Abstract
Collagen diseases are autoimmune diseases which are prone to be associated with lymphoreticular disorders. Reports on such association with each collagen disease are outlined in this review. Hemophagocytic syndrome and B cell lymphoma seem to be most frequently associated with systemic lupus erythematosus and Sjögren's syndrome, respectively. Increase of non-Hodgkin's lymphoma has been shown in patients with rheumatoid arthritis, but not with systemic sclerosis or dermatomyositis/polymyositis. It appears most likely that chronic dysregulation of immune system in collagen diseases leads to proliferation of lymphoreticular system. It should be noted that lymphomas have been observed during immunosuppressive therapy for collagen diseases. More specific treatments for each collagen disease are expected to prevent abnormal proliferation of lymphoreticular system.