Lymphoproliferative disease in Sjögren’s syndrome

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Scopus citations


Patients with Sjogren’s syndrome (SS) are at increased risk of non-Hodgkin B-cell lymphoma and multiple myeloma. Marginal zone lymphoma of mucosa-associated lymphoid tissue, usually affecting the major salivary glands, and diffuse large B-cell lymphoma are the most common subtypes. The development of lymphoma in SS is thought to arise from malignant transformation following sustained lymphoproliferation. Risk factors for the development of lymphoma reflect this pathogenetic model and include persistent salivary gland enlargement, high focus score, splenomegaly, lymphadenopathy, monoclonal proteins, and cryoglobulinemic vasculitis. In the majority of patients, lymphoma develops months to years after the diagnosis of SS. Most originate in the head and neck and thus present as one or more glandular masses and/or cervical lymphadenopathy. Diagnosis can often be achieved with either an excisional biopsy or an ultrasound-guided core needle biopsy with concomitant flow cytometry on material from cores and fine-needle aspiration. Treatment approaches are not only dictated by lymphoma subtype but also influenced by considerations specific to the underlying SS, including systemic disease activity. The prognosis of non-Hodgkin lymphoma in SS is generally favorable, with overall survival rates exceeding 90%.

Original languageEnglish (US)
Title of host publicationSjogren’s Syndrome
Subtitle of host publicationA Clinical Handbook
Number of pages24
ISBN (Electronic)9780323675345
ISBN (Print)9780323675352
StatePublished - Jan 1 2019


  • B cell
  • Cryoglobulinemia
  • Lymphoma
  • Multiple myeloma
  • Non-Hodgkin
  • Sjögren’s syndrome

ASJC Scopus subject areas

  • Medicine(all)


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