Kikuchi-Fujimoto disease and prognostic implications

Abstract

The most common examination findings in a 2006 case review of 266 KFD cases, by Kucukardali et al. Other findings include arthritis, weight loss, anorexia, hepatomegaly, splenomegaly and sweating. 18% of patients had leucopenia, with 16% having a raised ESR. SLE association: KFD's association with SLE is illustrated in Kucukardali e t al's case study, whereby 9% of European KFD patients and 28% of east-Asian patients had concomitant SLE. KFD can occur in patients with pre-existing SLE, coexist with SLE or evolve into SLE. The clinical and histopathologic features of KFD are closely simulated by SLE. Yilmaz et al reviewed two cases of KFD mimicking SLE, speculating that both SLE and KFD share a common hyperimmune reaction to antigenic stimuli, however then diverge into two distinct manifestations. It appears the association of KFD with SLE can predispose the patient to SLE at any time.There is no clear evidence to determine the best way to follow up KFD in regards to long-term complications and associations. It appears it would be appropriate to perform an SLE screen following the initial diagnosis of KFD, followed by a yearly SLE screen.Likewise, ESR monitoring in primary care can be used to assess for any autoimmune, infectious or malignant developments. 3-4% of KFD cases recur, with the optimum management of patients with recurrent KFD yet to be reported. Regardless, KFD has an excellent prognosis with most cases resolving within six months. Conclusion:Chronic lymph node enlargement and fever should be diagnosed carefully, with KFB a viable diagnosis. Gold standard is histological examination of involved lymph node. Although a selflimiting condition, KFD is associated with an increased risk of SLE, malignancy and other autoimmune diseases and therefore early diagnosis and long term follow up are recommended. Following initial investigations to rule out infectious and non-infectious aetiologies, we recommend long-term follow up in primary care with a yearly SLE screen and ESR blood test.