en
Scientific article
English

Kikuchi-Fujimoto disease: retrospective study of 91 cases and review of the literature

Published inMedicine, vol. 93, no. 24, p. 372-382
Publication date2014
Abstract

Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenopathy, most often cervical. It has been mainly described in Asia. There are few data available on this disease in Europe. We conducted this retrospective, observational, multicenter study to describe KFD in France and to determine the characteristics of severe forms of the disease and forms associated with systemic lupus erythematosus (SLE). We included 91 cases of KFD, diagnosed between January 1989 and January 2011 in 13 French hospital centers (median age, 30 ± 10.4 yr; 77% female). The ethnic origins of the patients were European (33%), Afro-Caribbean (32%), North African (15.4%), and Asian (13%). Eighteen patients had a history of systemic disease, including 11 with SLE. Lymph node involvement was cervical (90%), often in the context of polyadenopathy (52%), and it was associated with hepatomegaly and splenomegaly in 14.8% of cases. Deeper sites of involvement were noted in 18% of cases. Constitutional signs consisted mainly of fever (67%), asthenia (74.4%), and weight loss (51.2%). Other manifestations included skin rash (32.9%), arthromyalgia (34.1%), 2 cases of aseptic meningitis, and 3 cases of hemophagocytic lymphohistiocytosis. Biological signs included lymphocytopenia (63.8%) and increase of acute phase reactants (56.4%). Antinuclear antibodies (ANAs) and anti-DNA antibodies were present in 45.2% and 18% of the patients sampled, respectively. Concomitant viral infection was detected in 8 patients (8.8%). Systemic corticosteroids were prescribed in 32% of cases, hydroxychloroquine in 17.6%, and intravenous immunoglobulin in 3 patients. The disease course was always favorable. Recurrence was observed in 21% of cases. In the 33 patients with ANA at diagnosis, SLE was known in 11 patients, diagnosed concomitantly in 10 cases and in the year following diagnosis in 2 cases; 6 patients did not have SLE, and 4 patients were lost to follow-up (median follow-up, 19 mo; range, 3-39 mo). The presence of weight loss, arthralgia, skin lesions, and ANA was associated with the development of SLE (p < 0.05). Male sex and lymphopenia were associated with severe forms of KFD (p < 0.05). KFD can occur in all populations, irrespective of ethnic origin. Deep forms are common. An association with SLE should be investigated. A prospective study is required to determine the risk factors for the development of SLE.

Keywords
  • Adrenal Cortex Hormones/therapeutic use
  • Adult
  • Female
  • France/epidemiology
  • Histiocytic Necrotizing Lymphadenitis/diagnosis/drug therapy/epidemiology/pathology
  • Humans
  • Male
  • Retrospective Studies
  • Young Adult
Citation (ISO format)
DUMAS, Guillaume et al. Kikuchi-Fujimoto disease: retrospective study of 91 cases and review of the literature. In: Medicine, 2014, vol. 93, n° 24, p. 372–382. doi: 10.1097/MD.0000000000000220
Main files (1)
Article (Published version)
accessLevelRestricted
Identifiers
ISSN of the journal0025-7974
478views
0downloads

Technical informations

Creation11/07/2015 2:04:00 PM
First validation11/07/2015 2:04:00 PM
Update time03/14/2023 11:58:46 PM
Status update03/14/2023 11:58:45 PM
Last indexation01/16/2024 7:50:22 PM
All rights reserved by Archive ouverte UNIGE and the University of GenevaunigeBlack