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The most serious complication associated with primary Sjögren syndrome is the development of a lymphoproliferative disease, primarily non-Hodgkin lymphoma. Multiple studies have shown an increase in the risk of lymphoproliferative disease, but no increase in all-cause mortality. 29 – 31 In contrast with primary Sjögren syndrome, other rheumatologic diseases, including lupus, rheumatoid arthritis, and scleroderma, have been associated with increased mortality rates. 29 The risk of lymphoma in patients with primary Sjögren syndrome is 40 times that of the general population. 1 A prospective study of 484 Swedish patients showed that lymphoproliferative diseases caused six of 34 deaths (18 percent) in the seven years of follow-up, with an average age of 75 years at the time of death. 29 However, this study did not show an increase in rates of all-cause mortality in persons with primary Sjögren syndrome compared with the general population. 29 A larger cohort study of 723 Greek patients with 4,384 person-years of follow-up found that seven of 39 deaths (18 percent) were caused by lymphoma. 31 A total of 30 cases of lymphoma were recorded, with an average follow-up of six years.