Sjögren Syndrome and Cancer

Rheum Dis Clin North Am. 2020 Aug;46(3):513-532. doi: 10.1016/j.rdc.2020.05.004.


The association between malignancy and rheumatic diseases has been demonstrated in a multitude of studies. Little is understood regarding the pathogenesis of rheumatic and musculoskeletal diseases in association with malignancy. There is strong evidence regarding the association between Sjögren syndrome and lymphoma as well as risk factors for development of lymphoma in these patients. This article discusses the accumulating data on various malignancies described in primary Sjögren syndrome, highlighting non-Hodgkin lymphoma and thyroid, multiple myeloma, and skin cancers. These reported associations may have clinical implications in daily practice and contribute to understanding of both autoimmunity and cancer.

Keywords: Amyloidosis; Autoimmune disease; Lymphoma; Lymphoproliferative disorders; Malignancy; Salivary glands; Sjögren syndrome.

Publication types

  • Review