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Lymphoma and other malignancies in primary sjogren's syndrome A cohort study on cancer incidence and lymphoma predictors.

In English

Författare

Summary, in English

Objectives: To assess the risk of lymphoproliferative disease or other malignancy (standardised incidence ratios (SIRs)), in patients with primary Sjogren's syndrome according to the American-European Consensus Criteria (AECC), compared with patients with sicca syndrome (non-AECC) and the background population. To identify predictors of malignancy and describe lymphoma types and survival probabilities. Methods: A linked register study using information from the Malmo "Primary SS Register, Swedish Cancer Register, and Cause-of-Death Register for calculation of SIRs was carried out. Detected lymphomas were reclassified according to the WHO classification. Cox regression analysis was used to study the predictive value of clinical, laboratory, and histological findings at the time of diagnosis. Results: 507 patients with a median follow up of 8 years (range 1 month to 19 years) were included. SIRs (95% confidence interval (CI)) for malignancies in total and for non-Hodgkin's lymphomas (NHL) were 1.42 (0.98 to 2.00) and 15.57 (7.77 to 27.85), respectively, in those fulfilling the AECC (n = 286). In non-AECC sicca patients (n = 221) SIR for malignancy of any kind was 0.77 (0.41 to 1.32); no lymphoproliferative neoplasms were detected. Significant predictors of lymphoproliferative disease were purpura/skin vasculitis (hazard ratio (HR) = 4.64, 95% CI 1.13 to 16.45), low complement factor C3 (HR = 6.18, 95% CI 1.57 to 24.22), low C4 (HR = 9.49, 95% CI 1.94 to 46.54), CD4+ T lymphocytopenia (HR = 8.14, 95% CI 2.10 to 31.53), and a low CD4+/CD8+ T cell ratio <= 0.8 (HR = 10.92, 95% CI 2.80 to 41.83). 7/12 (58%) NHLs were diffuse large B cell lymphomas. Conclusion: A 16-fold increased risk for development of NHL was found. CD4+ T lymphocytopenia is an additional strong risk factor for developing lymphoma.

Publiceringsår

2006

Språk

Engelska

Sidor

796-803

Publikation/Tidskrift/Serie

Annals of the Rheumatic Diseases

Volym

65

Issue

Nov 10

Dokumenttyp

Artikel i tidskrift

Förlag

BMJ Publishing Group

Ämne

  • Rheumatology and Autoimmunity

Status

Published

Forskningsgrupp

  • Clinical Microbiology, Malmö
  • Pathology, Malmö
  • Internal Medicine - Epidemiology

ISBN/ISSN/Övrigt

  • ISSN: 1468-2060