Sjogrens Syndrome

Sjogrens Syndrome

Sjogrens Syndrome

Gary N. Foulks, MD, FACS
     
Because dry eye can be the first and/or most troubling manifestation of Sjogrens syndrome, patients with this condition frequently seek treatment from an eyecare specialist, often before seeking care elsewhere. For this reason, eyecare practitioners should consider the possibility of Sjogrens syndrome whenever patients present with severe dry eye.
     
Historically defined as a condition in which patients had dry eye, dry mouth, and arthritis, Sjogrens syndrome is now known to be a systemic autoimmune disease that causes exocrinopathies (which can affect saliva, tears, and other mucous membrane secretions) and other autoimmune problems. In Sjogrens patients, autoantibodies are directed toward several proteins including the muscarinic receptors that control tear secretion, resulting in inflammation of the lacrimal gland and reduced production of aqueous tears. 

Diagnosing Sjogrens Syndrome
     
Although patients with Sjogrens syndrome often present with signs and symptoms similar to other dry eye conditions, treatment of Sjogrens syndrome may require additional measures to address its systemic components. Fortunately, certain features can help to distinguish Sjogrens-associated dry eye from other aqueous-deficient states.
     
The diagnosis of Sjogrens syndrome is facilitated by a schema called the European-American Consolidated Diagnostic Criteria, in which a Sjogrens diagnosis requires at least four of the following six features: symptoms of dry eye, symptoms of dry mouth, physical evidence of dry eye, physical evidence of dry mouth, serological evidence of Sjogrens syndrome, or evidence of inflammation on minor salivary gland biopsy.1
     
Since some serologic tests are performed only as part of a full rheumatologic workup, eyecare providers must often make a tentative diagnosis based on clinical presentation. In addition to signs and symptoms of dry eye and dry mouth, clinicians should look for evidence of other autoimmune disease—such as arthritis, inflammatory bowel disease, or thyroiditis. Nonspecific symptoms that may also be associated with Sjogrens syndrome include Raynauds phenomenon, restless leg syndrome, and mental confusion (brain fog). While these clinical indicators are not always definitive, they may suggest that a more comprehensive workup is needed. Sjogrens syndrome patients are also at higher risk of developing lymphoma, so clinicians need to be alert to this condition.

Treatment Options
     
Much of the initial treatment for Sjogrens syndrome is directed toward controlling the symptoms of dry eye and dry mouth, since these are usually patients’ most significant complaints, but systemic treatments may also be necessary to address the underlying autoimmune condition.
     
Ocular symptoms of Sjogrens syndrome are often treated with topical cyclosporine (Restasis®; Allergan) to reduce ocular surface inflammation and oral drugs such as pilocarpine and cevimeline (Evoxac®; Daiichi Sankyo) to stimulate the production of tears and saliva. As with treatment for non-Sjogrens aqueous-deficient dry eye, use of lubricants and enhanced tear substitutes can help to improve ocular surface comfort. Environmental modifications (eg, eliminating drafts or installing a humidifier) are also helpful.
    
When more aggressive treatment is needed, or when patients have a number of symptoms other than dry eye and dry mouth, systemic treatment should be considered. This may include systemic corticosteroids to reduce inflammation, disease modifying antirheumatic drugs such as hydroxychloroquine sulfate (Plaquenil®; sanofi-aventis), and/or immunosuppressive medications such as cyclosporine, tacrolimus, infliximab, and rituximab. 

Gary N. Foulks, MD, FACS, is the Arthur and Virginia Keeney professor of ophthalmology, University of Louisville, KY, and is editor-in-chief of The Ocular Surface. 

REFERENCES

1. Vitali C, Bombardieri S, Jonsson R, et al; European Study Group on Classification Criteria for Sjogrens Syndrome. Classification criteria for Sjogrens syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002 Jun;61(6):554-8.