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Sjögren's Syndrome is a specific systemic inflammatory disorder that affects the mucous membranes. It causes dry mouth, decreased tear production, and dryness of other body’s membranes.

The cause is still unknown and it usually occurs in women around 50 years old. Patients with this condition may notice irritation, a gritty feeling, or painful burning in the eyes. Dry mouth or difficulty eating dry foods and swelling of the glands around the face and neck are also common. Some patients experience dryness of other mucous membranes and skin. Other non-specific symptoms that patients might experience include chronic fatigue and fever.


There are two types of this syndrome:

  • Primary Sjögren's syndrome - this type occurs in people with no other rheumatologic disease
  • Secondary Sjögren's occurs in people who have another rheumatologic disease, most often systemic lupus erythematosus and rheumatoid arthritis

Incidence

It is estimated that between 1 and 4 million Americans, or 1 to 2 percent of the population, have Sjögren's syndrome. This condition can affect people of any age, but symptoms usually appear between the ages of 45 and 55.

It affects women 10 times as often as men. It is also proven that about 50% of the patients also have rheumatoid arthritis or other connective tissue diseases, such as systemic lupus.

Possible causes of Sjögren’s syndrome

Unfortunately the cause of Sjögren's syndrome is not known, but most experts believe that this disease has an autoimmune background. People with this disease have abnormal proteins or antibodies in their blood suggesting that their immune system is reacting against their own tissue.
It is also proven that the decreased production of tears and saliva seen in this syndrome occurs because these glands are damaged by inflammation. Research suggests that genetic factors and possibly viral infections could be involved.

Symptoms of Sjögren’s syndrome

The majority of the patients with Sjögren’s syndrome have symptoms related to:

  • diminished tears and salivary gland functions
  • arthralgia
  • Raynaud’s phenomenon
  • fatigue

The patients usually complain of dry eye symptoms, including burning, itching or foreign body sensation and accumulation of thick secretions in the eyes. With time conjunctiva injection, reduced visual acuity and increased photosensitivity develop. 

Symptoms related to mouth

Drying of the mouth, a condition also known as Xerostomia, is frequent but variable in severity. Patients usually complain of difficulty in eating dry food, inability to speak continuously, oral soreness, changes in tasting and smelling and fissures of the tongue and lips. An increase in dental caries is due to decreased saliva volume and the relative loss of its antibacterial factors. Involvement of other exocrine glands occur less frequently.

Systematic features

Systemic features can complicate the course of the Sjögren’s syndrome and are seen in 30% of the patients. Most patients complain of:

  • easy fatigability
  • low-grade fever
  • myalgias
  • arthralgia
  • non-erosive

Renal involvement includes:

  • interstitial nephritis
  • hypostenuria
  • renal tubular dysfunction
  • Fanconi’s syndrome

Central nervous system involvement includes:

  • focal and diffuse defects
  • multiple sclerosis
  • progressive dementia
  • cognitive dysfunction
  • spinal cord involvement
  • myelitis

Diagnosis of Sjögren’s syndrome

The proper diagnosis of Sjögren’s syndrome should be based on the presence of two of the three following manifestations:

  1. keratoconjunctivitis sicca
  2. xerostomia
  3. connective tissue or lympho-proliferative disorder

Biopsy

Minor salivary gland biopsy serves as the cornerstone of the diagnosis.

Schirmer’s tear

Schirmer’s tear test is used for evaluation of tear secretion by the tear glands. The test is performed with strips of filtered paper slipped beneath the inferior lid of an anaesthetized eye. After 5 minutes the wetting length of the paper is measured. Wetting of less than 5 mm is a strong indication for diminished secretion.

Slit-lamp examination

Keratoconjunctivitis sicca, the product of decreased tear production, is diagnosed with slit-lamp examination. It is done after using Rose Bengal staining of the corneal epithelium. Rose Bengal is a dye which stains the devitalized epithelium of both the cornea and conjunctiva.
The differential diagnosis of Sjögren’s syndrome includes other diseases which may cause dry eyes or mouth or parotid salivary gland enlargement

Tests

Tests for Sjögren’s syndrome may include:

  • Antinuclear antibodies (ANA) (positive)
  • Rheumatoid factor (RF) (often positive)
  • Antibodies specific to Sjögren’s syndrome (SS): anti-SS-A and SS-B; SS-A is also called Ro, while SS-B is also called La (usually positive)

Complications of Sjögren’s syndrome

This syndrome carries some possible complications and most of them occur because of decreased tears and saliva. It is proven that patients with dry eyes are at the increased risk for infections around the eye and may have damage to the cornea. Dry mouth may cause an increase in dental decay, gingivitis, and oral yeast infections that may cause pain and burning.

There are not so many complications in other parts of the body in patients with Sjögren's syndrome. Pain and stiffness in the joints with mild swelling may occur in some patients, even in those without rheumatoid arthritis or lupus. Some patients also reported rashes on the arms and legs related to inflammation in small blood. Some neurological complications such as numbness, tingling, and weakness have also been described in some patients.

Treatment of Sjögren’s syndrome

Unfortunately there’s still no cure for Sjögren’s Syndrome. The first step is to moisturize and protect each problem area often through self-care and over-the-counter medications. The goal is to keep the auto-immune reactions, inflammation and extra-glandular symptoms in control.

Mouth treatment
Mouth treatment is rather easy and it consists of regular visits to the dentist for cleaning and good oral hygiene. Using sugar-free hard candy, gum, fluoride gels, floss and rinses minimize the risk of cavities. Over-the-counter and prescription artificial saliva are available. Smoking is prohibited. 

Eyes treatment
In order to avoid possible complications in the eyes, wind, breezes and smoke should be avoided. Goggles can be worn and over-the-counter or prescription artificial tears are also helpful. A surgery called punctal occlusion can help the tears accumulation.

Skin treatment
The skin may require 15-minute baths, moisturizing soaps and oil-based ointments to help restore water to it.

Medications
Medications that tend to spend body fluids should be avoided. Some mild pain-relieving medications including acetaminophen (Tylenol®) or Motrin® or Aleve® can reduce muscle or joint pain. There are even two prescription medications, Salagen® and Evoxac®, which are made to stimulate saliva production and may relieve the dry mouth symptoms. In some patients, the anti-rheumatic drug Plaquenil® has been beneficial in decreasing pain and salivary gland swelling.
In advanced cases of the disease, anti-inflammatory medications of increasing strength including non-steroidal anti-inflammatories, steroids, and various immunosuppressant medications may be prescribed. For patients with generalized symptoms, particularly when the disease affects internal organs, high doses of immunosuppressive medications may be necessary. Some of the medications used in these cases are Prednisone and, rarely, chemotherapy-type medications.

What is the prognosis?

Sjögren’s syndrome can damage vital organs of the body with symptoms that may remain stable, but also can worsen, or go into remission. Fortunately, a big majority of patients experiences only the mild symptoms of dry eyes and mouth, but some go through cycles of good health followed by severe disease. Many patients are able to treat problems symptomatically, while others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating. Most patients with Sjögren's syndrome remain healthy, but a a number of rare complications have been described, including an increased risk for some types of cancer.

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