Amyloidosis Sarcoidosis Graft vs. host disease
HIV disease (diffuse infiltrative lymphocytosis syndrome)
Bilateral chronic sialadenitis
Abbreviation: SS, Sjögren's syndrome.
TABLE 4 A Revised Classification Criteria for SS by the 2002 American-European Consensus Group
I. Ocular symptoms: a positive response to at least one of the following questions a. Have you had daily, persistent, troublesome dry eyes for more than 3 mo?
b. Do you have a recurrent sensation of sand or gravel in the eyes?
c. Do you use tear substitutes more than three times a day?
II. Oral symptoms: a positive response to at least one of the following questions a. Have you had a daily feeling of dry mouth for more than 3 mo?
b. Have you had recurrent or persistently swollen salivary glands?
c. Do you frequently drink liquids to aid in swallowing?
III. Ocular signs: that is, objective evidence of ocular involvement as defined as a positive result for one of the following a. Schirmer I test performed without anesthesia (<5 mm in 5 min)
b. Rose-bengal score or other ocular dye score (>4 according to van Bijsterveld's scoring system)
IV. Histopathology: in minor salivary glands (obtained through normal appearing mucosa) focal lymphocytic sialadenitis, evaluated by an expert histopathologist with a focus score >1 (2)
V. Salivary gland involvement: objective evidence of salivary gland involvement defined as a positive result for at least one of the following diagnostic tests a. Unstimulated whole salivary flow (<1.5mL in 15 min or <0.10 mL in 1 min)
VI. Autoantibodies: presence in the serum of the following autoantibodies: a. Autoantibodies to Ro(SSA) or La(SSB), or both
Rules for classification For primary SS
In patients without any potentially associated disease, primary SS may be defined as follows:
a. The presence of any four of the six items is indicative of primary SS, as long as either item IV (histopathology) or VI (serology) is positive b. The presence of any three of the four objective items (that is, items III, IV, V, VI)
c. The classification tree procedure represents a valid alternative method for classification, although it is should be more properly used in clinico-epidemiological survey (not shown)
In patients with a potentially associated disease (for instance, another well-defined connective tissue disease), the presence of item I or item II plus any two from items III, IV, and V may be considered as indicative of secondary SS Exclusion criteria
Past head and neck radiation; hepatitis C infection, HIV infection, preexisting lymphoma, sarcoidosis, graft vs. host disease, use of anticholinergic medication
Abbreviation: SS, Sjogren's syndrome. Source: Ref. 1.
including tumor necrosis factor-a (TNF-a), interferon-g, transforming growth factor-p, interleukin 6, and interleukin 10.
The pathogenesis of SS also includes polyclonal B-cell activation with germinal centers developing in the T-cell organ infiltrates, elevated circulating immunoglobulins IgG, IgA, and IgM, circulating autoantibodies [anti-SS-A, anti-SS-B, rheumatoid factor (RF), and antinuclear antibody], and elevated levels of B-cell proliferation factors BAFF and APRIL. Antagonist muscarinic receptor (M3) antibodies have also been identified in patients with SS, which may offer an explanation for the clinical findings of decreased exocrine secretion in this population. The B-cell component, in a few patients, can progress to mucosa-associated lymphoid tissue (MALT) lymphoma or high-grade B-cell non-Hodgkin's lymphoma.
The predominance of women affected by SS raises questions of genetic and hormonal roles. The SS genotype has not been defined, but HLA-DR3 has been observed in Caucasian patients with primary SS, although not in Japanese patients. Current evidence suggests that clinically distinct autoimmune diseases may be controlled by a common set of susceptibility genes. The influence of environmental factors will likely complicate the ultimate search for an SS susceptibility gene. There has been very little research in the area of hormonal influence on the pathogenesis of SS; however, dehydroepiandrosterone, a steroid hormone that is a precursor to both estrogen and testosterone, has shown no efficacy in the treatment of primary SS (4).
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