Sjogren’s Syndrome

Sjogren’s syndrome (pronounced Show- grins) is an auto immune disease – a disease in which the immune system turns against the body’s own cell. Sjögren’s syndrome is also known as “Mikulicz’s disease” and “Sicca syndrome”.

In Sjorgren’s Syndrome (SS) the immune system attacks the moisture producing glands and causes dryness in the mouth and eyes. In some cases, other parts of the body can be affected as well, resulting in multiple possible symptoms. It was first identified by a Swedish physician, Dr. Henrik Sjögren in 1933.

The immune system is responsible for protecting the body from various diseases by destroying harmful invaders like viruses and bacteria. In SS, the immune system attack the glands that produce tears and saliva (the lacrimal and salivary glands). Damage to these glands keeps them from working properly and causes dry eyes and dry mouth. Dry eyes are called kerato-conjunctivitis sicca, or KCS, and dry mouth is called xero-stomia. This disease can affect other glands too, such as the glands in the stomach, pancreas, and intestines, and can cause dryness in other places that need moisture, such as the nose, throat, airways, and skin. Since, SS causes inflammation in joints, muscles, skin, or other body tissue, it is considered to be a  rheumatic disease. Sjögren’s is also considered a disorder of connective tissue, which is the framework of the body that supports organs and tissues (joints, muscles, and skin).

Between 400,000 to 3.1 million Americans, i.e. about one to two percent of the population is affected by Sjogren’s syndrome. It generally affects people between 45 and 55 years old, although it can affect anyone regardless of age. It is also found mostly in women – women are 10 times more likely to have Sjögren’s than men.

Causes

While it’s not known exactly what causes Sjogren’s syndrome, a combination of the following may be to blame:

• Abnormal immune response
• Sex hormones
• Inheritance or genetics
• Environment (although the exact environmental factors aren’t known)

It is classified in the following :

• Primary – The syndrome is developed by itself and not as the result of another condition. For example, certain people are born with specific genes that make them more vulnerable to a faulty immune system. Then, many years later, an environmental factor, possibly a common virus, triggers the immune system to stop working properly.
• Secondary – The syndrome is developed in combination with another autoimmune disorder, such as lupus or rheumatoid arthritis

The causes are listed as below –

• Autoimmune Factor – Salivary glands that produce saliva exist in “grape-like” clusters. There are no or few lymphocytes in the normal salivary gland but are present in Sjogren’s syndrome. Lymphocytes are part of the immune system that normally protect the body from infection and tumors. When they appear to attack the body’s tissue (as in Sjögren’s syndrome, systemic lupus, or in rheumatoid arthritis), the term “autoimmunity” is used. Lymphocytes originate in the bone marrow. Two types of lymphocytes, termed “T cells” and “B cells” are responsible for mediating immune reactions. The entire lymphoid system is precisely regulated, largely by messenger molecules that instruct cells to “turn on” or “turn off.” Autoimmunity, the excessive reaction against one’s own tissues, then results from a failure of the normal regulation of T cells and B cells. This may be due either to an excessive production of helper signals or a failure to respond to suppressor signals. As a consequence, lymphocytes infiltrate the tissues and attack normal cellular structures.

• Virus & Infections – Studies suggest that a virus is involved. One possible candidate is the Epstein-Barr virus (EBV), which causes infectious mononucleosis, a condition characterized by swollen salivary glands, joint aches and fatigue. Virtually all adults have been infected with EBV by age 20 years. After the initial infection, this virus normally resides in the salivary glands for life but causes no problems. We and others have speculated that this virus (or a closely-related virus) may trigger an autoimmune response in genetically susceptible individuals.

It is thought that an as yet unknown infectious agent damages the salivary gland and attracts the “immune” lymphocytes into the salivary gland. These lymphocytes release specific autoantibodies such as rheumatoid factor (RF) and antinuclear antibodies; antibodies are directed against proteins termed Sjögren’s-associated antigens A and B (or SS-A and SS-B). These antibodies can enter the bloodstream and are measured in the blood tests that we obtain to confirm the diagnosis of Sjögren’s syndrome.

• Hereditary Factor – Particular genes (such as human leukocyte antigen or HLA genes) are inherited in the same manner from parents as are genes for hair color or eye color; that is, one gene from each parent. The HLA genes are important in controlling the immune response and many current research studies are trying to determine exactly how they perform this task. A specific gene named HLA-DR3 is found in high frequency in Caucasian patients with primary Sjögren’s syndrome.

• Evironmental Factors – These include:
  • Toxic Metal Exposure – Studies have shown that exposure to toxic metals such as mercury, cadmium, lead, arsenic, aluminum, nickel and other heavy metals can be linked to the autoimmune process: The heavy metals induce autoantibodies, which then create autoimmune diseases, including Sjogren’s syndrome. These free radicals then alter the body’s pH (which must be kept constant). An altered pH allows viruses, bacteria, candida and other pathogens to thrive, which then sets the stage for more free radicals. The free radicals damage the cells, making it impossible for the cells to communicate with each other. Autoimmunity results when the immune system attacks the damaged cells.
  • Toxic Chemical Exposure – Toxins such as pesticides, solvents, industrial chemicals, even household cleaners and hair dyes are being implicated in autoimmune diseases. These toxins are everywhere, and they greatly increase the risk of all diseases in general.
  • Smoking – Smoking increases the risk of several autoimmune diseases, primarily because of the chemicals in cigarettes. 

• Nutritional Deficiencies – Poor diet is an important factor in autoimmunity because poor nutrition compromises the immune system. Processed foods are loaded with chemicals, hormones, steroids, trans-fats and sugars, which promote the creation of free radicals in the body, which in turn damage the cells.

Symptoms

The main symptoms are

• Dry eyes—Your eyes may be red and burn and itch. People say it feels like they have sand in their eyes. Also, your vision may be blurry, and bright light, especially fluorescent lighting, might bother you.
• Dry mouth—Dry mouth feels like a mouth full of cotton. It’s difficult to swallow, speak, and taste. Your sense of smell can change, and you may develop a dry cough. Also, because you lack the protective effects of saliva, dry mouth increases your chances of developing cavities and mouth infections.

Both primary and secondary Sjögren’s syndrome can affect other parts of the body as well, including the skin, joints, lungs, kidneys, blood vessels, and nervous system, and cause symptoms such as –

• Dry skin
• Skin rashes
• Thyroid problems
• Joint and muscle pain
• Pneumonia
• Vaginal dryness – painful sexual intercourse
• Numbness and tingling in the extremities

When Sjögren’s affects other parts of the body, the condition is called extraglandular involvement because the problems extend beyond the tear and salivary glands. Finally, Sjögren’s can cause extreme fatigue that can seriously interfere with daily life.

Less common features of Sjögren’s syndrome are:

• Irritation of the nerves in the arms, hands, legs, or feet (neuropathy)
• Feeling of numbness or tingling
• Thyroid gland abnormalities
• Skin rashes
• Memory loss, difficulty concentrating or confusion
• Gastrointestinal problems, such as acid reflex, bloating, abdominal pain, or diarrhea
• Inflammation of the lungs, kidneys (unlike lupus nephritis), liver, or pancreas
• Cancer of the lymphatic tissue (occurs in up to 5% of patients with the disease)

Treatment

The goals of treatment are to decrease discomfort and reduce the harmful effects of dryness. Generally, physicians use medications to control symptoms (symptomatic treatment). The type of treatment will be tailored to each patient’s symptoms and needs.

• Good oral hygiene – Good mouth/dental care may prevent or reduce dental decays, infections, or tooth loss:
  • Toothpastes (biotene type) and oral gels are available for people with dry mouth These products may also have antibacterial action to reduce the severity of dental cavities over a long period of time.
  • Chewing sugar-free gums can be helpful.
  • Taking frequent sips of water without swallowing (spitting it out) may improve dry mouth.

• Increasing Eye Moisture –
  • Dry eyes are mainly treated with the use of artificial tears. A wide variety of over-the-counter products is available. Artificial tears can be used regularly and more often in dry environmental conditions such as on airplanes, in air-conditioned buildings, and on windy days.
  • While artificial tears are helpful, they often do not last long enough. Thicker      preparations (gel form) that last longer are available. These are often used at bedtime because they can sometimes cause blurry vision. Eye doctors can prescribe an eye drop called Restasis to treat more severe form of dry eyes. A small procedure called punctal plugs, to slow the disappearance of tears, is another treatment option when artificial tears are not sufficient.

• Medications – Medications that tend to reduce body fluids should be avoided.
  • Mild pain-relieving medications (analgesics), including acetaminophen, such as Tylenol or non-steroidal anti-inflammatory drugs – NSAIDs, such as Motrin and Aleve, can reduce muscle or joint pain.
  • In some patients, the anti-rheumatic drug hydroxychloroquine has been beneficial in decreasing pain and salivary gland swelling and improving fatigue, muscle pain, joint pain, or rash. This drug generally does not help with dry symptoms, however.
  • For patients with internal organ symptoms (particularly when the disease affects internal organs), steroids and immunosuppressive medications may be used. These include medicines such as prednisone (a steroid) and, rarely, chemotherapy-type medications.
  • Systemic corticosteroids and/or immunosuppressive agents like cytotoxic drugs have been used for various extra glandular symptoms of SS, such as: vasculitis, lung involvement kidney involvement. However, cytotoxic agents should be used with great care as they may increase the risk of lymphoma.
  • Water-based vaginal lubricants (K-Y Jelly, Astroglide, Replens, Luvena) can ease vaginal dryness and painful intercourse. Estrogen creams or other preparations may be helpful for women who have vaginal dryness due to reduced estrogen levels related to menopause.

It is important to know that the medications also involve numerous side effects that can prove harmful and may result in creating additional complications.

• Balance of rest and exercise – Guided exercise programs can help patients overcome fatigue, maintain flexibility, and overcome joint and muscle pain. Good sleep hygiene is helpful for improving fatigue and body pain.

Alternative Treatment

Alternative medicine definitely has more treatment options. The model of simply fixing the gut, stabilizing the blood sugar level, balancing the hormones, taking enough essential fatty acids and anti-inflammatory protocols, and so forth, are effective. However, they are not any different than treating any type of autoimmune disorders.

• Detoxification Therapy – Detoxification thrapy utilizes clinical procedures that safely reduce the body’s burden of toxic chemicals, including chemicals stored following occupational, accidental, and/or chronic airborne exposures. Chemicals bind to human tissues on the basis of their lipophilic properties — meaning literally “attracted to fats.”
• Green tea and EGCG for Sjogren’s syndrome – Green tea polyphenols reduce autoimmune symptoms in a murine model for human Sjogren’s syndrome and protect human salivary acinar cells from TNF-alpha-induced cytotoxicity. Green tea contains several antioxidants that have been shown to curb inflammation, prevent cell death, and possibly even ward off cancer. EGCG reduced the severity and delayed the onset of salivary gland damage associated with Sjogren’s syndrome.
• Fish oils, Wheat Germ oil & flax seeds – Effect of omega-3 and vitamin E supplementation on dry mouth in patients with Sjögren’s syndrome. Omega3 Fatty acid rich fish oil (FO) and vitamin E may delay the progress of certain autoimmune diseases. omega-3 (n-3) increases saliva production in patients with Sjögren’s syndrome. Wheat germ oil helps in stimulating saliva production in patients with Sjögren’s syndrome.
• DHEA – Low serum levels of sex steroids are associated with disease characteristics in primary Sjogren’s syndrome; supplementation with dehydroepiandrosterone restores the concentrations. It also helps in stimulating saliva production.
• Flavonoids – Plant-derived flavonoids are inhibitors of various intracellular processes, notably phosphorylation pathways, and potential inhibitors of cellular autoimmunity. This includes – apigenin and luteolin, fisitin, quercetin, morin and hesperitin. It acts as strong inhibitors for T cells.
• Rose hip herbal remedy for SS, Probiotic for gut
• Vitamin D – Vitamin D inhibits pro-inflammatory processes by suppressing the enhanced activity of immune cells that take part in the autoimmune reaction. Supplementation may be therapeutically beneficial particularly for Th1 mediated autoimmune disorders. Some reports imply that vitamin D may even be helpful in multiple sclerosis and diabetes type 1.

Integrated Treatment

Integrated medical practitioners treat the whole body as a single system and work with interdependent, oscillating energies and seek to achieve balance and integration of the entire body. There are natural, non-invasive, and wholistic approaches that incline toward discovering the imbalances, and integrate to correct them through diet and nutrition, exercise, acupuncture, massage, and individual customized education.

With the variety of symptoms that encompass Sjogren’s syndrome, it is very important to have a plan and helpful practitioner who works with full oversight and a set of complimentary skills under one roof, i.e. our center.