Sjogren's Syndrome

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Introduction

What is Sjogren’s syndrome?

Sjogren’s syndrome is a common autoimmune disorder that attacks moisture-producing glands of the body, such as the glands that produce tears and saliva. In an autoimmune disorder, the body’s immune system mistakenly attacks healthy tissues. In Sjogren’s syndrome, inflammation caused by this attack affects the glands’ ability to secrete moisture and mucus normally.

Sjogren’s syndrome can affect the glands in the eyes, mouth, throat, nose, airways, skin, digestive system, and vagina. In some cases, Sjogren’s syndrome can attack areas throughout the body, such as the lungs, kidneys, joints, blood vessels, and nervous system.

Symptoms of Sjogren’s syndrome are due to abnormal dryness of the affected glands and organs. Typical symptoms of Sjogren’s syndrome include dry eye, dry cough, dry mouth, and poor oral and dental health.

There are two types of Sjogren’s syndrome, primary and secondary. Primary Sjogren’s syndrome occurs in the absence of other autoimmune disorders. Secondary Sjogren’s syndrome occurs with other autoimmune disorders, such as lupus and rheumatoid arthritis, and is generally more serious than primary Sjogren’s syndrome. Secondary Sjogren’s syndrome is more likely than primary Sjogren’s syndrome to lead to complications.

Sjogren’s syndrome cannot be cured, but it can be treated to minimize symptoms and reduce the risk of complications. Treatment can include a combination of medication, good oral hygiene, and possibly surgery.

Serious complications, such as liver disease and lung disease, can occur with Sjogren’s syndrome, especially secondary Sjogren’s syndrome. 

Seek prompt medical care if you have symptoms of Sjogren’s syndrome, such as dry mouth and dry cough. Early diagnosis and treatment can help reduce the risk of complications. 


Symptoms

What are the symptoms of Sjogren’s syndrome?

Symptoms of Sjogren’s syndrome are the result of damaged moisture-producing glands and can affect the eyes, mouth, throat, nose, airways, skin, digestive system, and vagina. The parotid glands may become swollen. In some cases, symptoms of Sjogren’s syndrome can affect organs throughout the body, such as the lungs, kidneys, joints, blood vessels, and nervous system.

The types and severity of symptoms of Sjogren’s syndrome vary among individuals and according to the type of Sjogren’s syndrome. Symptoms generally develop slowly in both primary and secondary Sjogren’s syndrome. Symptom severity can range from mild to severe, and symptoms can remain constant, get worse, or go into remission in some cases. Symptoms generally begin in adults in their 40s.

Symptoms of Sjogren’s syndrome can include:

  • Changes in taste
  • Difficulty talking
  • Dry cough
  • Joint or muscle pain
  • Problems with chewing and swallowing
  • Problems with concentration and memory
  • Sensitivity to light (photophobia)
  • Swollen parotid glands
  • Skin rashes 

Symptoms that might indicate a serious condition

In some cases, Sjogren’s syndrome can lead to potentially serious complications, such as pneumonia, vasculitis, pancreatitis, and a higher risk of developing lymphoma. Seek prompt medical care if you have any of these symptoms:

  • Decreased urination
  • Eye pain or eye sores
  • High fever (higher than 101 degrees Fahrenheit)
  • Wet, loose cough that produces yellow, green, or white phlegm
  • Yellowing of the skin and whites of the eyes (jaundice)

Causes

What causes Sjogren’s syndrome?

The cause of Sjogren’s syndrome is not known, but it is believed that this autoimmune disorder may be triggered by a viral infection or a bacterial infection. Genetic factors may also play a role in Sjogren’s syndrome. Research indicates that the presence of certain genes may make you more susceptible to the abnormal immune process when confronted with an environmental trigger, such as a viral infection.

What are the risk factors for Sjogren’s syndrome?

The vast majority of people with Sjogren’s syndrome are women, and it generally appears during the fourth decade of life. However, Sjogren’s syndrome can affect all age groups, and it can occur in all races. You may be at higher risk of developing Sjogren’s syndrome if other members in your family had this condition. There is no way to prevent Sjogren’s syndrome or reduce your risk of developing the condition.

Treatments

How is Sjogren’s syndrome treated?

There is no cure for Sjogren’s syndrome, but with early recognition and treatment, it is possible to decrease symptoms and minimize the risk of complications. Treatment for Sjogren’s syndrome varies depending on the type of Sjogren’s syndrome, the severity of symptoms, the presence of complications, a person’s age and medical history, and other factors. Treatment plans use an individualized, multifaceted approach that may include:

  • Artificial tears and topical anti-inflammatory eye drops to help relieve dry eye

  • Good oral hygiene and regular dental exams and cleanings at least three times a year

  • Humidifiers to increase moisture in the environment

  • Hydrating with small sips of water. However, avoid excessive sips of water that can actually increase your dry mouth symptoms.
  • Avoiding caffeinated beverages and alcohol because they can increase dryness.
  • Immunosuppressant medications to reduce inflammation and the autoimmune response

  • Medications to relieve dryness of the mouth, such as a saliva substitute and drugs that stimulate the production of saliva

  • Chewing sugarless gum or slowly dissolving lemon drops or lozenges (sugarless is preferred) to increase saliva flow

  • Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen and aspirin, to control joint pain and muscle pain. Because NSAIDs can cause serious, even life-threatening side effects in some people, you should use NSAIDs only as recommended by your health care provider.

  • Surgery to close the tear ducts that drain tears from the eyes

  • Promptly treating any complications, such as pneumonia, corneal ulcerations, autoimmune hepatitis, and other liver problems

What are the possible complications of Sjogren’s syndrome?

Secondary Sjogren’s syndrome is more likely than primary Sjogren’s syndrome to lead to the most serious complications. Complications of either type of the condtirion include:

  • Adverse effects of treatment
  • Anemia and low white blood cell counts
  • Arthralgias (bone and joint pain) and myalgias (muscle pain)
  • Chronic eye pain and discomfort
  • Decreased vision
  • Dental cavities, mouth sores, and thrush
  • Lymphoma
  • Raynaud’s phenomenon
  • Scarring of the ocular surface
  • Vasculitis
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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2018 Oct 31
  1. Domino FJ (Ed.) Five Minute Clinical Consult. Philadelphia: Lippincott Williams & Wilkins, 2013.
  2. About Sjogren’s Syndrome. Sjogren’s Syndrome Foundation. http://www.sjogrens.org.
  3. NINDS Sjögren's Syndrome Information Page. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/sjogrens/sjogrens.htm.
  4. Sjögren's Syndrome. Medline Plus, a service of the National Library of Medicine National Institutes of Health. http://www.nlm.nih.gov/medlineplus/sjogrenssyndrome.html.
  5. Sjögren's Syndrome. National Institute of Arthritis and Musculoskeletal and Skin Diseases. http://www.niams.nih.gov/Health_Info/Sjogrens_Syndrome/#cause.
  6. Ramos-Casals M, Tzioufas AG, Stone JH, et al. Treatment of primary Sjögren syndrome: a systematic review. JAMA 2010; 304:452.
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