Kikuchi-Fujimoto Disease: A Distinct Pathological Entity but also an "Overlap" Autoimmune Syndrome: A Systematic Review

Abstract

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 indicate suggest that there is an alteration of the immune system that allows the autoimmune phenomena pathologies to occur in different temporal relationships.

Increased Expression of CD169 on Monocytes in Adult-Onset Kikuchi–Fujimoto Disease

“…Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis without granulocytic infiltration, was first described in young Japanese females in 1972 [1,2] and is a rare, benign lymphoproliferative disorder with a definite histological pattern that is associated with a number of clinical scenarios of an inflammatory nature [3,4]. It is clinically characterized by self-limiting systemic symptoms (fever, weight loss, etc.)…”

“…associated with localized tender lymphadenopathies, mostly of the cervical region. Nonetheless, extranodal involvement and complicated courses, in particular the subsequent or concomitant development of systemic inflammatory autoimmune diseases (SIADs) and/or hemophagocytic lymphohistiocytosis (HLH), have been recognized [4,5].…”