Familial Sjögren's syndrome with associated primary salivary gland lymphoma

Primary salivary gland lymphoma has been rarely documented in patients with or without Sjögren's syndrome. The association of disseminated lymphoreticular neoplasms with Sjögren's syndrome has been recognized, and the malignancy is usually widespread at the time of diagnosis. Familial occurrence of Sjögren's syndrome is likewise infrequently observed. In the present report we describe a patient with Sjögren's syndrome in whom a primary parotid gland lymphoma subsequently developed. In addition to the propositus, two of four siblings had definite evidence of Sjögren's syndrome and a third had several abnormal studies commonly associated with the disease. This observation suggests that genetic influence alone or in conjunction with other factors may facilitate the development of Sjögren's syndrome.