title: Kikuchi-Fujimoto disease: retrospective study of 91 cases and review of the literature.
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.
authors.affiliation: From the Department of Internal Medicine (GD, CR), Hôpital d'Instruction des Armées Bégin, Saint-Mandé; Department of Internal Medicine 2 (JH, ZA), Pitié-Salpêtrière University Hospital, Paris; Department of Internal Medicine (PC), Pitié-Salpêtrière University Hospital, Paris; Department of Clinical Immunology (LG), Saint-Louis University Hospital, Paris; Department of Rheumatology (OM), Bichat University Hospital, Paris; Department of Internal Medicine (CD), Martinique University Hospital, Fort-de-France; Department of Internal Medicine (BG), Mondor University Hospital, Créteil; Department of Internal Medicine (EA), Cochin University Hospital, Paris; Department of Internal Medicine (OL), Bicêtre University Hospital, Le Kremlin-Bicêtre; Department of Internal Medicine (TP), Bichat University Hospital, Paris; Hôpital Européen Georges Pompidou (JP), Paris; Department of Internal Medicine (MH), Hôtel-Dieu University Hospital, Nantes; Department of Internal Medicine (CB), Tenon University Hospital, Paris; Department of Internal Medicine (EH), Huriez University Hospital, Lille; Department of Internal Medicine (TC), Hôpital d'Instruction des Armées du Val de Grace, Paris; Department of Internal Medicine (RD), Avicenne University Hospital, Bobigny; Department of Internal Medicine (MG, AM, OF), Jean Verdier University Hospital, Bondy; Department of Pathology (FC), Pitié-Salpêtrière University Hospital, Paris; Department of Internal Medicine (DF), Saint-Louis University Hospital, Paris; Department of Pathology (TM), Necker University Hospital, Paris; France; and Department of Internal Medicine (VP), Hôpital des Trois-Chêne, Hôpitaux Universitaires de Genève, Genève; and Department of Internal Medicine (JS), Hôpitaux Universitaires de Genève, Genève, Switzerland. Drs. Prendki and Fain contributed equally.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Dumas
authors.forename: Virginie
authors.initials: V
authors.lastname: Prendki
authors.forename: Julien
authors.initials: J
authors.lastname: Haroche
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
authors.forename: Lionel
authors.initials: L
authors.lastname: Galicier
authors.forename: Olivier
authors.initials: O
authors.lastname: Meyer
authors.forename: Christophe
authors.initials: C
authors.lastname: Rapp
authors.forename: Christophe
authors.initials: C
authors.lastname: Deligny
authors.forename: Bertrand
authors.initials: B
authors.lastname: Godeau
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Aslangul
authors.forename: Olivier
authors.initials: O
authors.lastname: Lambotte
authors.forename: Thomas
authors.initials: T
authors.lastname: Papo
authors.forename: Jacques
authors.initials: J
authors.lastname: Pouchot
authors.forename: Mohamed
authors.initials: M
authors.lastname: Hamidou
authors.forename: Claude
authors.initials: C
authors.lastname: Bachmeyer
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Thierry
authors.initials: T
authors.lastname: Carmoi
authors.forename: Robin
authors.initials: R
authors.lastname: Dhote
authors.forename: Magdalena
authors.initials: M
authors.lastname: Gerin
authors.forename: Arsene
authors.initials: A
authors.lastname: Mekinian
authors.forename: Jérôme
authors.initials: J
authors.lastname: Stirnemann
authors.forename: Fréderic
authors.initials: F
authors.lastname: Charlotte
authors.forename: Dominique
authors.initials: D
authors.lastname: Farge
authors.forename: Thierry
authors.initials: T
authors.lastname: Molina
authors.forename: Olivier
authors.initials: O
authors.lastname: Fain
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D005260:Female; D005602:France / Q000453:epidemiology; D020042:Histiocytic Necrotizing Lymphadenitis / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones