Sjogren's syndrome is an illness named for the Swedish physician Henrik Sjögren, who first described it. (For those interested in details, in English, the name is pronounced as “show-grin.” In Swedish, spelled with the umlaut over the “o,” it is pronounced “ho-gruen.”)
While there are several ways to define a syndrome, in the medical context a syndrome is generally a cluster of symptoms that occur together. The defining cluster of symptoms for Sjogren’s includes the following:
If an individual has just those symptoms without evidence of another autoimmune disease they are said to have primary Sjogren’s. Often there are additional symptoms, such as arthritis, enlarged lymph nodes, or enlarged salivary glands.
The majority of patients with Sjogren’s syndrome experience those symptoms in conjunction with another autoimmune disease, in which case it is considered secondary Sjogren’s. Antibodies are tags in your body that mark material (usually foreign) that your immune system will then attack. However, in the case of autoimmune diseases such as Sjogren’s and lupus, the antibodies tag your own cells.
The antibodies Anti-SSA (Ro) and Anti-SSB (La) are present in 70% to 80% of individuals with Sjogren’s syndrome; about 30% of people living with lupus show these antibodies as well. Most often, the co-occurring autoimmune disease is rheumatoid arthritis. Other individuals with secondary Sjogren’s may have lupus, scleroderma, primary biliary cirrhosis, or a different kind of autoimmune disease.
Sjogren’s syndrome has a very slow, insidious onset. About 90% of individuals with Sjogren’s symptoms are women, and the syndrome tends to aggregate to older individuals. Unlike lupus, Sjogren’s afflicts all races equally.
Because Sjogren’s is so commonly associated with other autoimmune diseases, your doctor may run a variety of tests to rule out other conditions. If your doctor is concerned that you may have Sjogren’s, there are several diagnostic tests that can be performed, on the eyes, mouth, or through a battery of blood tests.
Over the long term, Sjogren’s can cause complications that affect the teeth, eyes, kidneys, lungs, and joints. It can also cause complications in children of pregnant women who have anti-SSA (Ro) antibodies, about one quarter of whom will develop a transient rash at birth (“neonatal lupus”) and about 1% of whom will develop a heart problem. In rare cases, those with Sjogren’s can also develop lymphoma.
Dr. Lockshin outlined some potential treatments and management tips for many of the systems Sjogren’s can impact. For Sjogren’s that is systemic, a steroid such as prednisone can be used effectively, as can conventional immunosuppressive agents like methotrexate and mycophenolate, TNF Alpha inhibitors and the newly available JAK inhibitors combat the inflammatory response that occurs with autoimmune disorders. Rituximab (commonly known as Rituxan) can also be used to treat systemic Sjogren’s.
If Sjogren’s is impacting the eyes, regular over-the-counter eye drops may work effectively to manage the dryness. If the dryness is more severe, you may be able to get a prescription for Restasis, which helps your eyes produce more tears naturally. Dr. Lockshin added that devices such as humidifiers and eye shields might help combat the dryness someone with Sjogren’s syndrome may experience.
There are also surgical options, such as inserting a punctal plug. This is a small device that is placed into the tear duct of the eye to block the drainage of liquid, allowing the eye to retrain the tears it is producing.
To treat the symptoms associated with Sjogren’s and the mouth, Dr. Lockshin stressed it is important to practice good dental hygiene, such as regular brushing and flossing. Pilocarpine hydrochloride (Salagen) is a prescription drug used to treat dry mouth by stimulating the saliva glands in your mouth. Vitamin E, if you puncture the capsule to let the liquid flow in the mouth, is also effective in combating the dry mouth that people living with Sjogren’s may experience.
Some other tips that Dr. Lockshin recommended to reduce discomfort of the mouth are chewing sugar free gum or to suck on a hard (sugar free) candy, fruit pits, or lemon rinds. These activities will increase saliva production and thus reduce discomfort from dry mouth.
Finally, Dr. Lockshin spoke about some new agents for lupus and RA that are in development.
As with rituximab, which the FDA has approved for rheumatoid arthritis, physicians in selected cases have tried such treatments for Sjogren's. Belimumab, approved by the FDA for lupus, might turn out to be helpful in Sjogren's syndrome. As more agents become available, they may make a huge difference in the life of someone living with Sjogren’s.
For now, even though Sjogren’s is not curable, it is manageable. It is not a disease that generally progresses, and it may remit spontaneously.
Summary of a presentation given at the SLE Workshop, a free support and education group held monthly for people with lupus and their families/friends. Original presentation made 10/27/2011.
Summary by Lysa Silverstein, Social Work Intern and SLE Workshop Coordinator
Michael D. Lockshin, MD
Director Emeritus, Barbara Volcker Center for Women and Rheumatic Disease