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, whether there is a chronological and a casual relationship between the pathologies. 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. All study types were considered (n = 103). Results: Total number of included studies are 43. 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" (n = 11 cases) or "simultaneous" (n = 20 cases) or "post" (n = 8 cases). 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 suggest that there is an alteration of the immune system that allows the pathologies to occur in different temporal relationships.