What is Sjogren's Syndrome?

"Sjögren’s (SHOW-grens) syndrome is an autoimmune disease that primarily affects moisture-producing glands. The damage caused to these glands leaves you with dry eyes and mouth. Other parts of the body (such as the skin, respiratory system, nerves, thyroid glands and digestive tract) can be affected by the inflammation as well.

In this disease, the body’s immune system mistakenly sends its disease-fighting cells to attack the lacrimal and salivary glands, which produce tears and saliva, respectively. Rather than protecting the body as it is intended to do, the immune system damages these glands causing dry eyes (called keratoconjunctivitis sicca) and dry mouth (xerostomia).

What causes it?

Scientists think that Sjögren’s syndrome is caused by a combination of genetic and environmental factors. Several different genes seem to be involved, but simply having one of these genes will not cause you to develop the disease. Some environmental trigger must activate the immune system. Researchers think that the trigger may be a viral or bacterial infection. Because most people with Sjögren’s syndrome are women, it is thought that hormonal factors play some role in the disease’s initiation.

Along with the main symptoms of dry eye and dry mouth, several other parts of the body can be affected by Sjögren’s syndrome.

• Eyes: Your eyes may burn and feel dry, gritty and itchy. They may appear red and can be sensitive to bright lights. Having dry eyes makes you more prone to getting eye infections.

• Mouth: Your mouth will feel dry, and you may have difficulty swallowing and speaking. Your sense of taste and smell may be altered. Because you are lacking natural saliva, which contains substances that fight bacteria and cleanse the mouth, you are susceptible to cavities and mouth infections. The fungal infection candidiasis (oral thrush) is particularly common among people with Sjögren’s syndrome and causes redness, burning and a white film in the mouth.

• Energy: Sjögren’s syndrome can cause extreme fatigue that can interfere with your daily life.

• Salivary glands: The saliva-producing glands located under your tongue, in front of your ears and near the jawline may become swollen and sometimes tender.

• Nose and throat: A dry nose and throat can cause a tickly feeling and persistent cough, leading to irritation and hoarseness.

• Skin: About half of people with Sjögren’s have dry skin. The skin may only itch, or it may also become so dry that it cracks and splits. Skin that is split or has been vigorously scratched is prone to infection. Some people with Sjögren’s (particularly when it is secondary to lupus) are sensitive to sunlight and can get sunburn from minor exposure.

• Vagina: Vaginal dryness is common in women with Sjögren’s syndrome. Painful intercourse is the most common complaint.

• Lungs: Pneumonia can be a complication of dry mouth when food becomes aspirated when swallowing is difficult or bacteria from the dry mouth can migrate to the lungs. The disease itself can cause inflammation of the bronchial tubes, windpipe and voice box.

• Kidneys: Some people with Sjögren’s syndrome have a diminished ability to concentrate urine. People may have distal renal tubular acidosis, which gives people acidic urine and can lead to calcium loss from bones. Parts of the kidney’s filtering system can be affected by this autoimmune disease, causing inflammation around the kidney’s filters (interstitial nephritis) or in the filters themselves (glomerulonephritis).

• Nerves: Peripheral nervous system involvement can cause carpal tunnel syndrome, peripheral neuropathy or cranial neuropathy. Carpal tunnel syndrome causes pain, numbness and tingling in the thumb and index and middle fingers. Peripheral neuropathy causes similar symptoms in the legs or arms. Cranial neuropathy causes face pain; loss of feeling in the face, tongue, eyes, ears or throat; and loss of taste and smell. Rarely, Sjögren’s syndrome can affect the brain and cause stroke-like symptoms.

• Digestive system: Inflammation in the esophagus, stomach, pancreas, and liver can cause painful swallowing, heartburn, abdominal pain and swelling, loss of appetite, diarrhea and weight loss. It can also cause hepatitis (inflammation of the liver) or cirrhosis (hardening of the liver).

• Connective tissues: The connective tissues of the body include the joints, muscles, bones, skin, blood vessel walls and lining of internal organs. Sjögren’s syndrome could affect any one of these on its own. In addition, several autoimmune disorders that effect the connective tissues are associated with Sjögren’s syndrome, including:
  • Polymyositis
  • Raynaud’s phenomenon
  • Rheumatoid arthritis
  • Scleroderma
  • Systemic lupus erythematosus
  • Vasculitis

• Thyroid disorders: Grave’s disease (overactive thyroid) or Hashimoto’s thyroiditis (underactive thyroid)

• Lymph nodes: About five percent of people with Sjögren’s develop lymphoma (cancer of the lymph nodes). Lymphoma occurring in patients with Sjögren’s syndrome often involves the salivary glands.

" - Arthritis Foundation