Living With Sjögren's Syndrome

This is a paper I wrote and presented in high school, December 1987. The lack of Oxford commas frightens me.

Living with Sjögren’s Syndrome
18 December 1987

Do your eyes burn, tire easily or feel as if there is something in them? Do your eyes feel dry and are they often red and itch? Do you need to drink water often while talking? Are your tongue and gums frequently sore and cracked? Have you noticed any swelling around or under your ears/jaw?

A common definition of Sjögren’s Syndrome is a “chronic autoimmune and inflammatory disease characterized by diminished lacrimal and salivary gland secretion (sicca complex), resulting in dry mouth and dry eyes.” The term ‘autoimmune exocrinopathy’ (the drying out of moisture glands) is proposed to describe a more detailed and expanded definition. (Talal, Norman. 19-20)

The main symptoms are decreased production of secretions from the tear and salivary glands, as noticed in keratonjunctivitis sicca (KCS, dry eyes) and xerostomis (dry mouth). Mucous-producing glands affected are: the nose, pharynx, esophagus, trachea, lungs and any other mucous-producing membranes affected b reduction of glandular secretions. Extraglandular functions affected are: the skin spleen, liver, kidney, muscles, lungs, nerves, the brain, blood vessels and the joints. The symptoms of the eyes are: accumulation of thick, ropy strands at the inner cantous, particularly upon awakening; photosensitivity; eye fatigue, corneal ulceration, vascularization; and opacification, followed rarely by perforation. Symptoms of the mouth are: distressing oral insufficiency and/or adherence of food to buccal surfaces; fissures or ulcers on tongue, buccal membranes, or lips, particularly at the corners of the mouth; of liquids, particularly at meals; and, red dry tongue. Other symptoms are: non-deforming arthritis in joints; dry nose, throat, hoarseness, or mild bronchitis; stomach inflammation, constipation, and pancreatitis.

There are several ways of diagnosing for Sjögren’s Syndrome. Careful examination of the eyes, including measurement of tear production. Stimulation with lemon juice will help determine saliva production in the mouth. X-ray exams of salivatory (parotid) glands and cells from the lips to determine whether or not lymphocytes are present in those glands. Diagnosis can be made by evidence of sicca without other signs of connective tissue disease, enough to at least diagnose primary Sjögren’s Syndrome (discussed below) by two out of three of the following: (1) characteristic lymphocytic infiltrates (focus sore on labial salivatory gland biopsy), definite KCS, and associates extraglandular connective tissue or lymphoporliferative disorder. Blood counts can be used, often showing anemia and infrequently a low white count. Schrimer test (whetting of the eyes) and minor salivatory gland biopsy can also be used. The slit lamp test, lysozyme agar diffusion test (not routine), rose brngal staining, and tear breakup time are also popular diagnosing procedures.

The cause of Sjögren’s Syndrome is unknown. It is believed, however, that genetics and viruses may play a part.

There is no known cure for Sjögren’s Syndrome. Primary Sjögren’s Syndrome (1SS) contains the symptoms of KCS and xerostomia. People with 1SS have more severe problems, most notable bulky enlargement of glands around face, jaws and neck. There is higher kidney involvement, usually more frequent urination. There is also usually an underactive thyroid, as demonstrated in sluggishness, feeling cold, constipation and deepening of voice.

Secondary Sjögren’s Syndrome (2SS) has the symptoms of 1SS, but also have some type of connective tissue disease, such as rheumatoid arthritis, lupus, and polymyocitis. There may also be thickening and inflammation of arteries (arteritis nodosa). There can be joint pain, primarily in the fingers, wrists, and knees for a significant part of the day. There may be joint stiffness for an hour or more in the morning. Extreme coldness, pain, and color change may occur in the fingers and toes. Distinct rash can occur on cheeks, accompanied by hair loss, both of which could worsen with exposure to the sun. A “genetic marker” has been found in people with 2SS- HLA-DR3; however, presence of this marker does not necessarily mean that a person will contract 2SS.

The treatment of Sjögren’s Syndrome is dependent upon the symptoms of each individual. Treatment is aimed at systemic relief. For the eyes, artificial tears and/or eye ointment soothe and lubricate them. Moisture-retentive soft contact lenses can serve as eye bandages; however, frequnt lubrication is needed to reduce the risk of infection. Goggles and eye shields help retain moisture and are hepful while sleeping. Humidifiers are useful - the symptoms of sicca are substantially reduced. In severely advanced cases, punctal occlusion - a surgery to close the drainage canals to help retain moisture - may be necessary.

There are several treatments available for the mouth. Oral lubricants and a diet of soft foods is recommended; avoid the use of sugar whenever possible, which contributes to cavities. Also drink plenty of liquids to keep the mouth moist.

Sjögren’s Syndrome was first recognized in the late 1800’s-1920’s. In 1933, Swedish ophthalmologist Henrik Sjögren wrote a report about the connection between the sicca complex and the connective tissue diseases, which were described in a triad; dry eyes (KCS), dry mouth (xerostomia), and rheumatoid arthritis.

It must be concluded that Sjögren’s Syndrome is one of the most common and least diagnosed autoimmune diseases.

Works-Cited

  • Howard, Paul F. MD. Sjögren’s Syndrome - An Overview. Published by Sjögren’s Syndrome Foundation, Inc.
  • Litton, Bonnie and Steve. The Effects of Sjögren’s Syndrome Other Than Eye and Mouth. Sjögren’s Syndrome Foundation, Volume II, Issue 2.
  • Michelson, Paul E. MD, FACS. Living With Dry Eyes.
  • Sjögren’s Syndrome Foundation, Inc. The Moisture Seekers. SSF. Volume IV, Issue 3.
  • —. Response letter. 22 November 1987.
  • —. SSF fact sheet. 22 November 1987.
  • —. Your mouth feels parched. Twin Cities: SSF
  • Talal, Normal MD. How to Recognize and Treat Sjögren’s Syndrome. PA Drug Update. May 1984.
  • Tarail, Jane. Sjögren’s Syndrome: A Dry-eyed Diary. American Journal of Nursing. March 1987.