What is Sjogren’s syndrome?
Sjogren’s syndrome is a chronic autoimmune condition. It is marked by inflammation in several glands in the body. Glands producing saliva and tears are most often affected. Like many other autoimmune diseases, most people with Sjogren’s syndrome are women. Sjogren’s can affect people of all ages and may accompany other autoimmune diseases like systemic lupus erythematosus or rheumatoid arthritis (RA).
What are the signs and symptoms of Sjogren’s syndrome?
The symptoms of Sjogren’s syndrome can vary greatly from patient to patient. Its hallmarks are dry eyes and dry mouth (called sicca symptoms). Patients also commonly develop fatigue and joint pain. Sjogren’s syndrome can affect most organs in the body, including the lungs, kidneys, nervous system, blood vessels and gastrointestinal tract, though these manifestations are rare.
How is Sjogren’s syndrome diagnosed?
There is no single test to confirm a diagnosis of Sjogren’s syndrome. Because symptoms can be nonspecific and involve several organ systems, it is often misdiagnosed or goes undiagnosed for several years. A diagnosis requires
- symptoms, such as sicca
- a positive autoantibody test or gland biopsy
- objective evidence of gland dysfunction
An eye doctor can perform several tests to show lacrimal (tear) gland dysfunction in people with dry eyes. These include a direct measure of tear production (called a Schirmer’s test) or use of special eye drops to identify dry spots on the surface of the eye.
Tests can be performed on the salivary gland to see saliva flow through the gland (sialography) or to find problems with the gland’s structure. In some cases, a biopsy of a salivary gland, usually from the lip, is needed to make the Sjogren’s diagnosis. A positive biopsy will show evidence of inflammation.
Are there treatments for Sjogren’s syndrome?
There is currently no cure for Sjogren’s syndrome. Treatment is generally aimed at controlling symptoms and targeting specific organ involvement. Rheumatologists, ophthalmologists, dentists and other specialists often work together to manage Sjogren’s syndrome and monitor response to therapy.
All people with Sjogren’s syndrome should have their medication list reviewed with a doctor to ensure they are not on medications that can make dryness worse. This includes discussion of all over-the-counter medication, including antihistamines or sleep aids.
Over-the-counter eye drops can help with mild cases of dry eye. (Drops should be preservative-free artificial tears.) Longer-lasting lubricating ointments or gels can be helpful overnight. There are also prescription-strength eye drops that an ophthalmologist might prescribe if the over-the-counter drops are not enough. Eye doctors sometimes insert punctual plugs, which block the normal drainage of tears and prolong the time that tears remain on the surface of the eye. Dry, windy or polluted environments can worsen dry eye. Exposure to these environments should be limited when possible. Wearing sunglasses to protect the eyes, especially on windy days, can be helpful as well.
The management of dry mouth includes staying well hydrated, using lubricating agents such as over-the-counter alcohol-free mouthwashes or artificial saliva and avoiding medications that can make dry mouth worse. Chewing sugar-free gum or sucking on sugar-free candies can help stimulate saliva flow. (Look for products that contain Xylitol.) There are two prescription medications (cevimeline and pilocarpine) that can help some people with dry mouth. Sleeping with a humidifier may also be helpful to ease the sicca symptoms. People with Sjogren’s should not smoke cigarettes as this can make dry eye and dry mouth worse. Exposure to secondhand smoke should also be avoided.
People with severe forms may require therapy to suppress the immune system. This could include corticosteroids or other medications aimed at reducing inflammation.
Recently, medications targeting B cells, a specific type of white blood cell, have been studied in Sjogren’s syndrome. These medications may be helpful in controlling certain severe types of the disease. Ongoing research is aimed at finding new therapies to help treat all forms.
Dr. Lindsay Lally is a rheumatologist at Hospital for Special Surgery (HSS). Dr. Lally specializes in the care of patients with various rheumatic conditions including vasculitis, systemic lupus erythematosus, scleroderma and rheumatoid arthritis and has a particular interest in the systemic vasculitides.