Alternative Names: Mikulicz disease” and “Sicca syndrome
Sjögren’s syndrome (SHOW-grins)is a systemic autoimmune disease in which immune cells attack and destroy the exocrine glands that produce tears and saliva.In some cases, other organs of the body are also affected, including the:
It is named after Swedish ophthalmologist Henrik Sjögren (1899–1986), who first described it.
Nine out of ten Sjögren’s patients are women and the average age of onset is late 40s, although Sjögren’s occurs in all age groups in both women and men. It is estimated to strike as many as 4 million people in the United States alone, making it the second most common autoimmune rheumatic disease.
Sjogren’s syndrome may be classified as primary or secondary. Primary Sjogren’s syndrome occurs alone; secondary Sjogren’s syndrome is seen alongside another disease, such as rheumatoid arthritis and systemic lupus erythematosis (SLE).
The disorder should not be confused with the Sjögren–Larsson syndrome, which was also denoted T. Sjögren syndrome in early studies.
There are many different symptoms of Sjogren’s. However, not everyone experiences the same ones or to the same degree.
The characteristic dryness of Sjogren’s means the eyes often feel very uncomfortable and may burn, itch or feel gritty. Mouth dryness makes talking, chewing and swallowing difficult.
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Other symptoms include:
•A sore or cracked tongue
•Dry nose and skin
•Dental problems are more likely (because of the lack of saliva)
As is often the case with any long-term condition, a person’s quality of life may be adversely affected. This may result in depression and make social life, work and relationships more difficult to maintain and enjoy.
Sjogren’s is also associated with an increased risk of miscarriage, Raynauds phenomenom and adverse reactions to medication such as antibiotics.
Sjogren’s syndrome is an autoimmune disorder. This means that your immune system mistakenly attacks your body’s own cells and tissues.
Scientists aren’t certain why some people develop Sjogren’s syndrome and others don’t. Certain genes put people at higher risk for the disorder, but it appears that a triggering mechanism — such as infection with a particular virus or strain of bacteria — is also necessary.
In Sjogren’s syndrome, your immune system first targets the moisture-secreting glands of your eyes and mouth. But it can also damage other parts of your body, such as your:
Although anyone can develop Sjogren’s syndrome, it typically occurs in people with one or more known risk factors. These include:
*Age. Sjogren’s syndrome is usually diagnosed in people older than 40.
*Sex. Women are much more likely to have Sjogren’s syndrome.
*Rheumatic disease. It’s common for people who have Sjogren’s syndrome to also have a rheumatic disease — such as rheumatoid arthritis or lupus.
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 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.
*Unborn baby. If you’re a woman with Sjogren’s syndrome and you plan to become pregnant, talk with your doctor about being tested for certain autoantibodies that may be present in your blood. In rare cases, these antibodies have been associated with heart problems in newborns
*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).
Diagnosing Sjögren’s syndrome is complicated by the range of symptoms a patient may manifest, and the similarity between symptoms of Sjögren’s syndrome and those of other conditions. Nevertheless, the combination of several tests can lead to a diagnosis of Sjögren’s syndrome.
Blood tests can be done to determine if a patient has high levels of antibodies that are indicative of the condition, such as anti-nuclear antibody (ANA) and rheumatoid factor (because SS frequently occurs secondary to rheumatoid arthritis), which are associated with autoimmune diseases. Typical Sjögren’s syndrome ANA patterns are SSA/Ro and SSB/La, of which SSB/La is far more specific; SSA/Ro is associated with numerous other autoimmune conditions but are often present in Sjögren’s.
Schirmer’s test measures the production of tears: a strip of filter paper is held inside the lower eyelid for five minutes, and its wetness is then measured with a ruler. Producing less than five millimeters of liquid is usually indicative of Sjögren’s syndrome. However, lacrimal function declines with age or may be impaired from other medical conditions. An alternative test is nonstimulated whole saliva flow collection, in which the patient spits into a test tube every minute for 15 minutes. A resultant collection of less than 1.5 mL is considered a positive result. It takes longer to perform than Schirmer’s test, but does not require special equipment.
A slit-lamp examination can reveal dryness on the surface of the eye. Salivary gland function can be tested by collecting saliva and determining the amount produced in a five minute period. A lip biopsy can reveal lymphocytes clustered around salivary glands, and damage to these glands due to inflammation.
Ultrasound examination of the salivary glands is the simplest confirmatory test and has the added advantage of being non-invasive with no complications. The parenchyma of the gland demonstrates multiple, small-2-6 mm hypoechoic lesions which are representations of the lymphocytic infiltrates. Often sialectasis with calculi are demonstrated if the disease is advanced. The sonographic findings have excellent symptom correlation. The other advantage of ultrasound is that complications of the disease such as extra-nodal lymphomas can often be detected as larger 1–4 cm hypoechoic intra-parenchymal masses.
There is also a radiological procedure which is a reliable and accurate test for Sjögren’s syndrome. A contrast agent is injected into the parotid duct, which opens from the cheek into the vestibule of the mouth opposite the neck of the upper second molar tooth. Widespread puddling of the injected contrast scattered throughout the gland indicates Sjögren’s syndrome.
The Revised Classification Criteria for Sjögren’s Syndrome requires the presence of signs, symptoms, and lab findings.
Patient-reported symptoms must include both ocular symptoms, such as daily, persistent, troublesome dry eyes for more than three months, and oral symptoms, such as needing to drink water to swallow food.
Objective evidence of eye involvement relies on Schirmer’s test and the Rose bengal score (or similar). Histopathology studies should show focal lymphocytic sialadenitis. Objective evidence of salivary gland involvement is tested through ultrasound examinations, the level of unstimulated whole salivary flow, a parotid sialography, or salivary scintigraphy. Autoantibodies against Ro (SSA) and/or La (SSB) antigens are also expected.
SS can be excluded from people with past head and neck radiation therapy, hepatitis C infection, Acquired immunodeficiency syndrome (AIDS), pre-existing lymphoma, sarcoidosis, graft-versus-host disease, and use of anticholinergic drugs (since a time shorter than four times the life of the drug).
It’s not possible to prevent Sjogren’s syndrome and there’s no cure, but treatments can help to relieve many of the symptoms. Treatment varies depending on which parts of the body are affected and may include:
•Artificial tears to help with dry eyes
•Saliva stimulants and mouth lubricants for dry mouth
•Anti-inflammatory medication for joint or muscle pain
•Corticosteroids or immunosuppressive drugs for lung, kidney, blood vessel or nervous system problems
Lifestyle and home remedies:
Many symptoms of Sjogren’s syndrome respond well to self-care measures.
To relieve dry eyes:
*Use artificial tears, an eye lubricant or both. Artificial tears (in eyedrop form) and eye lubricants (in eyedrop, gel or ointment form) help relieve the discomfort of dry eyes. Both types of product are available over-the-counter. You don’t have to apply eye lubricants as often as artificial tears. Because of their thicker consistency, though, eye lubricants can blur your vision and collect on your eyelashes. Your doctor may recommend artificial tears without preservatives because the preservatives can be irritating for people with dry eye syndrome.
* Increase humidity. Increasing the indoor humidity and reducing your exposure to blowing air may help keep your eyes from getting uncomfortably dry. For example, avoid sitting in front of a fan or air-conditioning vent, and wear goggles or protective eyewear when you go outdoors.
To help with dry mouth:
*Increase fluid intake. Drinking lots of fluids, particularly water, helps to reduce dry mouth.
*Stimulate saliva flow. Sugarless gum or hard candies can boost saliva flow. Because Sjogren’s syndrome increases your risk of dental cavities, limit sweets, especially between meals. Lemon juice in water can also help stimulate saliva flow.
*Try artificial saliva. Saliva replacement products often work better than plain water because they contain a lubricant that helps your mouth stay moist longer. These products may come as a spray or lozenge.
*Use nasal saline spray. A nasal saline spray can help moisturize and clear nasal passages so you can breathe freely through your nose. A dry, stuffy nose can increase mouth breathing.
Dry mouth increases your risk of dental cavities and tooth loss. The following precautions may help prevent those types of problems.
*Brush your teeth and floss after every meal.
*Schedule regular dental appointments, at least every six months.
*Use daily topical fluoride treatments and antimicrobial mouthwashes.
Other areas of dryness:
If dry skin is a problem, avoid hot water when you bathe and shower. Pat your skin — don’t rub — with a towel and apply moisturizer when your skin is still damp. Use rubber gloves when doing dishes or housecleaning. Vaginal moisturizers and lubricants help women who experience vaginal dryness.
Sjögren’s can damage vital organs of the body with symptoms that may plateau or worsen, but the disease does not go into remission as with other autoimmune diseases. Some people may experience only the mild symptoms of dry eyes and mouth, while others have symptoms of severe disease. Many patients are able to treat problems symptomatically. Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating. Debilitating fatigue and joint pain can seriously impair quality of life. Some patients can develop renal involvement (autoimmune tubulointerstitial nephritis) leading to proteinuria, urinary concentrating defect and distal renal tubular acidosis.
Patients with Sjögren’s syndrome have a higher rate of non-Hodgkin lymphoma compared to both patients with other autoimmune diseases and healthy people. About 5% of patients with Sjögren’s syndrome will develop some form of lymphoid malignancy. Patients with severe cases are much more likely to develop lymphomas than patients with mild or moderate cases. The most common lymphomas are salivary extranodal marginal zone B cell lymphomas (MALT lymphomas in the salivary glands) and diffuse large B-cell lymphoma.
Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.
- Well Blog: The Voices of Sjogren’s Syndrome (well.blogs.nytimes.com)
- Venus Williams has Sjogren’s Syndrome: What is it? (cbsnews.com)
- Venus Williams Describes Living With Sjögren’s Syndrome (inquisitr.com)
- What is Sjögren’s? (oup.com)
- Dear Venus, What You Should Know about Sjogren’s Syndrome (blogher.com)
- Well Blog: Venus Williams and Sjogren’s Syndrome (well.blogs.nytimes.com)
- What Is Sjögren’s Syndrome? (foxnews.com)
- What is Sjögren’s Syndrome? An Expert Explains Venus Williams’ Diagnosis (healthland.time.com)
- Why Sjogren’s Syndrome Caused Venus Williams to Quit US Open (livescience.com)
- Venus Williams and Sjogren’s Syndrome: Nothing to Drool Over (psychologytoday.com)