Primary Sjogren’s Syndrome

Sjögren’s syndrome is a chronic (long lasting) disorder that causes insufficient moisture production in some glands of the body, primarily in the eyes and mouth. 

Sjögren’s syndrome occurs when a person’s normally protective immune system attacks her/his body and destroys moisture producing glands, including salivary (saliva producing) glands and lacrimal (tear producing) glands.

The lungs, bowel, and other organs may be affected, but relatively less often.  

What Are The Symptoms Of Sjögren’s Syndrome?

The main symptoms of Sjögren’s syndrome are: Extremely dry eyes, causing a feeling of grit or sand, burning, and redness

Extremely dry mouth and throat, causing: difficulty chewing and swallowing, especially dry food such as crackers, decreased or altered sense of taste ,difficulty speaking, increase in dental cavities and even tooth loss at early age ,gingivitis (gum inflammation), and oral yeast infections (thrush) that may cause pain and burning

Some patients experience dryness in the nasal passages, throat, vagina

Dry cough or hoarseness

Enlarged parotid glands (located at the angle of jaw), looking like an infection

Excessive fatigue :Aches and pains in muscles and joints, and even the whole body, similar to fibromyalgia pain

Pain and stiffness in the joints with mild swelling may occur in some patients. 

What Causes Sjögren’s Syndrome?

Normally, the immune system (the body’s defense system) protects the body from infection and foreign substances such as bacteria and viruses. 

In autoimmune diseases such as Sjögren’s syndrome, the immune system triggers an inflammatory response when there are no foreign substances to fight off. 

This inflammatory response causes the body’s white blood cells to attack and destroy its own moisture producing glands.

Who is affected by Sjögren’s syndrome?

Sjögren’s syndrome occurs in about 0.5 to 1% of the population. 

More than 90% of people affected by Sjögren’s syndrome are women.  The disease can affect people of any race or age, but affects mostly middle aged individuals.

How is primary Sjögren’s syndrome diagnosed?

The diagnosis of Sjögren’s syndrome is based on several factors, including:

• Presence of dry eyes and mouth. Dry eyes can be diagnosed by an ophthalmologist (eye doctor) by measuring tear production or carefully examining the cornea (clear part of the eye).
• Certain laboratory tests also suggest that dry eyes and mouth are caused by autoimmune mechanisms. Examples include the presence of autoantibodies in the blood, known as ANA, anti SSA, or anti SSB (also known as anti Ro or anti La), even rheumatoid factor. 
• Biopsy of the inner lip done by best rheumatologist (performed in some cases to prove the diagnosis of primary Sjögren’s syndrome). The biopsy may show the inflammation that is damaging the salivary glands. 

How Is Sjögren’s Syndrome Treated?

There is no cure for Sjögren’s syndrome, but it can be treated and managed if managed with the help of rheumatologist.

The goals of treatment are to decrease discomfort and reduce the harmful effects of dryness. Generally, best rheumatologist use medications to control symptoms (symptomatic treatment). The type of treatment will be tailored to each patient’s symptoms and needs.

• Good oral hygiene may prevent or reduce dental decays, infections, or tooth loss: Toothpastes (biotene type) and oral gels are available for people with dry mouth symptoms.
• These products may also have antibacterial action to reduce the severity of dental cavities over a long period of time.
• Chewing sugar free gums can be helpful. 

Taking frequent sips of water without swallowing (spitting it out) may improve dry mouth. If patients develop yeast infections, anti-fungal therapies may be used.

Humidifiers and nasal saline irrigation may improve nasal dryness. 

Dry eyes are mainly treated with the use of artificial tears

• Artificial tears can be used regularly and more often in dry environmental conditions
• While artificial tears are helpful, they often do not last long enough. Thicker preparations (gel form) that last longer are available. These are often used at bedtime because they can sometimes cause blurry vision. 
• Eye doctors can prescribe an eye drop called Restasis to treat more severe form of dry eyes.
• A small procedure called punctal plugs, to slow the disappearance of tears, is another treatment option when artificial tears are not sufficient. 

Medications that tend to reduce body fluids should be avoided.

Mild pain relieving medications (analgesics), including acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs )can reduce muscle or joint pain.

In some patients, the antirheumatic drug hydroxychloroquine has been beneficial in decreasing pain and salivary gland swelling and improving fatigue, muscle pain, joint pain, or rash. This drug generally does not help with dry symptoms, however, Some patients benefit from using prescription medications that stimulate saliva flow, such as pilocarpine or cevimuline

For patients with internal organ symptoms (particularly when the disease affects internal organs), steroids and immunosuppressive medications may be used. These include medicines such as prednisone (a steroid) and, rarely methotrexate, azathioprine ,mycophenolate or cyclophosphamide

Balance of rest and exercise

Guided exercise programs can help patients overcome fatigue, maintain flexibility, and overcome joint and muscle pain. Good sleep hygiene is helpful for improving fatigue and glands.