Title:Kikuchi-Fujimoto Disease: A Distinct Pathological Entity but also an
"Overlap" Autoimmune Syndrome: A Systematic Review
Volume: 19
Issue: 2
Author(s): Fabiana Zaccarelli, Marco de Vincentiis, Giovanni D’Erme, Antonio Greco, Francesco Natalucci and Massimo Fusconi*
Affiliation:
- Department of Sense Organs, Sapienza University, Rome, Italy
Keywords:
Kikuchi-fujimoto disease, juvenile idiopathic arthritis, systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, sjogren's syndrome, histiocytes.
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 suggest
that there is an alteration of the immune system that allows the pathologies to occur in different
temporal relationships.
