Primary Sjogren’s Syndrome

  • Sjögren’s syndrome is a chronic (longlasting) 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 moistureproducing glands, including salivary (salivaproducing) glands and lacrimal (tearproducing) 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 Inner angle thick secretions
  • 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
  • Swelling of the glands around the face and neck are also common.
  • Pain and stiffness in the joints with mild swelling may occur in some patients.

Less common features of Sjögren’s syndrome are:

  • Irritation of the nerves in the arms, hands, legs, or feet
  • Feeling of numbness or tingling
  • Thyroid gland abnormalities
  • Skin rashes on the arms and legs related to inflammation in small blood vessels (vasculitis
  • Memory loss, difficulty concentrating or confusion
  • Gastrointestinal problems such as acid reflex, bloating, abdominal pain, or diarrhea
  • Inflammation of the lungs, kidneys (unlike lupus nephritis), liver, or pancreas may occur rarely and be difficult to diagnose.
  • Cancer of the lymphatic tissue Adult Hodgkin Lymphoma >(occurs inup to 5% of patients with the disease).

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 moistureproducing glands.
  • The exact cause for the abnormal immune response in Sjögren’s syndrome is unknown. There are four factors that may work together to cause the
    • abnormal immune response
    • inheritance
    • sex hormones inheritance
    • environment

Certain people may have a genetic or inherited factor that makes them more likely to develop Sjögren’s syndrome.

What are the forms of Sjögren’s syndrome?

Sjögren’s syndrome occurs in two basic forms:

  • Primary Sjögren’s syndrome – the disease by itself, not associated with any other illness
  • Secondary Sjögren’s syndrome – disease that develops in the presence of another autoimmune disease such as rheumatoid arthritis, systemic lupus erythematosus, or psoriatic arthritis

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 middleaged 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, antiSSA,orantiSSB(also known as antiRo or antiLa), even rheumatoid factor.
  • Biopsy of the inner lip (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 secondary Sjögren’s syndrome diagnosed?

  • Secondary Sjögren’s syndrome is generally diagnosed when someone with an established autoimmune disease such as rheumatoid or systemic lupus erythematosusdevelops extreme dryness of the eyes and mouth.
  • This diagnosis only rarely requires a lip biopsy.

Can other problems mimic Sjögren’s syndrome?

  • The use of certain medications such as tricyclic antidepressants and antihistamines can cause the symptoms of Sjögren’s syndrome.
  • Radiation treatments to the head and neck and other autoimmune disorders can also cause severely dry eyes and mouth.
  • Hepatitic C, sarcoidosis, and HIV infection can also cause these dry symptoms.

How is Sjögren’s syndrome treated?

  • There is no cure for Sjögren’s syndrome, but it can be treated and managed.
  • The goals of treatment are to decrease discomfort and reduce the harmful effects of dryness. Generally, physicians 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 sugarfree
    • gums can be helpful.
  • Taking frequent sips of water without swallowing (spitting it out) may improve dry mouth.Ifpatients 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 regularlyand 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 Medications that reduce gastric acid (such as proton-pump inhibitors and H2 blockers) may lessen symptoms of acid reflux.
  • 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 (Rheumatrex), azathioprine ,mycophenolate (Cellcept) 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.

Living with Sjögren’s syndrome

  • Sjögren’s syndrome cannot be cured, but in many cases proper treatment helps to alleviate symptoms.
  • People with Sjögren’s syndrome usually are able to live normal lives with very few adjustments.
  • When adiagnosis is made, many patients must focus a great deal of attention dealing with dry eyes and dry mouth, but these symptoms tend to subside with time.
  • Any pain or redness in the eyes should be evaluated promptly, as this may signal an infection.
  • To reduce risk for cavities and other dental problems, patientsmust pay close attention to proper oral hygiene and regular dental care.
  • Patients should see their physician regularly for general health screening. They also should pay close attention to any abnormal swelling in the glands around the face or neck, under the arms, or in the groin areas, as this may be a sign of lymphoma body pain.