Kikuchi-Fujimoto disease: a distinct pathological entity but also an "Overlap" autoimmune syndrome -a systematic review

Background: The association between KFD and autoimmune diseases, not only with systemic lupus erythematosus, has been repeatedly described. Objective: The aim of this review is to evaluate whether an overlap syndrome is present between KFD and autoimmune diseases. Methods: The databases used for the overlap case search were Medline and Embase from which we extrapolated the studies of interest. The search queries used were: Kikuchi-Fujimoto Syndrome and juvenile idiopathic arthritis or systemic lupus erythematosus or Systemic Sclerosis or Antiphospholipid Syndrome or Sjogren's Syndrome. Results: We have shown that there is an "overlap" syndrome between KFD and other autoimmune diseases. The chronology of disease onset was variable; autoimmune disease may be "preceding" or "simultaneous" or "post" Kikuchi-Fujimoto Syndrome. Also, the autoimmune disease can present with a complete clinical picture or only with the presence of autoantibodies. Conclusion: the different pathologies associated with KFD with different chronologies would indicate that there is an alteration of the immune system that allows the autoimmune phenomena to occur in different temporal relationships.