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Sjögren's Syndrome
Sjögren’s Syndrome (SjS)
SjS is a chronic inflammatory disease of unknown origin and one of the more prevalent autoimmune disorders. It is characterised by lymphocytic infiltration into exocrine glands, which causes diminished lacrimal and salivary gland secretion due to loss of proper function.
Most common symptoms are keratoconjunctivitis sicca and xerostomia. Extraglandular symptoms can occur usually in the skin and joints with 2.5% of patients developing non- Hodgkins lymphoma. Around 90% of patients affected by SjS are female.
There are two types of SjS, primary SjS and secondary SjS. Secondary SjS occurs in association with rheumatoid arthritis and other connective tissue disorders. ANA screens are positive in around 70% of patients with speckled fluorescence a key diagnostic indicator.
Ro/SSA and La/SSB antibodies are included in the classification criteria for SjS and are detectable in 60-75% and 30-50% of patients respectively. La/SSB antibodies in the absence of SLE-specific autoantibodies are considered to be specific for SjS. Other autoantibodies that react with the M3 muscarinic acetylcholine receptor (M3R), are described as highly sensitive and specific for SjS.
SjS is a chronic inflammatory disease of unknown origin and one of the more prevalent autoimmune disorders. It is characterised by lymphocytic infiltration into exocrine glands, which causes diminished lacrimal and salivary gland secretion due to loss of proper function.
Most common symptoms are keratoconjunctivitis sicca and xerostomia. Extraglandular symptoms can occur usually in the skin and joints with 2.5% of patients developing non- Hodgkins lymphoma. Around 90% of patients affected by SjS are female.
There are two types of SjS, primary SjS and secondary SjS. Secondary SjS occurs in association with rheumatoid arthritis and other connective tissue disorders. ANA screens are positive in around 70% of patients with speckled fluorescence a key diagnostic indicator.
Ro/SSA and La/SSB antibodies are included in the classification criteria for SjS and are detectable in 60-75% and 30-50% of patients respectively. La/SSB antibodies in the absence of SLE-specific autoantibodies are considered to be specific for SjS. Other autoantibodies that react with the M3 muscarinic acetylcholine receptor (M3R), are described as highly sensitive and specific for SjS.
References:
Zandbelt et al, Arthiritis Res Ther 2004 6:R33-R38Bacman et al, Clin Exp Immunol 1996 104:454-459
Youinou et al, Clin Rev Allergy 1994 12(2):253-74
Garci-Carrasco et al, Medicine(Baltimore) 2002 81:270-280
Vitali et al, Ann Rheum Dis 2002 61:554-8
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