Sjögren syndrome is a systemic autoimmune disorder that is characterized by the development of autoantibodies that attack the glands producing moisture.
This is a chronic disorder that leads to dry eyes, mouth, skin, and vaginal dryness- collectively known as sicca symptoms. Sicca comes from Latin meaning dry. In addition to dryness, patients can also experience systemic symptoms including joint pain, fatigue, and muscle aches. Rarely it can affect large organs. It is more common in women and is most prevalent between the ages of 40 to 50 years old. Due to the chronic inflammation with activation of the immune system, there is an increased risk of developing lymphoma.
Sjogren syndrome can occur in the setting of other autoimmune diseases like lupus or rheumatoid arthritis. When this occurs it is considered a secondary disorder. When Sjögren Syndrome occurs by itself, it is considered primary.
Who is Sjögren? (SHOW-gren)
Sjogren syndrome was named after a Swedish ophthalmologist Henrik Sjögren who was one of the first physicians to correlate a group of women all having a syndrome of eye dryness, oral dryness, and joint pain in the early 1900s.
Symptoms of Sjögren Syndrome:
Sjogren syndrome leads to severe dry eyes due to auto-antibodies attacking the lacrimal glands. These are the glands that produce tears. People with this disorder are unable to wear contact lenses. There is a gritty sensation or foreign body sensation in the eyes.
The antibodies attack the parotid gland in the mouth and can lead to swelling of the glands in the cheeks. The dryness in the month is severe and best characterized by being unable to eat a cracker without water. This increases the risk of developing cavities.
Dry skin is due to the attack of the oil and sweat glands of the skin. This can lead to irritation and chronic dry skin. Many different types of rashes can also develop due to Sjögren syndrome.
The vagina is another area of the body that can be targeted in Sjögren syndrome. This can lead to painful intercourse.
Sjogren syndrome is a systemic disorder and can affect other organ systems besides the glands. Most commonly patients may have symptoms of joint pain and muscles aches that are related to the inflammation due to the auto-antibodies produced in Sjögren Syndrome. The joint pain can be similar to rheumatoid arthritis leading to pain and swelling of the small joints of the hands. Although joint pain due to Sjögren’s syndrome is not destructive arthritis.
Diagnosis of Sjögren Syndrome
The first step in diagnosis is quantifying the severity of the dryness through history and physical exam. Some tests can be done in the office to evaluate for dryness. An eye doctor should formally evaluate the eyes for any sign of corneal damage under a special light. A dry mouth can be assessed by examination and history of multiple dental cavities.
The next step in diagnosis is looking at lab work for evaluation of auto-antibody production against the glandular tissue. Similar to other autoimmune diseases, it is important to correlate positive auto-antibodies with symptoms. People can have positive antibodies without having the disease. The auto-antibody that is positive in Sjögren syndrome is Anti-Ro/SSA autoantibody. This auto-antibody is part of the diagnosis criteria for Sjögren syndrome. The ANA is another auto-antibody that can be positive, but this is nonspecific and also is positive in other autoimmune diseases as well.
If initial laboratory tests are unrevealing, a biopsy of the salivary gland in the lip can be done to evaluate for inflammation and destruction from the autoimmune process to obtain a diagnosis.
Treatment of Sjögren syndrome is aimed at treating symptoms of dryness and preventing further destruction of the glands. Treatment of dry eyes includes using artificial teardrops, and cyclosporine drops which help decrease the inflammation. Other strategies to help with symptoms of dry eyes include having the tear ducts plugged to promote tear retention, or wearing glasses with side panels.
For treatment of dry mouth, close follow-up with the dentist for dental cleanings is important to prevent cavities. Sugar-free lozenges can stimulate salvia flow along with having small sips of water to improve the sensation of dryness. If symptoms are not improved with lozenges, then oral medications that stimulate salivary glands can be tried. Vaginal dryness can be treated with topical lubricants and estrogen.
If there is extra glandular involvement of the joints and muscles, then further immunosuppression may be required using oral immunosuppressive agents. Plaquenil and Methotrexate are two drugs that are used in Lupus and rheumatoid arthritis respectively that can be tried in cases of systemic symptoms.
To learn more about the diagnosis, management, and treatment of Sjogren Syndrome contact us to schedule an appointment.
Authored by: Kelsey Rigby, DO
Kent Hospital, Internal Medicine Resident