Epidemiology of Kikuchi-Fujimoto Disease in Martinique and Characteristics in an Afro-Caribbean Population: Close Relation with Lupus

Florence MOINET¹, Vincent MOLINIE², Katlyne Polomat¹, Nadege Cordel³, Guillaume BERAUD⁴, Dominique SAINTE MARIE⁵, Olivier DUFFAS⁶, Charlène BOMAHOU⁷, Serge ARFI¹, Jean-come MENIANE⁸, Suzy DUFLO⁹, Yves HATCHUEL¹⁰ and Christophe Deligny¹, ¹Rheumatology and Internal Medicine, Zobda Quitman Hospital, Fort de France, Martinique, ²pathology, Zobda Quitman Hospital, Fort de France, Martinique, ³Infectious diseases, dermatology and internal medicine, Centre Hospitalier universitaire de Guadeloupe, Pointe à Pitre, Guadeloupe, ⁴Internal medicine and infectious disease, CHU de Poitiers, Poitiers, France, ⁵Dermatologie, Centre Hospitalier André Rosemon, Cayenne, French Guiana, ⁶ENT, Zobda Quitman Hospital, Fort de France, Martinique, ⁷Internal medicine, Hopital Européen Georges Pompidou, Paris, France, ⁸Hematology, Zobda Quitman Hospital, Fort de France, Martinique, ⁹ENT, Centre Hospitalier universitaire de Guadeloupe, Pointe à Pitre, Guadeloupe, ¹⁰Pediatry, Zobda Quitman Hospital, Fort de France, Martinique

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: African-Americans and systemic lupus erythematosus (SLE)

Session Information

Date: Monday, November 9, 2015

Title: Epidemiology and Public Health Poster II: Pathogenesis and Treatment of Systemic Inflammatory Diseases

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose:

Kikuchi-Fujimoto disease (KFD) is a rare histiocytic necrotizing lymphadenitis affecting mainly Asian young women, associated to systemic lupus (SLE) in less than 25% of the cases. There is no epidemiology and no series devoted to African descent patients. Our objectives were to study (1) the incidence in the population of Martinique, (2) the characteristics of KFD afro-Caribbean patients from the 3 French American dependencies.

Methods:

A retrospective study including all patients with KFD compatible pathology report during a first episode or a recurrence in Martinique between 1991 and 2013 by three sources: (1) database of the two pathology units in the island (one public, one private), (2) the healthcare coding system of the academic hospital, (3) the departments of internal medicine, rheumatology, hematology and otorhinolaryngology files. In a second time, for a more comprehensive description of the disease’s characteristics, we explored by the same way two other overseas French departments (Guadeloupe, French Guiana) and also included more afro-Caribbean patients in the cohort. Inclusion criteria were a biopsy with compatible pathology report for the epidemiology in Martinique, adding for the whole series description, an auto-declared afro-Caribbean ethnicity. SLE was defined by the presence of 1997 ACR criteria.

Results: Thirty-six cases of KFD were included, all afro-Caribbean: 26 from Martinique, 8 from Guadeloupe, 2 from French Guiana. The mean age at KFD diagnosis was 30.5 years (range: 5-59) and the female/male sex ratio was 3/1 (27 females, 9 males). Initial features were: fever (25/30; 83.3 %), asthenia (19/26; 73 %), weight loss (16/25; 64.4 %). Lymph nodes were cervical in 88.8 % (32/36), inguinal in 16.1% (5/31), only axillary in 8.3%, only abdominal in 2.8 %. Lupus was underlying in 4/36 patients (11.1%), diagnosed concomitantly with KFD in 6 (16.7%) and after KFD in 1 (2.8%). Recurrence was noted in 9 (25 %) of the patients but 15 KFD (14 non SLE related and 1 SLE related) were lost to follow up. At the time of KFD diagnosis, ANA were available for 22 patients, and 12 were ≥ 1/320 (54.5 %). The evolution was a spontaneous regression in 16/29 (55.1%) and 12/28 (42.8 %) of the patients received oral steroids. 11/30 KFD cases (36.6 %) were associated with lupus: 9 SLE (30%) and 2 pure cutaneous lupus (6.6%). Twenty four cases were diagnosed in Martinique between 1991 and 2013 (women: n=16, men: n=8): the crude average annual incidence was 2.77 cases for 10⁶inhabitants (95%CI: 1.73-3.93), 3.5 for women (95%CI: 1.97-5.25) and 1.96 for men (95%CI: 0.74-3.43). Other autoimmune diseases were associated to KFD: primary Sjögren syndrome (n=1) and juvenile idiopathic arthritis (n=1, her mother had SLE).

Conclusion:

We report here the first KFD epidemiology. This study confirms: (1) the presence of KFD in the afro-Caribbean population, to be confirmed in other African heritage populations, (2) the strong association with autoimmune diseases (mainly lupus) in our black population. But, KFD epidemiology is difficult to carry out because of the usual transient nature of this disease, leading to no lymph node biopsy and frequent lost to follow up after diagnosis.

Disclosure: F. MOINET, None; V. MOLINIE, None; K. Polomat, None; N. Cordel, None; G. BERAUD, None; D. SAINTE MARIE, None; O. DUFFAS, None; C. BOMAHOU, None; S. ARFI, None; J. C. MENIANE, None; S. DUFLO, None; Y. HATCHUEL, None; C. Deligny, None.

To cite this abstract in AMA style:

MOINET F, MOLINIE V, Polomat K, Cordel N, BERAUD G, SAINTE MARIE D, DUFFAS O, BOMAHOU C, ARFI S, MENIANE JC, DUFLO S, HATCHUEL Y, Deligny C. Epidemiology of Kikuchi-Fujimoto Disease in Martinique and Characteristics in an Afro-Caribbean Population: Close Relation with Lupus [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/epidemiology-of-kikuchi-fujimoto-disease-in-martinique-and-characteristics-in-an-afro-caribbean-population-close-relation-with-lupus/. Accessed .

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