Sjogren's Overview and Support Websites
Sjogren's Syndrome (SJS) is an immune-mediated disorder of exocrine glands.
The most common symptoms are dry eyes (kerato-conjunctivitis sicca) and
dry mouth (xerostomia). The two forms of the disease are the primary form,
which is not associated with another underlying autoimmune disorder, and
secondary SJS, which is associated with a connective tissue disease such
as rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis,
and other dysfunctional immune disorders. The pathogenic relationship between
the types appears to depend on the associated disease. In secondary SJS,
symptoms may not occur for years after the underlying disease is diagnosed.
This may change the symptoms; for example, in rheumatoid arthritis, ocular
involvement is more prevalent than oral symptoms. Oral discomfort,
difficulty eating, tooth decay, and oral infections occur frequently in
patients with SJS. Fortunately, therapeutic options are available that
may help reduce the morbidity associated with xerostomia.
Diagnosis
Primary SJS is most prevalent in women, with a gender ratio of 9:1. It may occur at any age but is most common in the fourth and fifth decades of life. The incidence varies greatly from 1/100 to 1/1000 people, depending on diagnostic criteria. The San Diego (SD) and European Epidemiology Center (EEC, also called European Community) criteria are used most often to make a diagnosis of primary or secondary SJS.
San Diego Criteria
- Symptoms and objective signs of ocular dryness
- Schirmer's test less than 8 mm wetting/5 minutes and
- Positive rose bengal or staining of cornea or conjunctiva to indicate keratoconjunctivitis sicca
- Symptoms and objective signs of dry mouth
- Decreased parotid flow rate using Lashley cups or other methods and
- Abnormal biopsy of minor salivary gland (focus score of >/= 2 based
on average of four evaluable lobules)
- Evidence of a systemic autoimmune disorder
- Elevated rheumatoid factor >/= 1:320 or
- Elevated antinuclear antibody (ANA) >/= 1:320 or
- Presence of anti-SS-A (Ro) or anti-SS-B (La) antibodies
- Secondary Sjögren's Syndrome requires signs and symptoms above plus clinical
features sufficient to allow diagnosis of rheumatoid arthritis, systemic
lupus erythematosus, polymyositis, systemic sclerosis, or biliary cirrhosis
- Exclusion criteria: sarcoidosis, preexistent lymphoma, human immunodeficiency virus (HIV) infection, or other known causes of keratitis sicca or salivary gland enlargement
European Epidemiology Center Criteria
- Ocular symptoms (at least one)
- Daily, persistent, troublesome dry eyes for more than 3 months
- Recurrent sensation of sand or gravel in the eyes
- Use of a tear substitute more than 3 times/day
- Oral symptoms (at least one)
- Daily feeling of dry mouth for at least 3 months
- Recurrent feeling of swollen salivary glands as an adult
- Drinking liquids to help wash down dry foods
- Objective evidence of dry eyes (at least one)
- Schirmer I test 5 mm or below/5 minutes
- Rose bengal score of 4 or greater according to van Bijsterveld system
- Histopathologic signs
- Minor salivary gland biopsy with focus score of 1 or greater
- Objective evidence of salivary gland involvement (at least one)
- Salivary gland scintigraphy
- Parotid sialography
- Unstimulated whole sialometry 1.5 ml/15 minutes or below
- Laboratory abnormality (at least one)
- Anti-SS-A or anti-SS-B antibody
- ANA
- IgM rheumatoid factor
- Exclusion criteria: preexisting lymphoma, acquired immunodeficiency Syndrome,
sarcoidosis, graft-versus-host disease
Sjogren's Overview