ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2019

Kikuchi-Fujimoto disease : Features and Outcome Of 91 Patients In France

Guillaume Dumas1, Virginie Prendki2, Julien Haroche3, Patrice Cacoub4, Zahir Amoura3, Lionel Galicier5, Olivier Meyer6, Christophe Rapp7, Christophe Deligny8, Bertrand Godeau9, Elisabeth Aslangul10, Olivier Lambotte11, Thomas Papo12, Jacques Pouchot13, Mohamed Hamidou14, Gilles Grateau15, Eric Hachulla16, Thierry Carmoi17, Robin Dhote18, Magdalena Gerin19, Arsene Mekinian20, Fréderique Charlotte21, Dominique Farge5, Thierry Molina22 and Olivier Fain23, 1internal medicine, Hôpital d'Instruction des Armées Bégin, Saint Mandé, France, 2internal medicine, Geriatry, Hôpitaux Universitaires de Genève, Genève, Switzerland, 3Internal Medicine, Hôpital Pitié-Salpêtrière, AP-HP, UPMC Univ Paris 06 & French National Reference Center For Systemic Lupus and Antiphospholipid Syndrome, Paris, France, 4Groupe Hospitalier Pitié Salpétrière, Service de Médecine Interne, DHU i2B, Paris, France, 5Internal Medicine, Hopital St Louis, AP-HP, Paris, France, 6Rheumatology, Bichat University Hospital, Paris, France, 7intenal medicine, Hôpital d'Instruction des Armées Bégin, Saint Mandé, France, 8Rhumatologie Et Médecine Interne, Centre hospitalier Universitaire de Fort de France, Fort de France, Martinique, 9Internal Medicine, University of Paris, AP-HP, Hôpital Mondor Créteil, Creteil, France, 10internal medicine, hôpital Hotel Dieu, AP-HP, Paris, France, 11Internal Medicine, Hopital Kremlin Bicêtre, Kremlin Bicêtre, France, 12Internal Medicine, Bichat Hospital, Paris, Paris, France, 13internal medicine, Hôpital Européen Georges Pompidou, AP-HP, Paris, France, 14CHU Hôtel Dieu, Nantes, Nantes, France, 15Service De Médecine Interne, Hopital Tenon, Paris, France, 16Internal Medicine, University Hospital Lilles, Lille CEDEX, France, 17internal medicine, Hôpital d'Instrcution des Armées du Val de Grace, Paris, France, 18Internal Medicine, Avicenne University Hospital, Bobigny, France, 19Internal Medicine, Hôpital Jean Verdier, AP-HP, Bondy, France, 20internal medicine, Hôpital Jean Verdier, AP-HP, Bondy, FL, France, 21Department of Anatomic Pathology, Hopital Pitié-Salpétrière, Paris, France, 22Department of Anatomic Pathology, hôpital Hotel Dieu, AP-HP, Paris, France, 23Hôpital Saint Antoine, DHU i2B, Service de Médecine Interne, paris, France

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords:

Session Information

Title: Miscellaneous Rheumatic and Inflammatory Diseases II: Miscellaneous Rheumatic Diseases

Session Type: Abstract Submissions (ACR)

Background/Purpose:

Kikuchi Fujimoto disease (KFD) constitutes a rare cause of cervical lymphadenitis, usually reported in Asia. Data about this disease are scarce in Europe. The primary endpoint of this study was to describe KFD epidemiology in France. Secondary endpoints were to analyze more specifically the most severe expression of this disease, and forms associated with systemic lupus erythematosus (SLE).

Methods:

Retrospective observational study of KFD cases diagnosed in thirteen hospitals in France, between January 1989 and January 2011.

Results:

91 patients were included. 70 (77%) were female with a mean (+/- SD) age of 30+/-10.4 years. 33% of patients came from Europe, 32% from Africa or the Caribbean, 15.4% from Maghreb and 13% from Asia. Eighteen patients presented a history of systemic disease, eleven of them having systemic lupus erythematosus. Cervical lymphadenitis was present in 90% of the cases, and generalized lymphadenomegaly in 52%.  Hepatomegaly and splenomegaly were found in 14.8% of the patients, and deep-seated lymphadenitis in 18% of them. Adenitis was associated with fever in 67%, asthenia in 74.4%, weight-loss in 51.2% of the total number of patients. Regarding extra-nodal manifestations we can list cutaneous involvement (32.9%), arthralgia and myalgia (34.1%), and more rarely aseptic meningitis (n=2) or hemophagocytic lymphohistiocytosis (n=3). Laboratory findings brought up lymphopenia for 63.8% of the patients and inflammatory syndrome for 56.4% of them. Anti-nuclear (ANA) and anti ds-DNA antibodies were detected in respectively 45.2% and 18% of tested patients. A viral infection was diagnosed for 8.8% of the studied cases. Corticosteroid treatment was used in the treatment of 32% of the patients, hydroxychloroquine in 17.6% and intravenous immunoglobulins in a total of 3 patients. Even though good prognosis was observed, relapse occured for 21% of the patients. Considering the 33 patients presenting with ANA, systemic lupus erythematosus was diagnosed concomitantly in 10 cases, and within the next year in 2 cases. 6 patients did not develop SLE and 6 patients were lost to follow up (median (Q1-Q3) follow up of 19 [3-39] month).

Weight loss, arthralgia, cutaneous involvement and ANA were associated with SLE (p<0.05). Male sex and lymphopenia were factors associated with severity of the disease (p<0.05).

Conclusion:

KFD not exclusively occurs in Asian population. Associated SLE must be investigated. A prospective study is warranted to determine risk factors for SLE in patients with KFD.

Disclosure:

G. Dumas,
None;

V. Prendki,
None;

J. Haroche,
None;

P. Cacoub,
None;

Z. Amoura,
None;

L. Galicier,
None;

O. Meyer,
None;

C. Rapp,
None;

C. Deligny,
None;

B. Godeau,
None;

E. Aslangul,
None;

O. Lambotte,
None;

T. Papo,
None;

J. Pouchot,
None;

M. Hamidou,
None;

G. Grateau,
None;

E. Hachulla,
None;

T. Carmoi,
None;

R. Dhote,
None;

M. Gerin,
None;

A. Mekinian,
None;

F. Charlotte,
None;

D. Farge,
None;

T. Molina,
None;

O. Fain,
None.

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