Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting disease characterized by cervical lymphadenopathy. Although it was primarily thought to be a disease of young adults, it has been increasingly recognized in children. To define the characteristics of KFD in children, we reviewed the medical records of patients younger than 18 years of age who were diagnosed with KFD from 2001 to 2012 at Korea University Medical Center, as well as worldwide published reports of KFD. A total of 140 pediatric patients and 733 patients of all ages was analyzed. Compared to the female predominance found in adults (2:1), young boys were more commonly affected than young girls (1.4:1). Cervical lymphadenopathy was the most common clinical finding in children, as it was in adults. Lymphadenopathy was more likely to be tender (69 vs. 44 %, p < 0.001) but less generalized (1 vs. 8 %, p < 0.05) in children compared to adults. Fever (82 vs. 35 %, p < 0.001) and rash (10 vs. 4 %, p < 0.05) were observed in children more commonly than in adults. Leukopenia was observed in 50 and 38 % of children and adults, respectively. Rates of recurrence and association with autoimmune diseases in children were comparable to those of adults. Cervical lymphadenopathy was the most common clinical manifestation of KFD in all ages. While fever and rash were more common in children with KFD compared to adults, generalized lymphadenopathy was rarer.
Incomplete clinical manifestations in the younger group and IVGG nonresponsiveness in the older group are associated with the development of KD-associated CAA. These age-specific characteristics could aid the customization of the diagnostic and therapeutic strategies of KD, thereby helping to improve the outcome of this disease.
Clinical characteristics to predict the development of coronary artery abnormalities (CAA) in Kawasaki disease (KD) were assessed by reviewing medical records of patients diagnosed with KD at Korea University Medical Center from March 2001 to February 2005. Of the 285 patients diagnosed with KD, 19 developed CAA (6.7%). Compared with the CAA(-) group, the CAA(+) group had a longer duration of fever after intravenous gamma-globulin (IVGG) injection (2.4+/-2.9 vs. 1.5+/-1.2 days, p=0.008) and higher C-reactive protein (CRP)(12.3+/-7.8 vs. 8.7+/-7.1 mg/dL, p=0.038). In particular, the CAA(+) group tended to have more than 7 days of fever before IVGG and more than 3 days of fever after IVGG (26.3 vs. 5.3%, p<0.001; 26.3 vs. 6.4%, p=0.002). When the IVGG responsiveness was defined by the presence of defervescence within 3 days after IVGG, IVGG-non-responders showed a higher incidence of CAA (22.7 vs. 5.3%, p=0.002). Non-responders had a longer duration of fever after IVGG (5.5+/-1.9 vs. 1.2+/-0.6 days, p<0.001) and a significantly increased CRP, AST, ALT and total bilirubin. Multivariate regression analysis for CAA showed that the only factor significantly associated with the development of CAA was total fever that lasted for longer than 8 days (OR=4.052, 95% CI=1.151-14.263, p=0.0293). Conclusively, the most important predictor of CAA in KD is total duration of fever longer than 8 days. Early identification of IVGG non-responders and active therapeutic intervention for fever in KD cases might decrease the incidence of CAA.
Incomplete Kawasaki disease (KD) comprises a large proportion of the total number of cases. Although it has the potential of delaying diagnosis, it is not conclusive whether an incomplete presentation is a risk factor for coronary artery abnormalities (CAAs). We performed a meta-analysis to establish the risk of CAA in 20 studies including 4,504 cases and 32,519 controls, and the risk of giant aneurysm in two studies including 5,390 cases and 37,648 controls. The pooled results indicated that incomplete KD was associated with an increased risk of CAA [odds ratio (OR) = 1.447, 95 % confidence interval (CI) = 1.158-1.808, p = 0.001]. Subgroup analyses demonstrated higher associations in patients younger than 12 months (OR = 2.023, 95 % CI = 1.252-3.271, p = 0.004), Asians and Indians (OR = 1.57, 95 % CI = 1.234-1.999, p < 0.001 and OR = 7.088, 95 % CI = 1.640-30.631, p = 0.009, respectively). Subgroup analysis according to the period of patient enrollment before and after 2004 showed increased association of incomplete KD with CAA only among studies with patients enrolled after 2004 (OR = 1.969, 95 % CI = 1.240-3.127, p = 0.004). In conclusion, incomplete KD seems to be associated with an increased risk of CAA, and this is more prominent in patients younger than 12 months, Asians and Indians.
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