Kikuchi Disease - Pathophysiology

Pathophysiology

Some studies have suggested a genetic predisposition to the proposed autoimmune response. Several infectious candidates have been associated with Kikuchi's disease.

There are many postulates about the etiology of KFD. A microbial/viral or autoimmune cause has been suggested. Mycobacterium szulgai, Yesinia and Toxoplasma have been implicated. More recently there has been growing evidence of the role of Epstein-Barr virus, as well as other viruses (HHV6, HHV8, Parvovirus B19, HIV- and HTLV-1) in the pathogenesis of KFD. But serologic tests including antibodies to a host of viruses have consistently proven noncontributory and no viral particles have been identified ultrastructurally. It is now proposed that KFD is a nonspecific hyperimmune reaction to a variety of infectious, chemical, physical and neoplastic agents. In addition to SLE, other autoimmune conditions and manifestations such as antiphospholipid syndrome, polymyositis, systemic juvenile idiopathic arthritis, bilateral uveitis, arthritis and cutaneous necrotizing vasculitis have been linked to KFD. It is possible that KFD may represent an exuberant T-cell mediated immune response in a genetically susceptible individual to a variety of non-specific stimuli.