Other names : Mikulicz syndrome, Sicca syndrome
Sjögren's (pronounced SHOW-grins) syndrome is a chronic autoimmune disease in which a person’s white blood cells attack their moisture-producing glands. Historically, Mikulicz first reported these symptoms in 1898 so this condition was called "Mikulicz syndrome." It is more commonly named for Henrik Sjögren (1899-1986), a Swedish ophthalmologist, who reported the association of severe dry eyes [keratoconjunctivitis sicca (KCS)], dry mouth and rheumatoid arthritis in 1933. Later, it was recognized that patients might have dry eyes and dry mouth but no rheumatoid arthritis.
Today, according to the National Institute of Neurological Disorders and Stroke (NINDS), between 1 and 4 million people in the United States are living with this disease. The condition is found throughout the world and in all ethnic groups. Most people are more than 40 years old at the time of diagnosis. Women are 9 times more likely to have Sjögren's syndrome than men.
This desease is a disorder of your immune system often defined by its two most common symptoms — dry eyes (its medical term is xeropthalmia; your eyes may burn, itch or feel gritty — as if there's sand in them) and a dry mouth (its medical term is xerostomia; your mouth may feel like it's full of cotton, making it difficult to swallow or speak). In addition, Sjogren's syndrome may cause vaginal dryness that can cause dyspareunia, dryness of the trachea and bronchus (the airways) can lead to a dry cough and chest infections, and dry skin can occur. Sjogren's syndrome may affect other organs of the body including the kidneys, blood vessels, lungs, liver, pancreas, and brain. The risk for passing this disease on to family members is extremely low. There is a slightly increased incidence of autoimmune diseases in siblings and children. Many women with Sjögren's syndrome are interested in the risks of pregnancy and risks to the baby. Obstetrical authorities report slightly higher rates of recurrent fetal death and congenital heart block in those pregnancies complicated by maternal autoimmune disease.
There are two types of Sjogren's syndrome; primary and secondary. Primary Sjögren's syndrome is when Sjögren's syndrome occurs by itself. Secondary Sjögren's syndrome is when Sjögren's syndrome occurs in association with another autoimmune disease such as rheumatoid arthritis or systemic lupus erythematosus. (Sjögren's syndrome affects 5 out of 10 people who have rheumatoid arthritis.)
The cause of Sjögren's syndrome is not known, but it is considered an autoimmune disorder. People with this disease have abnormal proteins in their blood suggesting that their immune system, which normally functions to protect the body against cancers and invading infections, is reacting against their own tissue. As note, Sjögren’s syndrome causes increased levels of IL-1RA in CSF suggesting increased activity in the Interleukin 1 system and that this is associated with increased fatigue through cytokine induced sickness behavior.
Sjögrens syndrome is marked by lymphocytic infiltration of the lacrimal and salivary glands. Extra-glandular manifestations are common and result from similar infiltration of various organs. Immunohistologic studies have shown that the lymphocytic infiltrate is comprised predominantly of CD4-positive T cells, primarily of the Th1-type. Unlike normal epithelium, the glandular epithelial cells express MHC class II antigens and B7 co-stimulatory molecules, allowing them to serve as antigen-presenting cells.
Diagnosis of Sjögren's syndrome; The doctor needs to take a good history, seeking out features of dryness. The physical examination is helpful looking at the eyes and the mouth and observing whether they are dry, red and irritated and the skin is dry. A specialist eye examination is valuable and occasionally kidney and lung function tests are needed. Dry mouth and eyes alone may result from aging, medication or a condition known as Amyloidosis. Blood Tests for diagnosis include : ANA (Anti-Nuclear Antibody), SS-A (or Ro) and SS-B (or La) Antibodies to SS-A (or Ro) and SS-B (or La.), RF (Rheumatoid Factor), ESR (Erythrocyte Sedimentation Rate), IGs (Immunoglobulins). The Salivary Gland Tests include: Parotid Gland Flow, Salivary Scintigraphy, Sialography.
Treatment of this disorder is based on the symptoms. Dry eyes are treated with artificial tears, a tear stimulant, or eye lubricant. Dry mouth may be helped by frequent small drinks of water or chewing gum to stimulate saliva production. Arthritis symptoms are treated with anti-inflammatory medicines, such as aspirin, acetaminophen, and other NSAIDs.
These are the medications used to relieve the dryness caused by the Sjögren autoimmune attack on the body’s moisture-producing glands on table below [1].
Treatment of Sjögren's syndrome is aimed at symptomatic relief, at this stage there is no cure [2].
Dry Eyes
A variety of artificial tear preparations can be used to treat dry eyes. The severity of the dryness will determine how frequently you need to use the drops. Some preparations have preservatives - these last longer but may be irritating to the eye. It is a matter of trying one brand and seeing how you go. Lubricating ointments are longer acting preparations that are useful at night whilst sleeping. These tend to be greasy so are not appropriate for daytime use.
Dry Mouth
Dry mouth is harder to treat than dry eyes. There are some artificial oral lubricant preparations available, but none are entirely satisfactory. Some people find chewing sugarless gum is helpful, whilst others prefer simply to spray water into the mouth. Dietary modification using soft or moist foods may be of some assistance. The use of sugar should be avoided as it contributes to the development of dental caries. Good dental hygiene and frequent dental visits are essential.
Dry Skin
Sorbolene cream and moisturisers may be helpful. For dryness of the vagina, lubricating creams or oestrogen creams may be helpful.
Oral Medications
Oral medications are used in a majority of people. Non-steroidal anti-inflammatory drugs, or the anti-malarial drug Plaquenil, may be used to treat joint symptoms. Prednisone and immunosuppressant drugs are seldom used but are useful where there is severe lung or kidney involvement.
In an era of increasing health maintenance organizations (HMO's) and the need for diagnosis codes, it is often necessary for the patient to help their physician or dentist by informing them of currently accepted diagnosis codes. Individuals should consult a qualified health care provider for professional medical advice, diagnoses and treatment of a medical or health condition.
