An Overview of Sjogren's Syndrome

A Systemic, Autoimmune Disease

Eye drops for dry eyes.
choja/E+/Getty Images

Sjogren's syndrome—named after the Swedish doctor, Henrik Sjögren, who discovered it in the early 1900s—is an autoimmune, inflammatory disease which can occur as a primary condition with no other rheumatic disease or as a secondary condition along with another rheumatic condition. About 50 percent of Sjogren's syndrome occurs as the sole condition. The other 50 percent of Sjogren's syndrome occurs as a secondary condition, most likely with rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis, or dermatomyositis.

According to the Sjogren's Syndrome Foundation, Sjogren's syndrome is one of the most prevalent autoimmune conditions and affects as many as 4 million Americans. Ninety percent of Sjogren's syndrome patients are women. The average age of onset is over 40 years old—but men, women, and children can develop Sjogren's syndrome at any age.

Cause of Sjogren's Syndrome

The cause of Sjogren's syndrome is unknown. According to the American College of Rheumatology, genetic factors and viral infections may increase the risk of developing the syndrome. Hormones may also be a factor.

Symptoms Associated With Sjogren's Syndrome

According to the Sjogren's Syndrome Foundation, symptoms may include:

  • Dry eyes which may also feel gritty or burning
  • Dry mouth
  • Difficulty chewing, swallowing, and talking
  • Cracked or sore tongue
  • Dry, burning throat
  • Difference in taste or smell
  • Dental decay
  • Oral yeast infections
  • Joint pain
  • Digestive difficulties
  • Dry nose and skin
  • Swelling of glands around face
  • Vaginal dryness
  • Fatigue
  • Enlarged lymph nodes

Rare complications linked to Sjogren's syndrome include:

  • Rashes on arms and legs related to vasculitis
  • Inflammation in the lungs, liver, and kidney
  • Neurological complications resulting in numbness, tingling, and weakness
  • Lymphoma in a small number of patients

Diagnosis of Sjogren's Syndrome

Along with the symptoms, results from a physical examination and certain diagnostic tests help to formulate the diagnosis of Sjogren's syndrome. No single test can confirm the diagnosis by itself. Tests which help to formulate the diagnosis include:

  • Antinuclear antibody (ANA test) - 70 percent of Sjogren's patients test positive for ANA, but the antibody may be present in healthy people, too.
  • SSA (anti-Ro) and SSB (anti-La) - Antibodies typically found in people with primary Sjogren's syndrome. Not all Sjogren's patients test positive for SSA and SSB.
  • Rheumatoid factor - 60 to 70 percent of people diagnosed with Sjogren's syndrome are positive for rheumatoid factor.
  • Immunoglobulins - Normal blood proteins are elevated in Sjogren's syndrome.
  • Special ophthalmologic or dental tests may be ordered to check for abnormal dryness or other abnormalities. Tests may include: Schirmer's test for dry eyes (doctor puts paper strips under lower eyelids and measures wetness on the paper after 5 minutes); slit lamp exam (performed by an ophthalmologist using equipment to magnify and examine the eye, checking for dryness and inflammation); staining with vital dyes (checking for damage to the surface of the eye); mouth exam (the doctor checks for abnormalities and may obtain a saliva sample for quality check); lip biopsy (doctor removes tiny salivary glands from lower lip for microscopic examination).

    Treatment of Sjogren's Syndrome

    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:

    A Word From Verywell

    The average time from the onset of symptoms to diagnosis of Sjogren's syndrome is said to be more than 6 years. After all, there are many causes for dry eyes and dry mouth that need to be ruled out. Dry mouth and dry eyes are the most common symptoms associated with Sjogren's syndrome, but the symptoms are not exclusive. Certain medications, taken for other conditions, can cause dryness. Even menopause is associated with symptoms of dryness. It's important not to jump to conclusions because you are experiencing dry eyes or dry mouth. If you have these symptoms, see a doctor for a thorough evaluation and diagnosis. Rheumatologists manage Sjogren's syndrome patients, while dentists and eye doctors may treat specific symptoms. Sjogren's syndrome is a treatable condition. With effective treatment, most people can live well.

    We would be remiss not to mention the simple remedies for dry eyes. Blink your eyes to moisten them. Blink 5 or 6 times a minute. Protect your eyes from wind and drafts. Be sure to use a humidifier in your home. Avoid smoke, and avoid eye makeup that can be irritating. Similarly, there are common sense tips for managing dry mouth. Chew sugarfree gum or suck on sugarfree hard candy. Sip water throughout the day. Use lip balm to soothe dry lips.

    Sources:

    About Sjogren's Syndrome. Sjogren's Syndrome Foundation.

    Questions and Answers About Sjogren's Syndrome, NIAMS, Updated September 2016.

    Udell, James, MD. Sjogren's Syndrome. American College of Rheumatology. Updated March 2017.

    Continue Reading