Abstract
Kikuchi–Fujimoto Disease (KFD) was first described in Japan in 1972. The disease frequently mimics tuberculous lymphadenitis, malign lymphoma, and many other benign and malignant conditions. To our knowledge, there is no previous study comparing the clinical and laboratory characteristics of patients from different geographical parts of the world. We searched literature records beginning from 1991 and analyzed epidemiological, clinical, and laboratory data of 244 patients (including cases diagnosed in our institution) reported in 181 publications. Of the 244 cases, 33% were male and 77% were female. Mean age was 25 (1–64) and 70% was younger than 30. Most of the cases were reported from Taiwan (36%), USA (6.6%), and Spain (6.3%). Fever (35%), fatigue (7%) and joint pain (7%) were the most frequent symptoms, while lymphadenomegaly (100%), erythematous rashes (10%), arthritis (5%), hepatosplenomegaly (3%), leucopenia (43%), high erythrocyte sedimentation rate (40%), and anemia (23%) being the most common findings. KFD was associated with SLE (32 cases), non-infectious inflammatory diseases (24 cases), and viral infections (17 cases). SLE was more frequent in cases from Asia than Europe (28 and 9%, respectively). The disease was self-limiting in 156 (64%) and corticosteroid treatment was necessary in 16 (16%) of the cases. The mortality rate was 2.1%. Early diagnosis is crucial as the clinical and laboratory presentation generally imitates situations needing lengthy and costly diagnostic and therapeutic interventions. Additionally, association with SLE needs further investigation.
Similar content being viewed by others
References
Kapadia V, Robinson BA, Angus HB (1989) Kikuchi’s disease presenting as fever of unknown origin. Lancet 2:986
Chen JS, Chang KC, Cheng CN et al (2000) Childhood hemophagocytic syndrome associated with Kikuchi’s disease. Haematologica 85:998–1000
Yu HL, Lee SJ, Tsai HC et al (2005) Clinical manifestations of Kikuchi’s disease in Southern Taiwan. J Microbiol Immunol Infect 38:35–40
Lee KY, Yeon YH, Lee BC (2004) Kikuchi–Fujimoto Disease with prolonged fever in children. Pediatrics 114:752–756
Wang TJ, Yang YH, Lin YT, Chiang BL (2004) Kikuchi–Fujimoto disease in children: clinical features and disease course. J Microbiol Immunol Infect 37:219–224
Hsueh EJ, Ko WS, Hwang WS, Yam LT (1993) Fine-needle aspiration of histiocytic necrotizing lymphadenitis (Kikuchi’s Disease). Diagn Cytopathol 9:448–452
Kucukardali Y, Oncul O, Baloglu H (2002) Kikuchi disease: case report. Internet J Emerg Intensive Care Med 6(2) (http://www.ispub.com)
Dorfman RF, Berry GJ (1988) Kikuchi’s histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol 5:329–345
Sumiyoshi Y, Kikuchi M, Minematu T (1994) Analysis of herpesvirus genomes in Kikuchi’s disease. Virchows Arch 424:437–440
Pileri SA, Sabattini E, Costigliola P (1991) Kikuchi’s lymphadenitis and HIV infectious. AIDS 5:459–461
Kikuchi M, Yoshizumi T, Nakamura H (1977) Necrotizing lymphadenitis: possible acute toxoplasmic infection. Virchows Arch 376:247–253
Dominguez DC, Torres ML, Antony S (2003) Is human herpesvirus 6 linked to Kikuchi–Fujimoto disease? The importance of consistent molecular and serologic analysis. South Med J 96:226–233
George TI, Jones CD, Zehnder JL, Warnke RA, Dorfman RF (2003) Lack of human herpesvirus 8 and Epstein–Barr virus in Kikuchi’s histiocytic necrotizing lymphadenitis. Hum Pathol 34:130–135
Seno A, Torigeo R, Shimoe K (1994) Kikuchi’s disease (histiocytic necrotizing, lymphadenitis) with cutaneuos involvement. J Am Acad Dermatol 30:504–506
Letawe C, Pierard-Franchimont C, Rustom KA, Hermanns-Le T, Pierard GE (1999) Cutaneous lymph node Kikuchi –Fujimoto disease. Ann Dermatol Venereol 126:26–28
Quintas-Cardama A, Fraga M, Cozzi SN, Caparrini A, Maceiras F, Forteza J (2003) Fatal Kikuchi–Fujimoto disease: the lupus connection. Ann Hematol 82:186–188
Wong CY, Law GT, Shum TT, Wong KY, Li YK (2001) Pulmonary haemorrhage in a patient with Kikuchi disease. Monaldi Arch Chest Dis 56:118–120
Chmait RH, Meimin DL, Koo CH, Huffaker J (2000) Hemophagocytic syndrome in pregnancy. Obstet Gynecol 95:1022–1024
Wilkinson CE, Nichol F (2000) Kikuchi–Fujimoto disease associated with polymyositis. Rheumatology 39:1302–1304
Tsai MK, Huang HF, Hu RH et al (1998) Fatal Kikuchi–Fujimoto disease in transplant recipients: a case report. Transplant Proc 30:3137–3138
Kuo TT (1995) Kikuchi’s disease (histocytic necrotizing lymphadenitis): a clinicopathologic study of 79 cases with an analysis of histologic subtypes, immunohistology, and DNA ploidy. Am J Surg Pathol 19:798–809
Emir S, Gogus S, Güler E et al (2001) Kikuchi–Fujimoto disease (histiocytic necrotizing lymphadenitis) confused with lymphoma in a child. Med Pediatr Oncol 37:546–548
Nambiar S, Chandra RS, Schwartz RH et al (2001) Seven-year-old Indian girl with fever and cervical lymphadenitis. Kikuchi–Fujimoto disease. Pediatr Infect Dis J 20:464–465
Stephan JL, Jeannoel P, Chanoz J et al (2001) Epstein–Barr virus-associated Kikuchi disease in two children. J Pediatr Hematol Oncol 23:240–243
Ioachım HL, Ratech H (2002) Kikuchi–Fujimoto lymphadenopathy. In: Ioachım HL, Ratech H (eds) Ioachım’s lymph node pathology, 3rd edn, Lippincott Williams, Philadelphia, USA, pp 219–221
Rivano MT, Falini B, Stein H et al (1987) Histiocytic necrotizing lymphadenitis without granulocytic infiltration (Kikuchi’s lymphadenitis). Morphological and immunohistochemical study of eight cases. Histopathology 11:1013–1027
Pileri SA, Facchetti F, Ascani S et al (2001) Myeloperoxidase expression by histiocytes in Kikuchi’s and Kikuchi-like lymphadenopathy. Am J Pathol 159:915–924
Esdaile JM (1998) Adult still’s diseases. In: Klippel HJ, Dieppe PA (eds) Rheumatology. Mosby Year Book, 2nd edn. Europe Limited, pp 5:22.1–7
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kucukardali, Y., Solmazgul, E., Kunter, E. et al. Kikuchi–Fujimoto Disease: analysis of 244 cases. Clin Rheumatol 26, 50–54 (2007). https://doi.org/10.1007/s10067-006-0230-5
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10067-006-0230-5