Sjogren’s (Sjögren’s, pronounced SHOW-grins) syndrome is an autoimmune disease in which the immune system targets moisture-producing glands and causes dryness in the mouth and eyes. Other parts of the body can be affected as well, resulting in a wide range of possible symptoms.
The disease-fighting cells of the immune system attack the glands that produce tears and saliva (the lacrimal and salivary glands). The disease can affect other glands too, such as those in the stomach, pancreas and intestines, and can cause dryness in other places that need moisture, such as the nose, throat, airways, vagina and skin.
You might hear Sjogren’s syndrome referred to as rheumatic disease. A rheumatic disease causes inflammation in joints, muscles, skin or other body tissue, and Sjogren’s can do that. The many forms of arthritis, which often involve inflammation in the joints among other problems, are examples of rheumatic diseases. Sjogren’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).
It is believed that some 1 to 4 million people have the disease; most – 90% – are women. It can occur at any age, but it usually is diagnosed after age 40 and can affect people of all races and ethnic backgrounds. It is rare in children, but can occur.
Primary versus Secondary Sjogren’s Syndrome
Sjogren’s syndrome is classified as either primary or secondary. Primary Sjogren’s occurs by itself, and secondary Sjogren’s occurs with another disease. Both are systemic disorders, although the symptoms in the primary form are more restricted.
In cases of primary Sjogren’s syndrome, the doctor can trace the symptoms to problems with the tear and saliva glands. These patients are likely to have different antibodies circulating in their blood compared to people with the secondary form. These antibodies are called SS-A and SS-B; people with primary Sjogren’s are also more likely to have antinuclear antibodies (ANAs) in their blood. ANAs are autoantibodies that are directed against the body.
In cases of secondary Sjogren’s syndrome, the patient will have had an autoimmune disease such as rheumatoid arthritis or lupus before Sjogren’s developed. People with this type tend to have more health problems because they have two diseases, and they are also less likely to have the antibodies associated with primary Sjogren’s.
Causes
Researchers believe Sjogren’s syndrome is caused by a combination of genetic and environmental factors. Having one of the associated genes will not cause a person to develop the disease without some sort of trigger to activate the immune system.
It is thought that the trigger may be a viral or bacterial infection, and may work like this: A person who has a Sjogren’s-associated gene gets a viral infection. The virus stimulates the immune system to act, but the gene alters the attack, sending fighter cells (lymphocytes) to the eye and mouth glands. Once there, the lymphocytes attack healthy cells, causing the inflammation that damages the glands and keeps them from working properly. These fighter cells are supposed to die after their attack in a natural process called apoptosis, but in people with Sjogren’s syndrome, they continue to attack, causing further damage. The possibility that the endocrine and nervous systems play a role is also under investigation.
Diagnosis
The doctor will first take a detailed medical history, which includes asking questions about general health, symptoms, family medical history, alcohol consumption, smoking, or use of drugs or medications. The doctor will also do a complete physical exam to check for other signs of Sjogren’s.
There may be some tests, too. First, the doctor will want to check the eyes and mouth to see whether Sjogren’s is causing the symptoms and how severe the problem is. Because there are many possible causes of dry eyes and dry mouth, the doctor will take these into account.
One is generally considered to have definite Sjogren’s if one has dry eyes, dry mouth, and a positive lip biopsy. The doctor may decide to perform additional tests to see whether other parts of the body are affected, and whether various antibodies are found in the blood.
Signs, symptoms & indicators of Sjogren's Syndrome
(Mildly/highly) elevated ANA levels
Constant/frequent thirst
Fatigability
Moist eyes
(Very) dry mouth
Counter Indicators
Excess/abundant saliva in mouth
Dry nose
Low stamina
Joint pain/swelling/stiffness
Conditions that suggest Sjogren's Syndrome
Chronic Thyroiditis
Antithyroid antibodies are created when antibodies migrate out of the salivary glands into the thyroid gland. Antithyroid antibodies cause thyroiditis, a common problem in people with Sjögren’s.
Dry Eye
“Dry eye” is a symptom of certain autoimmune diseases like rheumatoid arthritis and Sjogren’s syndrome.
Absense of Sjogren's
Risk factors for Sjogren's Syndrome
Low DHEA Level
In a study of ten women with Sjogren’s syndrome, all were shown to have decreased serum concentrations of DHEA-S and an increased cortisol/DHEA-S ratio compared with healthy controls.
EFA (Essential Fatty Acid) Type 3 Requirement
A study found that people with Sjogren’s Syndrome, which is characterized by dry eyes, had the lowest levels of essential fatty acids. [Prostaglandins Leukot Essent Fatty Acids. 1998 Oct;59(4): pp.239-45]
Sjogren's Syndrome suggests the following may be present
EFA (Essential Fatty Acid) Type 3 Requirement
A study found that people with Sjogren’s Syndrome, which is characterized by dry eyes, had the lowest levels of essential fatty acids. [Prostaglandins Leukot Essent Fatty Acids. 1998 Oct;59(4): pp.239-45]
Sjogren's Syndrome can lead to
Chronic Thyroiditis
Antithyroid antibodies are created when antibodies migrate out of the salivary glands into the thyroid gland. Antithyroid antibodies cause thyroiditis, a common problem in people with Sjögren’s.
Dry Eye
“Dry eye” is a symptom of certain autoimmune diseases like rheumatoid arthritis and Sjogren’s syndrome.
Increased Risk of Lymphoma
pSS patients are 40 times more at risk than healthy people to develop lymphoma, a fatal lymphocytic cancer.
Sjogren's Syndrome could instead be
Retinitis Pigmentosa
Sjogren’s is an autoimmune disease sometimes misdiagnosed as retinitis pigmentosa. [Am J Ophthalmol, 1996 Dec, 122:6, pp.903-5 Abstract]
Recommendations for Sjogren's Syndrome
Testosterone
We hypothesize that androgen deficiency, which reportedly occurs in primary and secondary Sjögren’s syndrome (e.g., systemic lupus erythematosus, rheumatoid arthritis), is a critical etiologic factor in the pathogenesis of dry eye syndromes. We further hypothesize that androgen treatment to the ocular surface will promote both lacrimal and meibomian gland function and alleviate both “aqueous-deficient” and “evaporative” dry eye. Our results demonstrate that androgens regulate both lacrimal and meibomian gland function, and suggest that topical androgen administration may serve as a safe and effective therapy for the treatment of dry eye in Sjögren’s syndrome. [Annals of the New York Academy of Sciences 876:312-324 (1999)]
Tests, General Diagnostic
During the 85th General Session of the International Association for Dental Research, scientists are reporting that, instead of blood tests and biopsy, saliva can be used to detect primary Sjögren’s Syndrome (pSS), an autoimmune disease which affects about 4 million American, 90% being women. pSS patients are 40 times more at risk than healthy people to develop lymphoma, a fatal lymphocytic cancer.
Essential Fatty Acids
Those with Sjogren’s syndrome may have a metabolic block that interferes with the body’s ability to make GLA from the omega-6 containing oils. [Med Hypotheses 1980;6: pp.225–32, Med Hypotheses 1984;14: pp.233–47] Bypassing this metabolic step by taking GLA orally may help, especially if there are other indicators of a deficiency.
Key
| Weak or unproven link |
| Strong or generally accepted link |
| Proven definite or direct link |
| Strongly counter-indicative |
| Very strongly or absolutely counter-indicative |
| May do some good |
| Likely to help |
| Highly recommended |
Glossary
Autoimmune Disease
One of a large group of diseases in which the immune system turns against the body's own cells, tissues and organs, leading to chronic and often deadly conditions. Examples include multiple sclerosis, rheumatoid arthritis, systemic lupus, Bright's disease and diabetes.
Immune System
A complex that protects the body from disease organisms and other foreign bodies. The system includes the humoral immune response and the cell-mediated response. The immune system also protects the body from invasion by making local barriers and inflammation.
Stomach
A hollow, muscular, J-shaped pouch located in the upper part of the abdomen to the left of the midline. The upper end (fundus) is large and dome-shaped; the area just below the fundus is called the body of the stomach. The fundus and the body are often referred to as the cardiac portion of the stomach. The lower (pyloric) portion curves downward and to the right and includes the antrum and the pylorus. The function of the stomach is to begin digestion by physically breaking down food received from the esophagus. The tissues of the stomach wall are composed of three types of muscle fibers: circular, longitudinal and oblique. These fibers create structural elasticity and contractibility, both of which are needed for digestion. The stomach mucosa contains cells which secrete hydrochloric acid and this in turn activates the other gastric enzymes pepsin and rennin. To protect itself from being destroyed by its own enzymes, the stomach’s mucous lining must constantly regenerate itself.
Arthritis
Inflammation of a joint, usually accompanied by pain, swelling, and stiffness, and resulting from infection, trauma, degenerative changes, metabolic disturbances, or other causes. It occurs in various forms, such as bacterial arthritis, osteoarthritis, or rheumatoid arthritis. Osteoarthritis, the most common form, is characterized by a gradual loss of cartilage and often an overgrowth of bone at the joints.
Antibody
A type of serum protein (globulin) synthesized by white blood cells of the lymphoid type in response to an antigenic (foreign substance) stimulus. Antibodies are complex substances formed to neutralize or destroy these antigens in the blood. Antibody activity normally fights infection but can be damaging in allergies and a group of diseases that are called autoimmune diseases.
Rheumatoid Arthritis
A long-term, destructive connective tissue disease that results from the body rejecting its own tissue cells (autoimmune reaction).
Virus
Any of a vast group of minute structures composed of a protein coat and a core of DNA and/or RNA that reproduces in the cells of the infected host. Capable of infecting all animals and plants, causing devastating disease in immunocompromised individuals. Viruses are not affected by antibiotics, and are completely dependent upon the cells of the infected host for the ability to reproduce.
Apoptosis
Programmed cell death as signaled by the nuclei in normally functioning human and animal cells when age or state of cell health and condition dictates. Cancerous cells, however, are unable to experience the normal cell transduction or apoptosis-driven natural cell death process.
Biopsy
Excision of tissue from a living being for diagnosis.
Thyroid
Thyroid Gland: An organ with many veins. It is at the front of the neck. It is essential to normal body growth in infancy and childhood. It releases thyroid hormones - iodine-containing compounds that increase the rate of metabolism, affect body temperature, regulate protein, fat, and carbohydrate catabolism in all cells. They keep up growth hormone release, skeletal maturation, and heart rate, force, and output. They promote central nervous system growth, stimulate the making of many enzymes, and are necessary for muscle tone and vigor.
Serum
The cell-free fluid of the bloodstream. It appears in a test tube after the blood clots and is often used in expressions relating to the levels of certain compounds in the blood stream.
DHEA
Dehydroepiandrosterone (DHEA) is a steroid produced by the adrenal glands and is the most abundant one found in humans. DHEA may be transformed into testosterone, estrogen or other steroids. It is found in the body as DHEA or in the sulfated form known as DHEA-S. One form is converted into the other as needed.
Essential Fatty Acid
(EFA): A substance that the human body cannot manufacture and therefore must be supplied in the diet.
Prostaglandin
Any of a class of physiologically active substances present in many tissues, with effects such as vasodilation, vasoconstriction, stimulation of the smooth muscles of the bronchus or intestine, uterine stimulation; also involved in pain, inflammation, fever, allergic diarrhea, and dysmenorrhea. A potent hormone -- similar in structure to an unsaturated fatty acid -- that acts in extremely low concentrations on local target organs; first isolated from the prostate.
Fatty Acids
Chemical chains of carbon, hydrogen, and oxygen atoms that are part of a fat (lipid) and are the major component of triglycerides. Depending on the number and arrangement of these atoms, fatty acids are classified as either saturated, polyunsaturated, or monounsaturated. They are nutritional substances found in nature which include cholesterol, prostaglandins, and stearic, palmitic, linoleic, linolenic, eicosapentanoic (EPA), and decohexanoic acids. Important nutritional lipids include lecithin, choline, gamma-linoleic acid, and inositol.
Lymphoma
Any tumor of the lymphatic tissues.
Cancer
Refers to the various types of malignant neoplasms that contain cells growing out of control and invading adjacent tissues, which may metastasize to distant tissues.