Clinical and imaging findings of lymphoma in patients with Sjögren syndrome.

Abstract

To describe and correlate the clinical and imaging findings of lymphomas in patients with Sjögren syndrome. 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. 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. 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.