Abstract

KIKUCHI – FUJIMOTO DISEASE: A NARRATIVE REVIEW OF PATHOGENESIS, DIAGNOSIS AND MANAGEMENT WITH A FOCUS ON PHARMACOTHERAPY

Vani Devi S.*, Aiswarya Rajesh, Sofy Binu

ABSTRACT

Kikuchi-Fujimoto disease (KFD), which is also known as histiocytic necrotizing lymphadenitis, is a rare self-limiting inflammatory disease characterized by tender cervical lymphadenopathy and fever in young adults, especially people of Asian descent.[1,2] This rare disease was first described by Kikuchi, and separately by Fujimoto et al. in 1972, hence it has two alternative names.[1,7] The challenge in diagnosing KFD lies in the similarity of this disease to infectious lymphadenitis, lymphoma, but mostly to systemic lupus erythematosus (SLE).[1,7] This paper provides the history of the discovery of KFD and describes the current information about KFD in terms of epidemiology, the mechanism of immunopathogenesis, proposed molecular signature, clinical and histopathological findings, differential diagnosis, associations, management and the pharmacotherapy decision points. KFD diagnosis can be done via excisional lymph node biopsy, which demonstrates characteristic paracortical necrosis with abundant karyorrhectic debris, preponderance of CD8-positive T lymphocytes and histiocytes, and absence of neutrophils.[2,7] Most patients recover spontaneously within one to four months and only require symptomatic therapy; corticosteroids are only used for severe, prolonged, or complicated forms of the disease, while hydroxychloroquine is recommended as a possible steroid sparing therapy in recurrent cases and in children.[9,10,11] Because many KFD patients develop SLE later, a long follow-up is needed even in case of spontaneous recovery.[14,15,16,24] This paper highlights several limitations in the current literature on KFD, which limit its usefulness in terms of management recommendation, i.e. lack of randomized controlled trials of all treatments and absence of evidence-based clinical guidelines.

Keywords: Kikuchi-Fujimoto disease; histiocytic necrotizing lymphadenitis; hydroxychloroquine.`