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SJOGREN SYNDROME Setayesh Nekarae, Ramina Mirzazadeh, Shaghayegh Ekrami, Rose Bandvar 10A What is the Sjogren Syndrome? Sjögren is a chronic autoimmune disease in which people’s white blood cells attack their moisture-producing glands. In Sjogren's syndrome, the mucous membranes and moisture-secreting glands of your eyes and mouth are usually affected first — resulting in decreased production of tears and saliva. More than 4,000,000 of Americans are suffering from Sjögren’s. It is one of the most prevalent autoimmune disorders. Does this syndrome occur alone? About half of the time Sjögren’s occurs alone, and the other half it occurs in the presence of another autoimmune connective tissue disease such as rheumatoid arthritis, lupus, or scleroderma also have a higher risk of developing lymphoma. Symptoms of Sjogren Syndrome PRIMARY SYMPTOMS Dry eye i. Gritty, sandy feeling ii. Stinging feeling Dry mouth i. Dry, cracked tongue ii. Sore throat iii. Difficulty talking iv. Difficulty swallowing v. Difficulty chewing dry food vi. Change in sense of taste/smell vii. Increase in cavities viii. Mouth sores ix. Cracked lips MAY CAUSE DYSFUNCTION OF OTHER SYMPTOMS i. ii. iii. iv. v. vi. vii. viii. ix. x. xi. xii. xiii. Swollen partoid glands Nausea Dry skin Joint pain Dry nose Reflux Muscle pain Fatigue Muscle weakness Low-grade fever Vaginal dryness Neuropathy Dizziness i. ii. iii. iv. v. vi. vii. Kidneys Gastrointestinal system Blood vessels Lungs Liver Pancreas Central Nervous System Symptoms of Sjogren Syndrome Symptoms may remain steady, worsen, or, uncommonly, go into remission. Since the symptoms of this autoimmune disorder such as dry eyes and dry mouth are very common among people, and mostly observed from the age of 40 and above, it is often mistaken as age-related, thus ignored. However, some medications can also cause symptoms that are similar to those of Sjögren's syndrome. Symptoms of Sjogren Syndrome Who is more prone to get it? Nine out of ten Sjögren's patients are reported to be women. In addition to prevalence in women, having a firstdegree relative with an autoimmune disease and previous pregnancies have been identified as epidemiological risk factors. Differences in prevalence due to race and ethnicity are unknown. Causes of Sjogren Syndrome Sjogren's syndrome is an autoimmune disorder. This means that your immune system mistakenly attacks your body's own cells and tissues. Although the cause of Sjögren’s syndrome is unknown, it is believed to be under the influence of a combination of genetic, environmental, and several other factors, as is the case with many other autoimmune disorders Scientists aren't certain why some people develop Sjogren's syndrome and others don't. Certain genes put people at higher risk of the disorder, but it appears that a triggering mechanism — such as infection with a particular virus or strain of bacteria — is also necessary. Hormonal factors: Sex hormones seem to influence humoral and cell-mediated immune response, with estrogen being considered one of the biggest factors responsible for gender immunologic dimorphism. Various animal models have indicated a role for estrogen deficiency in Sjögren's syndrome; mice that lack aromatase (estrogen deficiency) develop a lymphoproliferative disease that resembles Sjögren's. Diagnosis Early diagnosis and proper treatment are important — they may prevent serious complications and greatly improve a patient’s quality of life. Since symptoms of Sjögren’s mimic other conditions and diseases, Sjögren’s can often be overlooked or misdiagnosed. On average, it takes nearly 3.9 years to receive a diagnosis of Sjögren’s. Types of Diagnosis Sjogren's syndrome can be difficult to diagnose because the signs and symptoms vary from person to person and can be similar to those caused by other diseases. Side effects of a number of medications also mimic some signs and symptoms of Sjogren's syndrome. A variety of tests can help rule out other conditions and help pinpoint a diagnosis of Sjogren's syndrome. Blood tests Your doctor may order blood tests to check for: •Levels of different types of blood cells •Presence of antibodies common in Sjogren's syndrome •Evidence of inflammatory conditions •Indications of problems with your liver and kidneys Types of Diagnosis Eye tests Your doctor can measure the dryness of your eyes with a test called a Schirmer tear test. In this test, a small piece of filter paper is placed under your lower eyelid to measure your tear production. A doctor specializing in the treatment of eye disorders (ophthalmologist) may also examine the surface of your eyes with a magnifying device called a slit lamp. He or she may place drops in your eye that make any damage to your cornea easier to see. Types of Diagnosis Imaging Certain imaging tests can check the function of your salivary glands. • Sialogram A special X-ray called a sialogram can detect dye that's injected into the salivary glands located in front of your ears. This procedure shows how much saliva flows into your mouth. • Salivary scintigraphy. This nuclear medicine test involves the intravenous injection of a radioactive isotope, which is tracked over the course of an hour to see how quickly it arrives in all your salivary glands. Types of Diagnosis Biopsy Your doctor may also want to do a lip biopsy to detect the presence of clusters of inflammatory cells, which can indicate Sjogren's syndrome. For this test, a small sliver of tissue is removed from salivary glands located in your lip and examined under a microscope. Treatment Neither a cure for Sjögren's syndrome nor a specific treatment is known to permanently restore gland secretion. Instead, treatment is generally symptomatic and supportive. Moisture replacement therapies such as artificial tears may ease the symptoms of dry eyes. Some patients with more severe problems use goggles to increase local humidity or have punctal plugs inserted to help retain tears on the ocular surface medications. Depending on your symptoms, your doctor may suggest medications that: • Increase production of saliva Drugs such as pilocarpine (Salagen) and cevimeline (Evoxac) can increase the production of saliva, and sometimes tears. Side effects may include sweating, abdominal pain, flushing and increased urination. •Address specific complications If you develop arthritis symptoms, you may benefit from nonsteroidal anti-inflammatory drugs (NSAIDs) or other arthritis medications. Prescription eyedrops may be needed if over-the-counter drops aren't helpful. Yeast infections in the mouth should be treated with antifungal medications. • Treat systemwide symptoms Hydroxychloroquine (Plaquenil), a drug designed to treat malaria, is often helpful in treating Sjogren's syndrome. Drugs that suppress the immune system, such as methotrexate, also may be prescribed. Surgery To relieve dry eyes, you may consider undergoing a minor surgical procedure to seal the tear ducts that drain tears from your eyes (punctal occlusion). Collagen or silicone plugs are inserted into the ducts for a temporary closure. Collagen plugs eventually dissolve, but silicone plugs stay in place until they fall out or are removed. Alternatively, your doctor may use a laser to permanently seal your tear ducts for a longer time. Prevention There is no prevention mechanism for Sjögren's syndrome due to its complexity as an autoimmune disorder. However, lifestyle changes can reduce the risk factors of getting Sjögren's syndrome or reduce the severity of the condition with patients who have already been diagnosed. Diet is strongly associated with inflammation that is mostly seen in many autoimmune related diseases including Sjögren's syndrome. An experimental study show that Sjögren's syndrome patients show high sensitivity to gluten that directly relates to inflammation. Moderate exercise is also found be helpful in Sjögren's syndrome patients mainly reducing the effect of lung inflammation Complications Complications of the Sjogren syndrome include: • The most common complications of Sjogren's syndrome involve your eyes and mouth. • Dental cavities. Because saliva helps protect the teeth from the bacteria that cause cavities, you're more prone to developing cavities if your mouth is dry. • Yeast infections. People with Sjogren's syndrome are much more likely to develop oral thrush, a yeast infection in the mouth. • Vision problems. Dry eyes can lead to light sensitivity, blurred vision and corneal ulcers. Less Common Complications Less common complications may affect your: • Lungs, kidneys or liver. Inflammation may cause pneumonia, bronchitis or other problems in your lungs; may lead to problems with kidney function; and may cause hepatitis or cirrhosis in your liver. • Lymph nodes. A small percentage of people with Sjogren's syndrome develop cancer of the lymph nodes (lymphoma). • Nerves. You may develop numbness, tingling and burning in your hands and feet (peripheral neuropathy).