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Example sentences for: sjögren
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The co-existence of liver disease and the presence of circulating AMAs in Sjögren's patients sera has been pointed as an indicator that liver pathology might be autoimmune and similar to that of primary biliary cirrhosis [ 4 7 8 10 ] . In previous studies, a pericholangial lymphocytic infiltration similar to that found in stage I of primary biliary cirrhosis, has been reported in 1°SS with abnormal liver function tests or positivity for AMA [ 4 7 ] . In our review of cases, we found predominance in hepatocellular liver damage rather than cholestatic disease, which would argue against a PBC-type of liver damage.
Furthermore, in many cases, Sjögren's syndrome diagnosis had been sought because of otherwise unexplained non-exocrine features.
Autoantibodies directed against the Golgi complex were first identified in the serum of a Sjögren's syndrome patient with lymphoma [ 1 ] . Subsequent reports described anti-Golgi complex autoantibodies (AGA) in patients with systemic rheumatic diseases such as systemic lupus erythematosus [ 2 ] , rheumatoid arthritis [ 3 ] , and Wegener's granulomatosis [ 4 ] . Immunoblotting and immunoprecipitation studies have shown that there was heterogeneity of reactivity among AGA [ 5 ] .
Casciola-Rosen and coworkers have proposed that modifications of autoantigens during cell death, particularly proteolytic cleavage, might be crucial for the generation of autoantibodies in autoimmune diseases [ 23 37 ] . Unique proteolytic fragments of α-fodrin and type 3 muscarinic acetylcholine receptor have been implicated in the generation of autoantibody responses in primary Sjögren's syndrome [ 38 39 ] .
The DQA1*0501 allele is strongly associated with early-onset pauciarticular JRA in Northern European populations [ 23 24 ] and with increased risk for juvenile dermatomyositis [ 25 ] and Sjögren's syndrome with high autoantibody production [ 26 27 ] . In our proposed model, aberrant class II MHC regulation could result from altered DEK binding and/or interaction with NF-Y, and aberrant class II expression may alter or enhance reactivity against DEK-derived or other self peptides.
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