Sjogren's Syndrome

Sjogren's syndrome is a condition which is often misdiagnosed or undiagnosed. Awareness and recognition of Sjogren's syndrome is important.

Experts believe 1 to 4 million people have the disease. Of this group, 90% are women. Sjogren's syndrome can occur at any age, but it usually is diagnosed after age 40 and can affect people of all races and ethnic backgrounds. Sjogren's syndrome is rare in children, but it can occur.

Many women have the disease but do not recognize the symptoms which are similar to those of other conditions including menopause.

Symptoms

It has been suggested that many more people suffer with Sjogren's syndrome than have been diagnosed. Sjogren's syndrome is considered to be:

* an autoimmune disease
* a rheumatic disease
* a connective tissue disorder

Abnormal production of autoantibodies in the blood which turn against various tissues in the body cause the disease. Inflammation in the glands of the body results from this abnormality.

Sjogren's syndrome is characterized by:

* dry mouth
* dry eyes
* hoarseness
* vaginal dryness
* skin dryness
* chronic fatigue

Diagnosis

The diagnosis of Sjogren's syndrome is based largely on the detection of dry eyes and mouth. Helpful diagnostic tools that serve in making the diagnosis include:

* Schirmer's test for dry eyes
* radiologic salivary scans
* salivary flow testing
* biopsy of salivary glands
* blood tests


Treatment

Treatment of Sjogren's syndrome is different for each person, depending on what parts of the body are affected. Although there is no cure for Sjogren's syndrome, mouthwashes, saliva substitutes, sprays, gels, and gum can relieve oral symptoms. Medications and drug treatment options for dry mouth associated with Sjogren's syndrome may include saliva and mucus stimulating drugs such as:

* Salagen (Pilocarpine hydrochloride)
* Evoxac (Cevimeline HCI)

Artificial tears and eye ointments can help relieve chronic dry eyes. Medications and drug treatment options for dry eye associated with Sjogren's syndrome may include:

* Restasis (Cyclosporine Opthalmalic Emulsion)
* Hydroxypropyl Cellulose (Eye Drops & Pellets)

Extraglandular problems, such as joint pain or muscle pain involvement, are often treated with NSAIDs (non-steroidal anti-inflammatory drugs). Lung, kidney, blood vessel, or nervous system problems may be treated with:

* corticosteroids
* DMARDs (disease-modifying anti-rheumatic drugs) or immunosuppressives





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Antiphospholipid Syndrome (APS)

Antiphospholipid syndrome (APS), also known as 'sticky blood,' is an autoimmune disorder -- the result of the immune system essentially turning on the body and attacking it in error. In the case of APS, the body makes antibodies to its own blood proteins.

Antiphospholipid syndrome can occur in individuals without any associated disease. This is called primary APS. The disorder may also occur with systemic lupus erythematosus (SLE) or another autoimmune disorder. This is called secondary APS

Symptoms of Antiphospholipid Syndrome
Along with the antibodies, the body begins producing blood clots. The blood clots can block arteries and veins, cutting off blood supply to a part of the body. The symptoms the individual experiences come from the location(s) and effects of the blood clots:

1.Veins or arteries of the arms or legs - Clots may cause pain, swelling, numbness, tingling in the hands or feet, or leg ulcers. If the blood supply was completely cut off to a part, such as a toe, the individual could lose the toe.

2. Arteries of the heart - Clots may cause chest pain or heart attack. The individual may also have a heart murmur or heart valve problems.

3. Blood vessels of the skin - Clots may cause bruises (purpura) or a blotchy, purplish rash called livedo reticularis.

4. lood vessels of the brain - A clot that cuts off blood supply to a part of the brain causes a stroke. An individual with APS may also experience migraine headaches or seizures.

5. Placenta during pregnancy - Women with APS may have frequent miscarriages or premature births.

The most serious form of antiphospholipid syndrome, called catastrophic APS, occurs when many internal organs develop blood clots over a period of days to weeks.

Diagnosis of Antiphospholipid Syndrome
Diagnosis of antiphospholipid syndrome relies on characteristic symptoms and signs, plus laboratory tests. If an individual has had blood clots in the legs without any other possible cause, for example, APS may be to blame. A blood test for anticardiolipin antibodies can help confirm the diagnosis. Other abnormal test results, such as decreased platelets or anemia, may be present. A computed tomography (CT) scan or magnetic resonance imaging (MRI) can confirm the presence of blood clots.

Treatment of Antiphospholipid Syndrome
Treatment for antiphospholipid syndrome is based on the individual's symptoms. Catastrophic APS requires hospitalization. Some individuals who have characteristic antibodies but no symptoms of APS may be started on daily low-dose aspirin to help reduce the risk of blood clots forming. If a blood clot is discovered, the person is started on anticoagulant medication such as Coumadin (warfarin) or Lovenox (enoxaparin).

With medication and lifestyle modifications (such as avoiding long periods of inactivity in which clots can form in the legs), most people with primary antiphospholipid syndrome lead normal, healthy lives. Those who have secondary APS may have additional problems due to their underlying rheumatic or autoimmune conditions.


source: rarediseases.about.com