Sjogren syndrome

Definition
Sjogren syndrome is a systemic inflammatory disorder characterized by dry mouth, decreased tearing, and other dry mucous membranes. It is often associated with autoimmune rheumatic disorders.

Causes, incidence, and risk factors
The cause of Sjogren syndrome is unknown. The syndrome occurs most often in women 40 to 50 years old. It is rare in children. Pediatric patients usually present with another autoimmune disorder before developing the signs of Sjogren syndrome.

Dryness of the eyes and mouth are the most common symptoms of this syndrome; they may occur alone, or with symptoms associated with Rheumatoid Arthritis or other connective tissue diseases. There may be an associated enlargement of the salivary glands. Other organs may become affected. The syndrome may be associated with Rheumatoid Arthritis, Systemic lupus erythematosus, scleroderma, polymyositis, and other diseases. Sjogren syndrome affects 3% of the population.

Symptoms

     
  • dry or itching eyes  
  • dryness of the mouth  
  • difficulty swallowing  
  • loss of sense of taste  
  • severe dental cavities  
  • hoarseness  
  • fatigue  
  • Joint pain or joint swelling  
  • swollen glands  
  • cloudy cornea

Signs and tests
A physical examination reveals dry eyes and mouth. Mouth sores may be present because of the mouth dryness.

Tests:

     
  • positive antinuclear antibodies (ANA) test  
  • positive salivary gland biopsy  
  • positive rheumatoid factor (possible)  
  • tear test  
  • slit lamp examination with rose bengal dye

Treatment

The goal is to relieve symptoms. Dry eyes may be treated with artificial tears as often as needed or an eye-lubricating ointment may be applied at bedtime.

Dry mouth may be helped by sipping water throughout the day, chewing sugarless gum, and avoiding drugs that cause mouth dryness such as antihistamines and decongestants. There are also drugs available (e.g., Pilocarpine) that increase the flow of saliva. Frequent brushing and flossing of the teeth with regular dental visits may prevent severe dental cavities associated with the dry mouth.

Arthritis symptoms may be mild and are commonly treated with nonsteroidal anti-inflammatory medications and physical therapy.

Expectations (prognosis)
The disease is usually benign and prognosis (probable outcome) depends on associated diseases. There is an increased risk of lymphoma.

Complications

Calling your health care provider
Call for an appointment with your health care provider if you develop symptoms of Sjogren syndrome.

Johns Hopkins patient information

Last revised: December 5, 2012
by Potos A. Aagen, M.D.

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