Kikuchi Fujimoto disease is a rare, self-limiting condition that is usually prevalent in Asian countries, clinically presenting as tender cervical lymphadenopathy with or without systemic symptoms. The etiology of the disease is yet to be uncovered, although certain studies have suggested an underlying viral infection or autoimmune disease to trigger the onset of Kikuchi Fujimoto disease2. A hospital based case study was conducted on 67 patients diagnosed with Kikuchi’s disease, in order to determine the sociodemographic profile, symptomatology, and the outcome of the disease.
The mean age of Kikuchi’s disease prevalence was found to be 27.1 years in this study while in a study done in Sub-Saharan Africa, in October 2017, an average of 21 years was recorded10. The youngest patient in our study was a 4 year old girl and the oldest, a 77 year old man. Of the study population 16 were children (ranging from 4–18 years old) with confirmed cases of Kikuchi’s disease. A study done on 6 confirmed pediatric cases of Kikuchi’s disease, in North India, in September 2019 reported a mean age of 10.8 years15.
On analysis of the gender prevalence in this study, a female predominance of 1.91:1 was observed. Meanwhile, in a study done South India, there was a significant female majority of 2.4:19. However, a gender comparison of 1:1 was observed in the results obtained from a study conducted in Italy, in September 20165. A reversal of the gender ratio in our study was observed in a study carried out in Korea which showed a significant male majority of 2.8:116, and another conducted in North India where the ratio was 2:115.
Younger females were noted to be more predisposed to KFD in our study with ages ranging from 4–38 years, all considerably younger than their male counterpart in this study. A study of 9 patients was conducted in Sri Lanka by Abeysekara R A et al, where all the cases were female patients in the age group of 12–30 years13. A similar study was carried out by Adhikari R C et al, in Nepal where 5 of the 6 cases were females and the age range was 13–32 years14.
The most common clinical presentation of these patients was tender swelling on the side of the neck (74.6%). Similar results were obtained from a study done in Michigan, in May 2018 with 60–90% of the cases having posterior cervical lymphadenopathy1. Systemic symptoms such as fever (52.2% of the cases) and hepatosplenomegaly (14.9% of the cases) was found abundantly in this study. Fever was noted to be associated frequently with tender cervical lymphadenopathy (n = 30, 44.8%). This coexistence of symptoms was replicated in the Michigan study in 35–77% of the patients1. Tender cervical lymphadenopathy was the most common symptom in studies done in Italy5 (60–98%), Saudi Arabia2 (56–98%) and Michigan1 (60–90%). The involvement of axillary lymph nodes was encountered in 9% of the cases in this study, while the same in a study conducted on 24 cases in Bangalore yielded, 13%9.
One 22 year old male patient in this study presented with symptoms of cervical lymphadenopathy for 15 days associated with systemic symptoms of fever, neck stiffness and meningitis, was treated with steroids, tapered over a period of 1 month. Follow-up over 2 years revealed that lymphadenopathy subsided in 3 months and the patient recovered fully with no recurrence reported. In a study done in Japan by Komagamine T et al, which reported 5 cases of recurrent aseptic meningitis associated with Kikuchi’s Disease, all the cases resolved within several months with 3 requiring treatment with steroids11. Thus the use of steroids in patients with recurrence of Kikuchi Fujimoto disease with neurological involvement is considered beneficial following extensive investigation to ensure its safety11.
The histopathology findings of the excisional lymph node biopsy remains gold standard for confirming Kikuchi’s disease. In this study, the findings were sub grouped into 3 morphological phases as done in previous studies5,7,8, and a vast majority of the confirmed cases presented in proliferative phase of KFD (n = 40, 59.7%).
In contrast to a study that was conducted in Bangalore, in 2013, where the lymphadenopathy lasted from 1 week to 3 months9, this study results suggested a longer duration of symptoms and varied from, 2 weeks to 6 months.
The follow up outcomes of the patients over a period of 9 months revealed that all recovered fully with no complications in this study.
A significant majority of our patients recovered after symptomatic management (62.7% of the cases), which was the choice of initial treatment in previous studies as well1,11,15. One fourth of the patients in this study were treated successfully with steroids (25.4%). A similar result was obtained in a study done by Guleria et al in 2019, where 2 of the 6 patients required steroid therapy15. In a study done in Florida, in 2014, the treatment with methylprednisolone showed a drastic response within 24 hours6.
Even though Kikuchi’s disease is known to be a self-limiting condition, this study showed that only a small fraction of patients recovered completely without any treatment (9%). In contrast, in Florida, majority of the patients had self-limiting cases of KFD which typically lasted 1 to 4 months6, while in a study conducted in England, following the hospital visit for the lymph node biopsy, most of their symptoms resolved spontaneously within 6 months7.
There was minimal recurrence of 3% when patients were followed up for 9 months in this case study, similar results were observed in a studies done in Michigan1, Saudi Arabia2 and Spain12 where the recurrence rate reported was 3–4%. Even though there was no recurrence of symptoms in the children reported in our study, Han H J et al in Korea, recorded a recurrence rate of 27% after a follow up of 1 year16. Meanwhile, a study done in Florida revealed that recurrences in Kikuchi’s disease can occur as late as 8 years following the initial presentation and in this study the recurrence was reported as 15%6. Therefore, long-term follow-up is essential to determine the rate of recurrence of KFD.