Sjögren’s Syndrome (pronounce it as Shur-grin’s) is a very common, and highly prevalent chronic (gradual) autoimmune disease in which the white blood cells (WBCs) is directed to act against one’s own moisture producing glands, especially the salivary and the tear glands. As a result, people with Sjögren’s Syndrome experience symptoms like dry eyes, dry mouth, and dryness of other areas like nose, throat, and skin, which requires moisture. Dryness of eyes is medically called as keratoconjunctivitis sicca (KCS) and dryness of mouth is called as xerostomia. Sjögren’s Syndrome usually progresses gradually, and results in more complex disorder affecting other organs like intestinal tract, kidneys, liver, pancreas, blood vessels, lungs, brain, and joints.
Causes
The exact cause of Sjögren’s Syndrome is unknown, but it is an autoimmune disease that is caused when the WBCs invade the moisture glands that are responsible for fluid/mucus production, and finally resulting in decreased saliva (dry mouth), decreased tear production (dry eyes), and dryness of other mucous membrane. Scientists believe that Sjögren’s Syndrome is usually caused by the presence of a gene, which predisposed Caucasians, Japanese, African-Americans, and Chinese populations. But the mere presence of this gene do not cause Sjögren’s Syndrome, there must be a trigger that activates the immune system. The trigger may be bacterial or viral infection.
People at risk
Sjögren’s Syndrome is very common in women than in men. It is estimated that 9 out of 10 people with Sjögren’s Syndrome are women. It usually occurs in people above 40 years of age. People suffering from conditions like rheumatoid arthritis, connective tissue disease like lupus or scleroderma are at higher risk for Sjögren’s Syndrome.
Symptoms
The symptoms of Sjögren’s Syndrome vary in people according to the severity of the disease. The hallmark symptoms of Sjögren’s Syndrome are dryness of mouth and eyes that last for 3 months. Apart from this, the patient also experiences grainy and itchy feeling in the eyes, trouble speaking, swallowing, difficulty eating dry foods, feeling tired, and body aches. In severe cases, dryness can be noticed in other parts like nose, throat, vagina (in females), and skin. Dryness in the joints causes the joints to swell and finally results in arthritis. Dryness of Sjögren’s Syndrome can damage the lung tissue and result in persistent, long-lasting cough. The nervous system and the kidney are also damaged. Dryness also causes skin rashes.
Diagnosis
Diagnosis of Sjögren’s Syndrome is difficult because the signs and symptoms of the disease vary from person to person and the symptoms mimic the signs of various other diseases. Therefore a variety of test is done to rule out other conditions and confirm Sjögren’s Syndrome.
Blood test is done to check the number of blood cells, level of glucose, presence of antibodies specific to Sjögren’s Syndrome, clues for any inflammatory conditions, and indications for liver and kidney problems.
Various eye tests are also done.
- Shirmer test: This test helps to measure the dryness of eyes. A small piece of filter paper is placed under the lower eye lid to check the amount of tear produced.
- Slit-lamp test: Ophthalmologist uses a magnifying instrument to carefully observe the eyes and check for the severity of dryness and if any inflammation is present. A drop of liquid containing dye is dropped into the eyes to check any damage in the cornea of the eyes.
The salivary glands may also be tested.
- Sialogram: A special dye is injected into the parotid gland, located behind the jaws and in front of the ears. Then a special X-ray, called sialogram, of the salivary gland is taken to check the amount of saliva produced.
- Salivary scintigraphy: This involves injection of radioactive isotopes that are tracked to measure the level of salivary gland function.
- Spit test: In this test the patient is asked to spit into a measuring jar every 1 minute, for about 15 minutes. The amount of saliva is measured to determine the severity of mouth dryness.
- Biopsy: A small sample of the tissue is taken from the salivary glands around face or under the inner lips. Presence of WBCs in the sample confirms Sjögren’s Syndrome
As Sjögren’s Syndrome causes inflammation of the lung, an x-ray of the chest may be helpful in diagnosis. Urine test is done to detect whether Sjögren’s Syndrome has damaged the kidneys
Treatment
There is no cure for Sjögren’s Syndrome. Treatment for Sjögren’s Syndrome is not specific; it depends on the symptoms and the organs affected. For eye dryness, doctor may prescribe eye drops, and for mouth dryness some saliva substitutes. The patient can also try chewing sugarless gum and keep drinking lots of water to prevent mouth dryness. In few cases, doctor may prescribe medicine like pilocarpine and cevimeline, which increases saliva production and rarely improves tear production also. Yeast infection in the mouth is treated with anti-fungal medicines. If the patient complains of arthritis/joint pain then doctor may go for non-steroid anti-inflammatory drug, like ibuprofen to reduce swelling and pain, or other arthritis remedies may be recommended. Corticosteroids medicines are given to patients to suppress the inflammation that threatens the well being of lungs, kidneys, blood vessels, and nervous system. In case of dry skin unscented skin lotions are recommended to avoid skin dryness. Saline nasal spray and vaginal lubricants are recommended for patients suffering from nasal and vaginal dryness. Immunosuppressive drugs like hydroxychloroquine, methotrexate, and cyclophosphamide are prescribed to suppress the activity of immune system. Other medicines are also prescribed depending on the organ that is being affected by Sjögren’s Syndrome.
Outlook
Dryness of eyes and mouth can be really a very uncomfortable issue. Though there is no cure for Sjögren’s Syndrome, patient with it should learn to cope up and take extra care in keeping their eyes and mouth moist.
