Sjögren's Syndrome: Seeing the Symptoms

Diagnosing Sjögren's is notoriously difficult, but recognizing the signs and knowing when to defer to another specialist can help.

By Peter Donshik, MD

It is estimated that approximately four million people in the United States are living with Sjögren's syndrome, a chronic autoimmune disease. Despite the fact that it is one of the most prevalent autoimmune disorders (second only to rheumatoid arthritis), there is a lack of awareness of the disease—even within the healthcare industry—and thus it goes undiagnosed and untreated. Often, the ophthalmologist can suspect the diagnosis; however, it can take years to make a definitive diagnosis.

About 90% of the sufferers are women and the onset is usually between 40 and 60 years of age, but it may affect any age group. There are two classifications—primary Sjögren's syndrome, where the patient has keratitis sicca (dry eyes) either alone or with dry mouth (xerostomia), and secondary Sjögren's syndrome, where the disease is associated with another autoimmune connective tissue disease—often rheumatoid arthritis, lupus or scleroderma. These patients also have a significant risk for non-Hodgkin's lymphoma Thus it is very important that when a patient presents with Sjögren's-like symptoms, a thorough work-up is initiated.

Searching for Signs

In Sjögren's syndrome, the cells in the body's moisture-producing glands are attacked, causing a decrease in tear production. Symptoms of dry eye are often what first bring patients into the ophthalmologist's office. The symptoms consist of burning, dryness, foreign body sensation, itching, sensitivity to light and blurred vision. There is intolerance to certain environmental conditions like wind, drafts, smoke and dry weather. The visual symptoms worsen at the end of the day, especially with close work and reading.

Blepharitis has to be ruled out because this condition can cause similar complaints. In addition, many patients with Sjögren's have associated blepharitis or meibomian gland dysfunction. In the ophthalmic exam, we pay close attention to the lids. The ophthalmic evaluation consists of a detailed history, especially related to dry eye complaints. Tear production is evaluated with the Schirmer test. Less than 5-6 mm of wetting in five minutes is consistent with aqueous-deficient dry eye.

The stability of the tear film must be evaluated. An unstable tear film can be related to either aqueous-deficient dry eye or evaporative dry eye. The presence of ocular surface disease is determined by evaluating the cornea and conjunctiva surface with fluorescein and lissamine green dyes. The determination of tear hyperosmolarity can also help to make the diagnosis of keratitis sicca.

The Diagnostic Trifecta

The diagnosis of Sjögren's can be difficult. A diagnosis can take a very long time—six, seven or even eight years. A certain number of symptoms must be present in order to make a diagnosis of primary or secondary Sjögren's. Since the disease can affect multiple organs, these patients can also present with fatigue, malaise, fever, skin problems and bloody nose. Other disorders present with similar symptoms, so it is important to refer the patient to a rheumatologist to rule out other diseases. Since dry mouth is an important component of this condition, it is important that patients with Sjögren's syndrome be evaluated by a dentist to diagnosis and treat any oral pathology. These patients have an increased incidence of cavities and other dental problems.

Dentists

Dentists may perform a lip biopsy to determine whether or not the salivary glands are involved, and help make the diagnosis. They can also perform a salivary flow test, a sialogram or a salivary scintigraphy.

There have been a few moisture-increasing medications, like pilocarpine (Salagen) and cevimeline (Evoxac) but because they work better at increasing saliva than tear production, they are rarely used by ophthalmologists. They also can have significant side effects, including sweating, flushing and stomach pain. Patients will be directed to increase fluid intake and to stimulate saliva flow to combat dry mouth. There are also artificial saliva products and nasal saline sprays, which can reduce the nose dryness that causes an increase in mouth breathing.

Rheumatologists

If we think systemic medications are needed, we refer them to a rheumatologist, who may decide on the need for Plaquenil, corticosteroids or other medications. Rheumatologists generally have the primary responsibility of diagnosing and managing the non-ocular components of the disease, as well as helping in making the diagnosis of Sjögren's syndrome. They will usually run a number of blood tests to check for certain antibodies.

The five main tests are anti-nuclear antibody (ANA), rheumatoid factor (RF), SS-A and SS-B, erythrocyte sedimentation rate and immunoglobulins. Around 70% of Sjögren's patients will have a positive ANA test, around 60% to 70% will have a positive RF result, 70% will be positive for SS-A and about 40% will be positive for SS-B.

Easing the Dryness

The ophthalmologist's role is to improve ocular discomfort and vision, try to prevent ocular complications and increase quality of life related to visual symptoms. Treatment depends on the severity of the condition. As a first step, ocular lubrication (usually non-preserved artificial tears) is prescribed. Lacrisert can supplement artificial tears by slowly releasing fluid over the course of the day.

Punctal plugs, either for temporary or permanent closure, can be considered to help maintain fluid in the eye. Inflammation can be managed with Restasis. However, only about 20-30% of patients in my practice benefit from this drug. Burning and stinging can be a problem and limit its acceptance. In selected patients, a short course of topical steroids may be indicated. However, there is the potential for raising intraocular pressure and the risk of cataracts and possible infection. For extremely severe ocular surface disease, some centers use autologous serum drops made from the patient's blood diluted with artificial tears. However, since this is a great growth medium, there is potential for infection.

We can have patients try to manage their environment by using a humidifier to increase the moisture in the air and avoiding air conditioning, drafts and wearing makeup. We remind patients to blink more while on the computer to decrease tear evaporation.

There are moist chamber goggles, which are glasses designed so they enclose the eye and are pressed against the face to preserve evaporation.

The data is somewhat sparse on the benefit of nutritional supplements, though there is some rational for increasing omega-3 fatty acids in our diet, either by eating a lot of salmon and walnuts or using omega-3 supplement products. The theoretic rational for omega-3s is that they increase meibomian secretions and possibly have some effect on decreasing the pro-inflammatory mediators and stimulating tear production.

There are drugs under investigation that may be beneficial but haven't yet been approved, such as anti-inflammatory drugs and drugs that increase the secretion of tears or tear components (i.e., the mucus or lipid layer). A variety of these drugs are looking promising as ways to help alleviate the pain of Sjögren's. OM

For more detailed information, refer to the Sjögren's Syndrome Foundation Web site at www.sjogrens.org.

Peter Donshik, MD, has practiced medical and surgical ophthalmology in the greater Hartford area since 1976. He subspecializes in corneal and external diseases, laser vision correction, contact lenses and corneal transplant surgery. A member of numerous societies, he is presently on the board of directors of the Sjögren's Syndrome Foundation and President of the JCAHPO Foundation. He can be reached at pdonshik@snet.net.