What is Sjogren's Syndrome
Sjögren's syndrome (SS) is a chronic inflammatory and lymphoproliferative disease with autoimmune features characterized by a progressive mononuclear cell infiltration of exocrine glands, notably the lacrimal and salivary glands (autoimmune exocrinopathy). These lymphoid infiltrations lead to dryness of the eyes (keratoconjunctivitis sicca), dryness of the mouth (xerostomia), and frequently, dryness of the nose, throat, vagina, and skin. SS is associated with the production of autoantibodies because B cell activation is a consistent immunoregulatory abnormality. The spectrum of the disease extends from an organ-specific autoimmune disorder to a systemic process (musculoskeletal, pulmonary, gastric, hematologic, dermatologic, renal, and nervous system involvement). SS may occur alone (primary) or in association with almost any of the autoimmune rheumatic diseases (secondary), the most frequent being rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). SS also is associated with an increased risk for B cell lymphoma development.
Clinical Features of Sjogren's Syndrome
The clinical spectrum of SS is highly variable, extending from an organ-specific autoimmune exocrinopathy to a systemic disease affecting multiple organs. The disease is characterized by insidious onset, with dry eyes and mouth as the most common clinical presentation in adults. The patients may often have difficulty in specifying the time of the onset of the disease
Sjogren's Syndrome is rare in children, in whom bilateral parotid swelling may be the predominant clinical feature, both at onset
and during the disease course
). In adults, the disease manifestations are not influenced by age
but several studies have demonstrated that young adults with primary Sjogren's Syndrome have a higher frequency of autoantibodies than do middle-aged and elderly patients, indicating a more active immunologic disease process.
Symptoms of Sjogren's Syndrome
European classification criteria screening questions for dry eye and mouth symptoms
|I. Ocular symptoms: a positive response to at least one of three selected questions:
- Have you had daily, persistent, troublesome dry eyes for longer than 3 months?
- Do you have recurrent sensation of sand or gravel in the eyes?
- Do you use tear substitutes more than 3 times a day?
|II. Oral symptoms: a positive response to at least one of the three selected questions:
- Have you had a daily feeling of dry mouth for longer than 3 months?
- Have you had recurrently or persistently swollen -salivary glands as an adult?
- Do you frequently drink liquids to aid swallowing dry food?
EU-USA classification criteria for Sjögren's syndrome
||Symptomatic xerostomia for >3 months: a positive -response to at least one of the above three EU oral screening questions
||Symptomatic dry eyes for >3 months: a positive response to at least one of the above three EU ocular screening questions recurrent
||Positive Schirmer I test 5 mm/5 min or rose Bengal score (or other ocular dye score, e.g., lissamine green)
||Abnormal labial gland biopsy (focus score 1)
||Positive result for unstimulated whole salivary flow ( 1.5 mL in 15 min) or abnormal parotid sialography or salivary scintigraphy
||Antibodies to Ro (SS-A) or La (SS-B), or both
|Primary Sjögren's syndrome
|The presence of any 4 of the 6 items listed above, as long as either item IV (histopathology) or VI (serology) is -positive. However, to avoid the misclassification of nonsymptomatic patients, an additional criterion for primary Sjögren's syndrome has been added, namely, three positive results out of the four objective items (ocular signs, histopathology, salivary gland involvement, or serology).
|Secondary Sjögren's syndrome
|In patients with another connective tissue disease such as rheumatoid arthritis, the presence of item I or item II plus any two from among items III, IV, and V may be -con-sidered as indicative of secondary Sjögren's syndrome.
|Any patient with past head and neck radiation treatment, hepatitis C infection, acquired immunodeficiency -syndrome, preexisting lymphoma, sarcoidosis, graft-versus-host disease, use of anticholinergic drugs