Mucosa-associated lymphoid tissue lymphoma in Sjögren's syndrome: risks, management, and prognosis

Rheum Dis Clin North Am. 2008 Nov;34(4):921-33, viii. doi: 10.1016/j.rdc.2008.08.006.


Sjögren's syndrome is a chronic inflammatory disease primarily affecting the exocrine glands. Its association with lymphoma is well documented, with salivary extranodal marginal zone lymphomas of the mucosa-associated lymphoid tissue type being the most common and constituting a major disease complication. These neoplasms are antigen-stimulated B-cell lymphomas characterized by localized stage, indolent clinical course, and recurrence in other extranodal sites. This article presents a review of the literature and discusses the clinical, histopathologic, therapeutic, and prognostic aspects of these tumors in Sjögren's syndrome. In addition, it highlights the predictor markers of lymphoma development.

Publication types

  • Review

MeSH terms

  • Autoimmunity
  • Humans
  • Lymphoma, B-Cell, Marginal Zone / etiology*
  • Lymphoma, B-Cell, Marginal Zone / pathology
  • Lymphoma, B-Cell, Marginal Zone / therapy
  • Meta-Analysis as Topic
  • Prognosis
  • Risk Factors
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / pathology
  • Sjogren's Syndrome / therapy