea
Uvea | Vitreous & Retina | Optic Nerve & Brain | Oculosystemic Disease

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