{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T06:04:22Z","timestamp":1791180262039,"version":"4.1.0"},"reference-count":40,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2015,9,23]],"date-time":"2015-09-23T00:00:00Z","timestamp":1442966400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Stavros Niarchos Foundation Research (grant to University of Athens, Department of Physiology)"},{"name":"Hellenic Rheumatology Association (grant to Dr. Mavragani)"},{"name":"GlaxoSmithKline (unrestricted educational grant to Dr. Voulgarelis)"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2015,10]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To study the prevalence, clinical associations, and functional implications of the His159Tyr mutation of the BAFF receptor (BAFF\u2010R) in patients with Sj\u00f6gren's syndrome (SS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The BAFF\u2010R His159Tyr mutation was evaluated using polymerase chain reaction (PCR)\u2013based assays in 247 patients with SS (of whom 70 had SS complicated by lymphoma [SS\u2010lymphoma]), 145 with systemic lupus erythematosus (SLE), and 101 with rheumatoid arthritis (RA), as well as 180 healthy controls. Real\u2010time PCR and Western blotting were performed for the quantification of both NF\u2010\u03baB1 and NF\u2010\u03baB2 messenger RNA (mRNA) transcript and protein levels in isolated B cells from patients with SS\u2010lymphoma carrying the mutation (SS\u2010lymphoma\u2013BAFF\u2010R<jats:sub>His159Tyr<\/jats:sub>\u2013derived B cells) compared to B cells from patients with SS\u2010lymphoma who were not carriers of the mutation and healthy controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Both the SS\u2010lymphoma and SS\u2010nonlymphoma patient subgroups exhibited significantly higher frequencies of the His159Tyr BAFF\u2010R mutation compared to healthy controls (8.6% of SS\u2010lymphoma patients and 6.2% of SS\u2010nonlymphoma patients versus 1.7% of healthy controls; <jats:italic>P<\/jats:italic>\u2009=\u20090.02 and <jats:italic>P<\/jats:italic>\u2009=\u20090.04, respectively). The corresponding frequencies of the His159Tyr BAFF\u2010R mutation in SLE and RA patients were 3.5% and 3%, respectively. Of interest, 71.4% of the SS patients with mucosa\u2010associated lymphoid tissue (MALT) lymphoma who were between the ages of 31 and 40 years at disease onset were mutation carriers. The generalized odds ratio for the development of SS\u2010related MALT lymphoma in the younger age at onset (age &lt;40 years) group in the presence of the BAFF\u2010R mutation was 6.1 (95% confidence interval 2.0\u201318.7) (<jats:italic>P<\/jats:italic> &lt; 0.01). Expression of NF\u2010\u03baB at both the mRNA and protein level was up\u2010regulated in SS\u2010lymphoma\u2013BAFF\u2010R<jats:sub>His159Tyr<\/jats:sub>\u2013derived B cells.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This study identifies an increased prevalence of the BAFF\u2010R His159Tyr mutation in patients with SS, particularly in those with SS complicated by MALT lymphoma whose disease onset occurred at a younger age. BAFF\u2010R<jats:sub>His159Tyr<\/jats:sub>\u2013mediated activation of the alternate NF\u2010\u03baB pathway might contribute to the pathogenesis of SS\u2010related lymphoproliferative disease.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.39231","type":"journal-article","created":{"date-parts":[[2015,6,20]],"date-time":"2015-06-20T04:07:48Z","timestamp":1434773268000},"page":"2732-2741","source":"Crossref","is-referenced-by-count":63,"title":["A BAFF Receptor His159Tyr Mutation in Sj\u00f6gren's Syndrome\u2013Related Lymphoproliferation"],"prefix":"10.1002","volume":"67","author":[{"given":"Aristea","family":"Papageorgiou","sequence":"first","affiliation":[{"name":"University of Athens Medical School Athens Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clio P.","family":"Mavragani","sequence":"additional","affiliation":[{"name":"University of Athens Medical School Athens 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