What is Sjogren's Syndrome?
What is Sjogren's Syndrome?
- Sjogren's Syndrome: Autoimmune disease damaging the eye tear ducts and other glands.
- Sjogren's Syndrome: WHAT: Keratoconjunctivitis sicca. Keratoconjunctivitis Sicca (KCS): an eye condition in which there is decreased tear production and inflammation of the cornea and conjunctiva. WHY: Keratoconjunctivitis sicca is found in a significant number of patients with rheumatoid arthritis. KCS has also been described in systemic lupus erythematosus, polyarteritis nodosa, and scleroderma. KCS plus xerostomia is called the sicca syndrome. The association of the sicca syndrome with rheumatoid arthritis is known as Sjogren's syndrome. HOW: Clinically, patients with KCS may present with itching, burning eyes. They may complain of a dry sensation or of a foreign body sensation of the eyes. The conjunctiva is hyperemic, and thick strands of mucus may cover the eye. With slit lamp examination there is less than 1 mm of tear width at the margins of the upper and lower lids. There is increased debris and mucus strands within the tear film, seen best after blinking. Tiny punctate opacities are seen throughout the corneal surface. The Schirmer tear test (which is most accurately performed without topical anesthesia) shows less than 5 mm of wetting of filter paper at the end of 5 minutes. Finally, rose bengal, which is a water soluble dye specific for devitalized cells and mucin, will stain the dessicated corneal and conjunctival cells a bright red color. REFS: 1) Henkind, P and Gold, DH: Ocular manifestations of rheumatic disorders. Rheumatology 4:13, 1973. 2) Havener, W: Synopsis of ophthamology. St. Louis, C.V. Mosby Company, 1979. 3) Anderson, JR; Whaley, K; Williams, J and Buchanan, WW: A statistical aid to the diagnosis of keratoconjunctivitis sicca. Quart J Med 41:175, 1972. DN19292-9.
Source - Diseases Database
Sjogren's Syndrome is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Sjogren's Syndrome, or a subtype of Sjogren's Syndrome,
affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)
Ophanet, a consortium of European partners,
currently defines a condition rare when it affects 1 person per 2,000.
They list Sjogren's Syndrome as a "rare disease".
Source - Orphanet
Sjogren's Syndrome: Introduction
Types of Sjogren's Syndrome:
Types of Sjogren's Syndrome:
Broader types of Sjogren's Syndrome:
How many people get Sjogren's Syndrome?
Prevalance of Sjogren's Syndrome: 1 to 4 million people with Sjogren’s Syndrome (NIAMS)
Prevalance Rate of Sjogren's Syndrome: approx 1 in 272 or 0.37% or 1 million people in USA [about data]
Undiagnosed prevalence of Sjogren's Syndrome: estimated 2 million
Undiagnosed prevalence rate of Sjogren's Syndrome:
approx 1 in 136 or 0.74% or 2 million people in USA [about data]
Prevalance of Sjogren's Syndrome:
Experts believe 1 to 4 million people have the disease.
(Source: excerpt from Questions and Answers About Sjögren's Syndrome: NIAMS)
Who gets Sjogren's Syndrome?
Patient Profile for Sjogren's Syndrome: Usually women after 50, though any age possible. Occurs rarely in children.
Profile for Sjogren's Syndrome:
Most--90 percent---are women. It can occur at any age, but it usually is
diagnosed after age 40 and can affect people of all races and ethnic
backgrounds. It's rare in children, but it can occur.
(Source: excerpt from Questions and Answers About Sjögren's Syndrome: NIAMS)
Gender Profile for Sjogren's Syndrome: Women 9:1, about 90% (NWHIC).
How serious is Sjogren's Syndrome?
Prognosis of Sjogren's Syndrome: Not life threatening. Not life-shortening. Lifelong treatment.
Complications of Sjogren's Syndrome:
see complications of Sjogren's Syndrome
Prognosis of Sjogren's Syndrome:
Sjogren's can damage vital organs of the body with
symptoms that may plateau, worsen, or go into remission. Some people may
experience only the mild symptoms of dry eyes and mouth, while others go
through cycles of good health followed by severe disease. Many patients
are able to treat problems symptomatically. Others are forced to cope with
blurred vision, constant eye discomfort, recurrent mouth infections,
swollen parotid glands, hoarseness, and difficulty in swallowing and
eating. Debilitating fatigue and joint pain can seriously impair quality
of life.
(Source: excerpt from NINDS Sjogren's Syndrome Information Page: NINDS)
...
The
disease has a benign course, but in rare cases malignant cancer of
the lymph nodes may develop. (Source: excerpt from Connective Tissue Diseases: NWHIC)
What causes Sjogren's Syndrome?
Causes of Sjogren's Syndrome: see causes of Sjogren's Syndrome
Cause of Sjogren's Syndrome: Autoimmune disorder primarily of saliva and tear glands.
Causes of Sjogren's Syndrome:
Normally, the immune system works to protect us from
disease by destroying harmful invading organisms like viruses and
bacteria. In the case of Sjögren's syndrome, disease-fighting cells
attack the glands that produce tears and saliva (the lacrimal and
salivary glands). Damage to these glands keeps them from working
properly and causes dry eyes and dry mouth.
(Source: excerpt from Questions and Answers About Sjögren's Syndrome: NIAMS)
Risk factors for Sjogren's Syndrome:
see
risk factors for Sjogren's Syndrome
What are the symptoms of Sjogren's Syndrome?
Symptoms of Sjogren's Syndrome:
see symptoms of Sjogren's Syndrome
Complications of Sjogren's Syndrome:
see complications of Sjogren's Syndrome
Can anyone else get Sjogren's Syndrome?
Contagion of autoimmunity:
generally not; see details in contagion of autoimmune diseases.
Sjogren's Syndrome: Testing
Diagnostic testing: see tests for Sjogren's Syndrome.
Misdiagnosis: see misdiagnosis and Sjogren's Syndrome.
How is it treated?
Doctors and Medical Specialists for Sjogren's Syndrome: Rheumatologist, Allergist, Dermatologist, Gastroenterologist, Gynecologist, Neurologist, Ophthalmologist, Otolaryngologist, Pulmonologist, Urologist
;
see also doctors and medical specialists for Sjogren's Syndrome.
Treatments for Sjogren's Syndrome:
see treatments for Sjogren's Syndrome
Research for Sjogren's Syndrome:
see research for Sjogren's Syndrome
Organs Affected by Sjogren's Syndrome:
Organs and body systems related to Sjogren's Syndrome include:
Name and Aliases of Sjogren's Syndrome
Main name of condition: Sjogren's Syndrome
Class of Condition for Sjogren's Syndrome: autoimmune
Other names or spellings for Sjogren's Syndrome:
Sjögren's syndrome, Sjogren's, Keratoconjunctivitis sicca, Keratitis sicca, Xerophthalmia, Dry eye syndrome, Dry eyes
Sjögren's syndrome, Sicca syndrome, Keratoconjunctivitis sicca
Source - Diseases Database
Dry eye syndrome, Keratitis sicca, Xerophthalmia
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)
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