To evaluate the incidence and spectrum of malignancy of primary Sjögren's syndrome (pSS).
250 pSS who were followed-up in Peking Union Medical College (PUMC) Hospital were analyzed.
Four of them were diagnosed with histopathological findings of 2 non-Hodgkin Lymphoma, 1 AILD, 1 multiple myeloma. Two died of secondary infection while receiving chemotherapy for lymphoma, 2 remained remitted.
The risk factors were persistent enlargement of major salivary glands, appearance of monoclonal serum lg, and disappearance of auto antibodies.