乾燥症是常見的自體免疫疾病，一般來說被認為是相當良性的病程，症狀緩慢進行。但是約5%-10% 的原發性乾燥症病患將發生淋巴癌，而造成患者較高的死亡 率。原發性乾燥症病患發生淋巴癌的危險因子包含唾液腺腫大，淋巴結腫大，雷諾氏症，自體抗體SSA 或SSB 陽性，類風濕因子陽性，單株免疫球蛋白升高，低第四 補體等。與原發性乾燥症相關的淋巴癌，通常進行的病程緩慢，腫瘤體積小，沒有發燒、體重減輕等腫瘤伴隨症狀。原發性乾燥症相關的淋巴癌生成，病理機轉是抗 原驅動造成。所以，以免疫調節劑控制乾燥症的疾病活性，來壓抑長期Ｂ細胞的過度刺激，或將是預防淋巴癌產生的方法。

Primary Sjögren’s syndrome has been generally acknowledged as a chronic benign autoimmune disease. Patients with Sjögren’s syndrome have an increased risk of non-Hodgkin lymphoma compared with the general population, and this increased risk is evident for both primary and secondary forms of Sjögren’s syndrome. Persistent salivary gland enlargement, lymphadenopathy, Raynaud phenomenon, presence of antibodies to SS-A or SS-B, rheumatoid factor, and low serum complement C4 are risk factors of developing lymphoma. Non-Hodgkin lymphomas complicating Sjögren’s syndrome are characterized by an indolent course, small tumour burden, and an excellent performance status. Lymphomagenesis exemplifies the development of antigen-driven B-cell lymphoma and the control of disease activity is the primary objective of the management of the patient in order to repress chronic B cell stimulation.