Kikuchi-Fujimoto disease, also known as subacute necrotizing lymphadenitis or subacute necrotizing histiocytosis, is an idiopathic disease characterized usually by cervical lymph node enlargement (80%).
It typically affects young women. Although it may affect any race, there is higher incidence in Asian populations 8.
It usually presents clinically with cervical lymphadenopathy that is sometimes tender. Accompanying systemic symptoms include low-grade fever, night sweats, malaise and joint or abdominal pains resembling a viral illness 3,4.
The cause is unknown and debated, with both viral infection and autoimmune mediated mechanisms proposed in the literature.
Typically (~80%) a single nodal group is involved, most frequently (70%) the posterior triangle. The nodes are soft and sometimes tender.
Imaging findings are non-specific and demonstrate primarily cervical nodal enlargement. The modality used depends on the suspected differential diagnoses at the time of presentation.
A review of CT neck findings in patients with Kikuchi disease (n=96) showed 1:
- homogenous nodal enlargement (83%)
- perinodal infiltration (80%)
- central nodal necrotic change (16%)
Treatment and prognosis
It usually runs a benign course that is self-limiting with most lymph nodes returning to normal size within the first six months of diagnosis.
History and etymology
The condition was first described in 1972 by Masahiro Kikuchi (1934-2012), a professor of histopathology and haematopathology, at Fukuoka University medical school 6,7. Ironically, in view of his specialism, his cause of death was lymphoma.
- kimura disease: painless lymphadenopathy
- 1. Kwon SY, Kim TK, Kim YS et-al. CT findings in Kikuchi disease: analysis of 96 cases. AJNR Am J Neuroradiol. 2004;25 (6): 1099-102. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 2. Martínez-vázquez C, Hughes G, Bordon J et-al. Histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto's disease, associated with systemic lupus erythemotosus. QJM. 1997;90 (8): 531-3. doi:10.1093/qjmed/90.8.531 - Pubmed citation
- 3. Haaga JR, Boll D. CT and MRI of the whole body. Mosby. (2009) ISBN:0323053750. Read it at Google Books - Find it at Amazon
- 4. Bosch X, Guilabert A. Kikuchi-Fujimoto disease. Orphanet J Rare Dis. 2007;1 (1): 18. doi:10.1186/1750-1172-1-18 - Free text at pubmed - Pubmed citation
- 5. Mahajan T, Merriman RC, Stone MJ. Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis): report of a case with other autoimmune manifestations. Proc (Bayl Univ Med Cent). 2011;20 (2): 149-51. Free text at pubmed - Pubmed citation
- 6. Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytosis. Nippon Ketsueki Gakkai Zasshi 1972;35:379–380.
- 7. Obituaries: Masahiro Kikuchi. BMJ 2013;347:f3524 doi: https://doi.org/10.1136/bmj.f3524 (Published 11 July 2013)
- 8. S. Lee, J.H. Yoo, S.W. Lee. Kikuchi Disease: Differentiation from Tuberculous Lymphadenitis Based on Patterns of Nodal Necrosis on CT. (2012) American Journal of Neuroradiology. 33 (1): 135. doi:10.3174/ajnr.A2724 - Pubmed