Diffuse infiltrative lymphocytosis syndrome in human immunodeficiency virus infection--a Sjögren's-like disease

Certain adults and children with human immunodeficiency virus (HIV) infection develop a syndrome characterized by marked parotid gland enlargement, sicca symptoms and pulmonary insufficiency. Lymphocytic tissue infiltration is accompanied by circulating CD8 lymphocytosis, and autoantibodies are infrequently observed. Progressive lymphocytic visceral involvement may necessitate the careful use of immunosuppressive therapy, in addition to antiretroviral agents. Specific associations with particular MHC class I and II gene products suggest that this disorder may represent a distinctive host immune response to HIV. Infection with HIV should be considered in all high-risk individuals presenting with the sicca syndrome. Similarly, it should be considered in individuals in whom Sjögren's disease is a diagnostic possibility but where atypical features are present.