Introduction  

Sjögren's syndrome refers to an autoimmune condition characterized by white blood cells attacking healthy tissue and organs. As a result of the disease, the immune system attacks the glands that help to lubricate the mouth and eyes, leading to dry mouth and dry eye.

An individual may also experience dryness in other parts of the body that require moisture like the skin, throat, and nose. Sjögren's syndrome also affects the kidneys, nerves, digestive organs, blood vessels, lungs, and joints.
 

Also Known As

  • Sicca syndrome
     
  • Sjogren syndrome
     
  • Keratoconjunctivitis sicca
     
  • Gougerot-Sjogren syndrome
     
  • Sjogren-Gougerot syndrome
     
  • Secreto-inhibitor-xeroderma stenosis
     
  • Dacryosialoadenopathia atrophicans
     
  • Gougerot-Houwer-Sjogren syndrome
     
  • Keratoconjunctivitis sicca-xerostomia

 

Causes & Risk Factors 

Sjögren syndrome is associated with immune system dysfunction, where the immune system damages healthy parts of the body. Researchers believe that Sjögren’s syndrome results from a combination of factors from the environment and genetics. The many various genes may predispose an individual to the risk of developing Sjögren’s syndrome. However, the disease’s development is triggered by environmental factors such as bacterial or viral infections. The body's ability to fight infection reduces due to genetic variations. 

Although the disease can affect both men and women of any age and race, it is more common among women 40 years and above. Sjögren's syndrome is often associated with conditions like lupus and rheumatoid arthritis.
 

Signs & Symptoms 

Generally, Sjögren's syndrome results in common symptoms like joint pains and dry mouth. Other symptoms include fatigue, dry skin, vaginal dryness, swollen salivary glands, and rashes, especially after exposure to the sun.

The disease affects the eye in the form of dry eye. Signs and symptoms of dry eye may include:

  • Blurry vision
     
  • Burning eyes
     
  • Parched eyes
     
  • A gritty feeling in the eye
     
  • Extreme sensitivity to light
     
  • Discomfort from contact lens wear
     
  • Blepharitis (inflamed eyelid margins)

 

Diagnosis

To diagnose Sjögren's syndrome, the eye doctor takes a patient’s medical history and conducts several clinical and ophthalmic tests. Mouth tests are done to measure the amount of saliva. A blood test may be ordered to check for antibodies and blood markers common to Sjögren's syndrome patients. The eye doctor may refer the patient to a rheumatologist to review the blood tests and assist with treatment. The doctor may also request for a lip biopsy to confirm Sjögren's syndrome.

Specific tests to confirm Sjögren's syndrome disease associated with dry eye include:

  • Examining meibomian glands located in the eyelids. Blocked or swollen meibomian glands can cause dry eye
     
  • Schirmer's test where the doctor measures the quantity of tears by placing a small paper strip beneath the lower eyelid for about five minutes
     
  • A dye test where the doctor can view the rate of tears drying up under a microscope. The doctor can see the effect of dry eye on the cornea and other parts of the eye

 

Treatment

Currently, no known cure for Sjögren's syndrome exists. Treatment is aimed at relieving the symptoms. Concerning the eye, treatment focuses on the relief of dry eye symptoms. Because it's an ongoing condition, symptoms of dry eye should be treated; otherwise, the eyes remain very uncomfortable and irritated. Though rare, failure to treat dry eyes can lead to infection and scarring and affect vision. 

Different people will require different types of treatment and the options include:

  • Treatment of meibomian gland dysfunction
     
  • Use of a humidifier in case the air is too dry at home
     
  • Drugs to treat autoimmune disorders to help with dryness
     
  • Medications that help the body produce more saliva and tears
     
  • Avoiding medications like allergy medications that may induce dryness
     
  • Drinking plenty of water or sucking on candy (sugar-free), gels, and lozenges
     
  • Artificial tears and ointments to lubricate the eyes and prevent the eyes from drying out too quickly

 

Prognosis & Long-Term Outlook

Sjögren's syndrome is a life-long condition and requires that patients are informed of its management. Different people are affected differently, with some treating it as a nuisance while others are heavily impacted daily. The majority of Sjögren's syndrome patients lead healthy lives without severe complications. However, patients are at risk of developing dental problems and infections involving the eyes because of the constant decrease in saliva and tears.

Though rare, certain complications may arise, and they include:

  • Skin rash
     
  • Abnormal kidney and liver function tests
     
  • Problems with the lungs (pneumonia-like)
     
  • Numbness and weakness due to neurologic problems
     
  • Increased risk to non-Hodgkin lymphoma (a type of cancer)

 

Prevention & Follow Up

The patient can do certain things to prevent ocular surface dryness and other forms of dryness associated with Sjögren's syndrome. They may include:

  • Practising good oral hygiene
     
  • Wearing goggles or spectacles
     
  • Avoiding smoking or taking alcohol
     
  • Avoiding looking at screens or reading for long periods
     
  • Reduction of systemic medications that can cause or increase dryness
     
  • Avoidance of specific environments like smoke-filled rooms and windy places
     
  • Being careful about where to place heating ducts or air conditioning at home
     
  • Avoidance of specific environments like smoke-filled rooms and windy places
     
  • Being careful about where to place heating ducts or air conditioning at home
     

Seeing the physician regularly for a general check-up or seeking ophthalmic care in case of red, painful eyes, or discharge in the eyes