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Offline skyblue1

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Ladies.....Sjögren's Syndrome
« on: September 01, 2011, 04:58:02 PM »
Bringing this to your attention, in case you arent aware of it:

                              Sjögren's Syndrome

Tennis star Venus Williams withdrew from the U.S. Open on Wednesday, citing a diagnosis of Sjögren's syndrome, a little-known autoimmune disorder that causes fatigue and joint pain. TIME Healthland asked Dr. Victoria Shanmugam, an assistant professor of rheumatology at Georgetown University, to explain the condition.
Shanmugam is not involved in Williams' treatment but currently sees other patients with Sjögren's (pronounced show-grens).

What is Sjögren's syndrome?
It's an autoimmune disease that is typically characterized by inflammation in the tear ducts and salivary glands. We're not sure why it focuses on these tissues. Many people will have a very mild form and experience dryness of the eyes and mouth, fatigue, joint pain and myalgias [or muscle pain]. Many people will have that and nothing else. But a small proportion of people will go on to have multi–organ system disease that behaves a little more like lupus [another autoimmune disease in which the body's own immune cells start to attack healthy tissues, particularly in the joints].

How common is Sjögren's?
It's not that uncommon. Estimates vary, and there may be a fair number of people who have Sjögren's and don't know it. So the estimates vary widely. A European study found that about 4% of the population may be affected, while a study by the Mayo Clinic found a much lower prevalence of around 1%.

It's more common in women than in men, and often people don't get diagnosed until middle age.

Is there a connection between Sjögren's and lupus?
There is a degree of overlap between the two diseases. We look at the inflammatory pattern in each organ to help distinguish between Sjögren's and lupus. For example, [in Sjögren's] there is a particular type of inflammation in the kidney that affects how the kidneys deal with salt and acid balance, which produces a very different picture than the inflammation of the kidney's glomerulus, which is involved in filtering waste from the blood, that we see in lupus.

Some people will have particular types of inflammation in the lung and liver that is only seen in Sjögren's, which can sometimes go on to become lupus. But the fact that [Williams] was diagnosed with Sjögren's doesn't necessarily mean she will go on to develop lupus.

What causes Sjögren's?
That's the million-dollar question in rheumatology. We think people probably have some kind of genetic predisposition — but we don't know for sure — and some environmental factor sets off the immune system to trigger the disease. It's not well understood why this particular person [will get diagnosed] and why now at this point in their life. But we do know that stress makes immune diseases worse.

Can Sjögren's be treated?
Yes. We'll treat people dependent on where they are on the disease spectrum. For the milder cases, we have topical [options] to treat dryness of the eyes and mouth. These can suppress the immune system locally in the eye to reduce inflammation. We also warn patients to get regular dental checkups.

For slightly more severe disease, we use disease-modifying drugs that are similar to treatments for lupus. Hydroxychloroquine, an anti-malaria [drug] can be helpful for joint pain, muscle aches and fatigue. If there is more severe inflammation involving the lung, kidney or liver, we can pick more potent immune-suppressing medications such as steroids or methotrexate to really suppress the immune system.

Do you think Williams will be able to return to playing professional tennis?
Her decision to take a little time off and recover and get her health under control is a reasonable decision. There is no reason to think that having this diagnosis shouldn't allow her to continue to be active in the future. Many many people with Sjögren's lead very full and active lives. And in most cases, it doesn't affect their ability to hold down a full-time job and maintain all their usual activities.



Read more: http://healthland.time.com/2011/09/01/wh...z1WkDGM0G4


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Offline skyblue1

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Re: Ladies.....Sjögren's Syndrome
« Reply #1 on: September 01, 2011, 05:00:26 PM »
Sjögren's syndrome ( /ˈʃoʊɡrɨnz/ shoh-grinz), also known as "Mikulicz disease" and "Sicca syndrome",[1] is a systemic autoimmune disease in which immune cells attack and destroy the exocrine glands[2] that produce tears and saliva.

It is named after Swedish ophthalmologist Henrik Sjögren[3] (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.[citation needed] 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.[citation needed]

Sjögren's syndrome can exist as a disorder in its own right (primary Sjögren's syndrome) or it may develop years after the onset of an associated rheumatic disorder such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, primary biliary cirrhosis etc. (secondary Sjögren's syndrome).[citation needed]

An autoantigen is alpha-Fodrin.[4]

It should not be confused with the Sjögren–Larsson syndrome, also denoted T. Sjögren syndrome in early studies

Signs and symptomsThe hallmark symptom of the disorder is a generalized dryness, typically involving dry mouth and dry eyes (part of what are known as sicca symptoms). In addition, Sjögren's syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body, including the kidneys, blood vessels, lungs, liver, pancreas, peripheral nervous system (distal axonal sensorimotor neuropathy) and brain.

Sjögren's syndrome is associated with increased levels of IL-1RA in CSF, an interleukin 1 antagonist, suggesting that there was first increased activity in the interleukin 1 system, then an auto-regulatory up-regulation of IL-RA in attempts to reduce the successful binding of Interleukin 1 to its receptors. It is likely that Interleukin 1 is the marker for fatigue, however IL-1RA increases are observed in the CSF and is associated with increased fatigue through cytokine induced sickness behavior.[5] Patients with secondary Sjögren's syndrome also often exhibit signs and symptoms of their primary rheumatic disorders, such as SLE, Rheumatoid Arthritis or Systemic Sclerosis.

[edit] DiagnosisDiagnosing Sjögren's syndrome is complicated by the range of symptoms a patient may manifest, and the similarity between symptoms from Sjögren's syndrome and those caused by 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.[6] [7]

The Schirmer 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.[8] It takes longer time to perform than a Schirmer test, but does not require specific equipment.

A slit-lamp examination is done to look for 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.

A radiological procedure can also be used as a reliable and accurate way of diagnosing Sjögren's syndrome. A contrast agent is injected into the parotid duct (of Stensen), which is a duct opening 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[9] 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 3 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 4-fold life of the drug).



http://en.wikipedia.org/wiki/Sj%C3%B6gren's_syndrome

The_Chosen_One

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Re: Ladies.....Sjögren's Syndrome
« Reply #2 on: September 03, 2011, 10:54:12 PM »
Two questions - does it run in families and if so, why hasn't Serena shown symptoms?

and is she aware that taking steroids, even for an illness such as this could end her career?

Those type of medications could be on the banned list, and they'd want to make sure she got an exemtion and clean B-samples before letting her play again.