Sjogren's syndromeRisk, Predictors, and Clinical Characteristics of Lymphoma Development in Primary Sjögren's Syndrome
Section snippets
Patients and Methods
All patients consecutively diagnosed with pSS and followed up consecutively at our department between January 1988 and December 2008 were eligible for the study. Patients were followed at regular intervals of 6 to 12 months or more frequently if required. All patients fulfilled 4 or more of the preliminary diagnostic criteria for pSS proposed in 1993 by the European Community Study Group (13). A complete history and physical examination was obtained in all cases. Diagnostic tests for
Results
The study cohort consisted of 244 patients (235 females and 9 males), with a mean age of 57.8 ± 13.6 years (median 59 year; range 17-88 years) at pSS diagnosis. All patients fulfilled the diagnostic criteria for pSS proposed by the European Community Study Group (13), and 201 (82.38%) fulfilled the American-European Consensus criteria (18). The median follow-up was 8.6 years (1 to 20 year).
Eleven patients developed a NHL: 4 of MALT type, 3 DLBCL, 3 follicular lymphoma, and 1 both MALT and
Discussion
The present series is 1 of the largest single-center experiences on patients with pSS-associated lymphoproliferative disease. In accordance with previous reports (5, 7, 8, 9) we confirmed the preponderance of B-cell malignancies in pSS patients. Eleven of 244 (4.5%) patients developed a NHL in our series, similar to others that estimated this probability about 5 to 8% (5, 7, 8, 9, 11). The relative risk of developing lymphoma was estimated to be 16 times higher than the general population,
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The authors have no conflicts of interest to disclose.