Yesterday, I had a female patient complaining of increasingly irritable eyes with symptoms of red, burning eyes and associated fluctuating vision when reading. With examination it became obvious she was suffering from severe dry eye disease (DES). The question was, which of the many causes of dry eye disease were we dealing with in this case?

Going through the many differentials with her it was soon clear that the cause was not one of the common ones like allergies, meibomian gland disease, excessive computer usage, changes in diet or any changes in medication like hormone replacement therapies, anti depressants, blood pressure, etc.

Then as a side note she dropped the possible answer in my lap. “Oh, I was just diagnosed with rheumatoid arthritis“.

“Is your mouth dry?“, I asked. “Yes“, she answered, “How did you know?“. Aghh I thought, one for the Dr House differential white board. “I now know the possible cause of your dry eyes, it’s called Sjögren syndrome! We will need to send you for some blood tests“. That sounded just like Dr Gregory House. Better not insult her though, I don’t have a walking stick to protect myself!

For those who are unfamiliar with this disease, here is a recent article written by Dr Ernest L Bowling in the Optometric Management journal explaining Sjögren syndrome:

“Sjögren syndrome is one of the most common rheumatologic disorders after rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Sjögren Syndrome is a chronic,slowly progressive autoimmune disease that affects the exocrine glands, most commonly the lacrimal and salivary glands. Sjogren syndrome is a clinical condition of aqueous tear deficiency combined with dry mouth (xerostomia).

The syndrome is classified as primary Sjögren syndrome in patients without an associated autoimmune disease, and secondary Sjögren syndrome in patients who have a confirmed autoimmune disease. Secondary Sjögren is associated with rheumatic disorders such as rheumatoid arthritis, as I remembered, and also with systemic lupus erythematosus, polyarteritis, Wegener’s granulomatosis, scleroderma, polymyositis, dermatomyositis, primary biliary cirrhosis and autoimmune thyroid disease.

Since the disease was first identified in 1933 by Dr. Henrik Sjögren, it has been proven to affect virtually every racial and ethnic group and people at any age, but about 90% of those afflicted are women, the majority of whom are older than 40. According to the National Institute of Neurological Disorders and Stroke (NINDS), between 1 and 4 million people in the United States have Sjogren syndrome.

Although the hallmark symptoms are dry eyes and dry mouth, Sjögren may also cause dysfunction of other organs such as the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and the central nervous system. Patients may also experience extreme fatigue and joint pain and have a higher risk of developing lymphoma. Non-Hodgkin lymphoma occurs in 4% to 5% of Sjogren syndrome patients, who are estimated to have a 44 times greater risk of developing that disease than the general population.

Diagnosis

The following tests are commonly used to determine the presence of SS in addition to the patient’s report of symptoms and health history.

Common blood tests include:

• Complete blood cell count often shows anemia. Chronic inflammation in SS can lead to destruction of red blood cells (RBCs) and inhibition of erythropoietin, a hormone produced by the kidneys, which stimulates RBC production.
• Serum erythrocyte sedimentation rate and C-reactive protein are indicators of inflammation and are frequently elevated in patients with SS.
• Serum immunoglobulins are antibodies that are usually elevated in SS.
• Serum antinuclear antibody is elevated in approximately 90% of patients with SS and other immune diseases.
• Serum rheumatoid factor is present in about 60% of patients with SS, as well as with RA.
• Serum Sjögren antibodies (Ro/SS-A and La/SS-B) are more specific for SS but can also be detected in patients with other autoimmune diseases, such as SLE and RA. SS-A and SS-B are sometimes called “Ro antibodies.”

Minor salivary gland biopsy is the best single test to establish a diagnosis of Sjögren syndrome. While this is the most definitive test, performing it is not absolutely necessary from a clinical standpoint.

Patients with Sjögren syndrome are essentially treated symptomatically and observed for the development of other rheumatic disorders or lymphoma. This can be initiated without performing a biopsy. If the diagnosis is in doubt or if a definitive diagnosis is needed, then this is the best test.

Management of Sjogren Syndrome

Goals of ophthalmic management of Sjögren syndrome are: to relieve symptoms, to promote healing of the ocular surface, and to prevent complications such as persistent epitheliopathy and corneal ulcers. The treatment of dry eyes depends on the severity of eye dryness, which is graded according to degree of symptoms, conjunctival injection and staining, corneal damage, tear quality, and lid involvement.

Encourage patients to try over-the-counter artificial tears, which can provide temporary relief of dry eye symptoms, but caution them that if they use artificial tears frequently, they should choose products with special nonirritating preservatives, or those without preservatives. Preservative-free products in single-dose units decrease the risk of microorganism contamination. Ocular lubricants can also be helpful at night but are usually unsuitable for use during waking hours because they tend to cloud vision. Cyclosporine ophthalmic emulsion (Restasis) can relieve dry eye by treating the underlying inflammatory process.

 

Encourage annual ophthalmic exams in addition to exams by the patient’s rheumatologist. Ensure that patients receiving hydroxychloroquine undergo baseline and periodic ophthalmic exams. This medication, used to help manage Sjögren syndrome associated arthritis, can cause retinal damage. Explain the importance of limiting exposure to environmental irritants such as smoke and excessive sunlight that may cause further dryness of the eyes. Wearing sunglasses can help limit the eyes’ exposure to light. Medications such as antihistamines should be evaluated before use because they can cause further dryness of the eyes and other organs.

Scleral contact lenses are beneficial as the lock moisture between the eye surface and the lens keeping the eye lubricated whilst the lens is worn

Dry mouth may be helped by frequent small drinks of water or sugarless chewing gum to stimulate saliva production, and mouth lubricants can be used as necessary. Sialogogues (medications that stimulate saliva production) may help patients who have some remaining functional salivary gland function. Pilocarpine hydrochloride (Salagen) and cevimeline hydrochloride (Evoxac) are two available agents that can reduce oral symptoms and increase salivary secretion.

Dental care is important as those affected are prone to cavities. Pain and other arthritis symptoms are treated with anti-inflammatory medicines, such as aspirin and other NSAIDs. In some severe cases, immunosuppressant drugs and steroids may be indicated.

Conclusion

Sjögren’s syndrome can damage vital organs of the body with symptoms that may remain stable, worsen, or go into remission. Some people may experience only the mild symptoms of dry eyes and mouth, while others go through cycles of good health followed by severe disease. Many patients are able to treat problems symptomatically. Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating.”

So after explaining the basics of Sjögren’s syndrome to the patient, I referred her to the physician to get her blood workup done to confirm the diagnosis. Now, where did I put that bottle of Vicodin?