OBJECTIVE: To describe and correlate the clinical and imaging findings of 
lymphomas in patients with Sjögren syndrome.
METHODS: The authors reviewed the medical and imaging records of 27 cases of 
lymphoma from among a total of 463 patients with Sjögren syndrome. The estimated 
prevalence of lymphoma in patients with Sjögren syndrome was 5.8%. There were 22 
women and 5 men. Histopathologically, 26 of the 27 neoplasms were non-Hodgkin 
lymphoma, including 6 mucosa-associated lymphoid tissue lymphomas, and the other 
neoplasm was Hodgkin lymphoma. The clinical and imaging findings of lymphomas 
were analyzed.
RESULTS: No obvious correlations were present between the duration or severity 
of Sjögren syndrome and the lymphoma development. At the initial diagnosis, 
extranodal involvement was observed in 14 (52%) of the 27 patients, including 
the salivary gland (n = 9), lacrimal gland (n = 2), lung (n = 2), and thyroid 
gland (n = 1), mostly in the neck organs. On the other hand, nodal involvement 
was observed in 21 (78%) of the 27 patients. Of these 21 patients, 19 had at 
least cervical lymph node involvement.
CONCLUSION: Patients with Sjögren syndrome are at increased risk of lymphoma 
development. Because most lymphomas initially involve the neck organs, including 
the lymph nodes, meticulous imaging studies mainly focused on the cervical 
regions are recommended in the follow-up of patients with Sjögren syndrome.