Etiology |
Unknown (possible viral exposure) |
|
Clinical |
Xerostomia Keratoconjunctivitis sicca |
|
Histological |
Polyclonal lymphocytic infiltrations in the salivary and lacrimal glands Lymphocytic focus, >50 mononuclear cells/mm2 (CD4+ > CD8+)Monoclonal B cell proliferation Progressive destruction of the acinar and ductal cells |
|
Serological |
Hypergammaglobulinemia Anti-SSA/Ro and anti-SSB/La autoantibodiesAnti-α-fodrin autoantibody Rheumatoid factor Antinuclear antibodiesAnti-type 3 acetylcholine muscarinic receptor |
|
Additional organ involvement |
Heart, blood vessels, lungs, liver, pancreas, stomach, kidneys, bladder, thyroid gland (secondary SjS) |
|
Immunobiology |
Diminished apoptosis of lymphocytes Abnormal MHC expression, H2+-glandular ductal epithelium Epithelial cell expression of Fas/FasL |
|
Other |
9 : 1 female : male ratio Disease presents in absence of other rheumatic diseases |