Sjogren’s Syndrome: Symptoms, Diagnosis and Treatment

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
• Secondary Sjogren’s Syndrome: This variant is associated with other autoimmune diseases like rheumatoid arthritis, systemic lupus erythematosus, and scleroderma among others

Symptoms of Sjogren’s Syndrome

The symptoms are primarily due to the decreases secretion from the various glands that are affected

• Dry mouth or xerostomia
• Difficulty swallowing dry food
• Difficulty speaking
• Burning sensation in the mouth
• Dental cavities and tooth decay
• Bad breath
• Dry eyes
• Burning sensation and redness
• The feeling of sand or grittiness in the eyes
• Thick and viscous tears
• Eye strain and fatigue
• Sensitivity to light
• Lungs: Mucous glands in the airways can get involved with the production of thick secretions that can lead to breathlessness and cough
• Pain during sexual intercourse or dyspareunia: due to decreased secretions from a gland in the genital region

The earliest symptoms are dryness of the mouth and eyes. These individuals may later develop symptoms related to the inflammatory process in the body.

• Fevers
• Muscle aches
• Joint pain and swelling
• Raynaud phenomenon: color changes and pain in the hands-on exposure to cold
• Kidney disease

Diagnosis of 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:

• Dry mouth
• Good oral hygiene
• Adequate hydration with water
• Drugs to stimulate secretion: pilocarpine or cevimeline
• Medications to prevent fungal infection: nystatin, clotrimazole lozenges
• Dry eyes
• Artificial tears: methylcellulose, polyvinyl alcohol
• 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.
• Nancy Garrick DD. Sjogren’s Syndrome [Internet]. National Institute of Arthritis and Musculoskeletal and Skin Diseases. 2017 [cited 2018 May 28].