This is an autoimmune disease, where the body’s immune system attacks various glands in the body, predominantly, the salivary glands and tear glands, cause dry mouth and eyes. The condition affects females more than males, most commonly middle-aged women. The glands affected are infiltrated by lymphocytes, which are a type of white blood cells. The infiltration leads to the destruction of the cells responsible for secretions and results in dryness of the corresponding region.
Classification of Sjogren’s Syndrome
Primary Sjogren’s Syndrome: This is a standalone disease with symptoms occurring from only Sjogren’s syndrome
The diagnosis is based on clinical examination and tests to demonstrate the decreased function of the various glands involved. The following tests are routinely done
Sialometry: this tests the extent of saliva production
Imaging: Salivary gland imaging using ultrasound and MRI scans
Eye examination using a slit lamp: this equipment allows a detailed examination of the surface of the eye to look for superficial ulcers that can occur from dryness.
Schirmer’s test: This tests the function of the tear glands.
Biopsy of the labial gland in the lip: This may reveal infiltration of the gland by lymphocytes.
Blood tests to look for certain antibodies:Ro/SS-A and La/SS-B are two of them routinely tested for, although these may be found in various other autoimmune disorders too.
Erythrocyte Sedimentation Rate (ESR): this is tested for in the blood and is raised in inflammatory states. It is generally found to be elevated in Sjogren’s syndrome.
Treatment of Sjogren’s Syndrome
The treatment is tailored to address symptoms from gland dysfunction and for other symptoms:
Drugs to stimulate tear secretion: similar to treatment of dry eyes
Soft contact lenses, corneal transplantation in severe cases
Avoiding smoke-filled and windy areas
Systemic symptoms: anti-inflammatory drugs like steroids, methotrexate may be helpful
Raynaud phenomenon: protection from the cold, calcium channel blockers (vessel dilators)
Complications of Sjogren’s Syndrome
The most significant complication is the occurrence of malignancy or cancer in the affected gland. Individuals with long-standing Sjogren’s syndrome can develop lymphoma of the gland.
Monitoring for symptoms suggestive of lymphoma (fevers, sweating, weight loss, and sudden growth of a mass) is important with early diagnosis and management proving beneficial in curing the patient.
Prognosis of Sjogren’s Syndrome
Sjogren’s Syndrome is a chronic disease that progresses slowly over many years. It may be problematic if associated with other autoimmune diseases. Most individuals live normal lives with the disease. Adequate management of symptoms and close monitoring for transformation to lymphoma, with appropriate treatment, will ensure good survival and quality of life.
Resources:
Haralampos M. Moutsopoulos, Athanasios G Tzioufas, Sjogren’s Syndrome, Harrison’s Principles of Internal Medicine, 19th Edition, 2015.