Sjogren's syndrome is an autoimmune disease with a known predisposition for 
lymphoma development. Eight of 120 patients with primary Sjogren's syndrome 
followed at the University of Ioannina over the past 7 years developed 
non-Hodgkin's lymphoma diagnosed according to the Kiel classification. The 
lymphomas differed by location and grading. Six were called low grade 
(immunocytoma) and two intermediate grade non-Hodgkin's lymphomas. Five of the 
immunocytomas involved the minor salivary or lacrimal glands. Immunoperoxidase 
staining for light chains revealed monoclonal populations. Two patients showed 
spontaneous regression not previously reported in Sjogren's syndrome. Thus, in 
Sjogren's syndrome, low grade non-Hodgkin's lymphomas and especially 
immunocytomas are the most common lymphomas. These lymphomas tend to evolve very 
slowly and may regress spontaneously. Given these facts, a conservative approach 
to treatment is indicated in those patients with only localized disease.