There are currently over 100 different autoimmune diseases, and autoimmunity is considered to be one of the top two reasons for illness in the United States. Approximately 50 million people suffer from an autoimmune disease. Sjogren’s Syndrome is one of the most common as well as the most under-recognized and possibly under-appreciated of the autoimmune diseases.
What is Sjogren’s Syndrome?
In autoimmune disease, the body’s immune system goes awry. Normally it is responsible for protecting the body against foreign invaders like viruses and bacteria etc, much like an army protects their country. When it becomes confused, the immune system begins to recognize organs and tissues as foreign invaders and begins to attack the body.
All autoimmune diseases have the same problem — an errant immune system — but each disease is known by the specific antibodies that attack different specific parts of the body. In the case of Sjogren’s Syndrome, antibodies wage war on the moisture producing glands and mucus membranes of the body. Unfortunately, that is not the end of the story. Although the main targets are these moisture producing glands, the entire body is affected and systemic symptoms can be disabling for some patients.
Just as there is a female preponderance in Migraine Disease, 90% of Sjogren’s patients are female.
Sjogren’s Syndrome is characterized by periods of increased disease activity called a flare. Flares are triggered by many different things that injure or stress the body including UV light from the sun or fluorescent lighting; emotional stress such as the death of a family member, childbirth or relocation; or even physical injury such as that from a car accident or surgery. Many, many things can cause a flare.
Sjogren’s Syndrome can be primary (not associated with another disease/disorder) or it can be secondary (in addition to another autoimmune disease). It is thought that there is a genetic component to Sjogren’s Syndrome.
It’s known that Sjogren’s patients are also at greater risk for Lymphoma.
Hallmark symptoms of Sjogren’s are what you might expect and include dry eyes and dry mouth. However, our bodies are water based, and symptoms of the immune attack on moisture producing glands and mucus membranes means other serious moisture related problems patients struggle with every day including:
- Chronic inflammation
- Corneal ulcerations
- Eye infections
- Dry nose
- Nose bleeds
- Chronic or recurrent sinusitis
- Mouth sores/ulcerations
- Swollen salivary glands
- Salivary gland stones
- Dental decay, dentures
- Problems with speech
- Problems with taste
- Dry, cracked lips that may peel
- Dry skin
- Dry hair
- Difficulty chewing
- Difficulty swallowing
- Chronic or recurrent bronchitis
- Interstitial lung disease
- B12 and other vitamin deficiencies
- Stomach upset
- Personal/vaginal dryness
- Rectal dryness and hemorrhoids
Organ dysfunction is common and can range from mild to life threatening. Systems and organs that are frequently involved include:
- Brain/neurological system
- Blood vessels/vasculitis/Raynaud’s Phenomenon
- Digestive system
- Connective tissues
- Skin, hair and nails
- Reproductive system
Diagnosis is difficult because Sjogren’s symptoms often mimic other diseases and disorders. It also likes to hide. It may take several years after symptoms are apparent before antibodies begin to show themselves in blood work. Some cases of Sjogren’s never have positive blood work. Diagnosis is based on lip biopsies and other tests for symptoms of dryness or immune attack/destruction.
Blood work usually begins with an ANA (Antinuclear antibody) test. If that is positive, specific antibodies are sought. Sjogren’s Syndrome has two antibodies associated with it — for short they’re often called SSA and SSB antibodies. A positive diagnosis is based on the presence of one or more of these antibodies, symptoms and other positive testing.
SSA antibodies are closely linked and often crossover with lupus. In fact, SSA antibodies in a mother carrying a baby can result in heart block and neonatal lupus in the child. These antibodies are associated with some of the systemic symptoms of Sjogren’s Syndrome, especially joint and musculoskeletal problems, and often debilitating fatigue.
SSB antibodies are very specific for Sjogren’s and are unique to the disease with no known crossover with other autoimmune diseases. They are often highly associated with dryness of eyes and mouth, thickened mucosa, as well as inflamed glands etc.
Other blood tests may reveal other abnormalities including irregularities in red blood cell counts, white cell and platelet counts, irregularities in a part of the immune system called Complement, and in markers for chronic inflammation.
The doctor you will see for diagnosis and treatment of Sjogren’s is called a rheumatologist. There are few rheumatologists in the United States that specialize in Sjogren’s Syndrome.
Treatment from a non-Sjogren’s specialist is unfortunately often insufficient. Most non-specialists are dismissive of patient complaints. Sjogren’s is often mistakenly seen as a disease that is “not worth treating”.
Sjogren’s specialists are usually aggressive and treatment starts early, often with a first line drug called Plaquenil — an anti-malarial medicine that is often very helpful for patients. There are rare, but significant side effects to the treatment, and not all patients can tolerate the drug.
Other medicines used for Sjogren’s Syndrome itself include Disease Modifying Anti-rheumatic Drugs (DMARDs). This group of drugs is powerful and designed to inhibit the immune system. Drugs used include steroids and chemotherapy agents often used in cancer patients, as well as biologics — special agents made by using products from animals such as mice.
A new controversial therapy called Low Dose Naltrexone is being used on some patients with some success. This therapy works on the principle that the immune system needs more of certain types of cells to be balanced. It increases the immune system in the areas that are deficient. This acts similarly to giving an army new Generals to tell it what to do. It has few side effects, but is less accepted and more difficult to get.
Symptoms of Sjogren’s are treated as they arise. For example, NSAIDs are common for pain control and to relieve inflammation, although some patients wind up needing more powerful pain medication to maintain quality of life. Medicine to relieve reflux is common. Vitamin deficiencies are treated with supplements. There are medicines to address dry eye, nose and mouth and patients sometimes wear special glasses or goggles and face masks to preserve humidity in these dry areas. Antibiotics may be necessary for infection. Many other symptoms are addressed with medicines to help the patient be more comfortable or to relieve dysfunction caused by the autoimmune attack.
Because of the greatly increased risk of lymphoma, testing is done regularly to monitor for any changes that could indicate a problem early so treatment is more likely to be successful.
Living a life with Sjogren’s is living with a chronic, incurable disease. Although Sjogren’s itself is not usually fatal, it can be debilitating with patients ending up with organ transplants, secondary infections and other serious problems.
Sjogren’s Syndrome and Migraine
Sjogren’s can cause changes in the body that can act as Migraine triggers. Additionally, the medicines patients must take can also often act as triggers. Sjogren’s antibodies themselves can affect the brain and central nervous system.
A Migraine attack and the medicines used to treat it cause stress on the body that can often trigger an autoimmune flare. This can often be like a snowball down a hill when the autoimmune flare then triggers another Migraine, which triggers further autoimmune action against the body, and on and on and on.
If Migraineurs have to change their lives to try and minimize triggers and subsequent Migraine attacks, the Sjogren’s patient has to work that much harder to minimize triggers for both their conditions. Additionally, just as non-specialists are often not well versed on Migraine Disease, non specialists are also not well versed on Sjogren’s. These patients often find themselves traumatized by their diseases, but also by the health care system at whom they’re looking to for help.
How do I know so much about Sjogren’s Syndrome? In addition to being a Migraineur, I am a Sjogren’s Syndrome patient.
Do you know someone with Migraine and Sjogren’s Syndrome? What are their symptoms like? Their triggers? What do they do to prevent autoimmune flares and Migraine attacks? How do they deal with stigma?
If you are a Migraineur dealing with Sjogren’s Syndrome, what words of advice do you have for others who may need help learning how to live with these two comorbid conditions?