Kikuchi-Fujimoto Disease: A Case Report of Fever and Lymphadenopathy in a Young White Man
Background: Kikuchi-Fujimoto disease (KFD) is a rare cause of rapidly evolving tender cervical lymphadenopathy. It is often initially misdiagnosed and managed as infectious lymphadenitis. Although most cases of KFD are self-limited and improve with antipyretics and analgesics, some are more refractory and may require corticosteroids or hydroxychloroquine therapy.
Case Presentation: A 27-year-old White man presented for evaluation of fevers and painful cervical lymphadenopathy. He was found to have KFD on excisional lymph node biopsy. His symptoms proved challenging to manage with corticosteroids but eventually improved with hydroxychloroquine monotherapy.
Conclusions: KFD diagnosis should be considered irrespective of geographic location, ethnicity, or patient sex. Hepatosplenomegaly is a relatively rare manifestation of KFD that can make it especially difficult to distinguish from lymphoproliferative disorder, such as lymphoma. Lymph node biopsy is the preferred diagnostic approach to achieve a timely and definitive diagnosis. Although usually self-limited, KFD has been associated with autoimmune conditions, including systemic lupus erythematosus. Securing the diagnosis of KFD is therefore crucial to ensuring patients are monitored appropriately for the development of associated autoimmune conditions.
Patient Perspective
This began for me in September 2020 out of the blue. I woke up one day with a random lymph node in my neck but otherwise felt completely healthy, and within 2 to 3 weeks I had never been more sick in my entire life. It came with bouts of fevers, neck pain from the swelling, stomach pain (I later learned an enlarged spleen was the source), terrible night sweats, violent chills where the shaking was uncontrollable for hours at a time, loss of appetite, and countless other symptoms that have come and gone over the past year.
It did take a little while to get a diagnosis, but I understand the autoimmune field is tricky. For about 4 to 5 weeks, I was told to prepare for a lymphoma diagnosis. I ended up doing 2 rounds of prednisone, one for 3 weeks at the end of 2020 and one for 2 months from March to May. The initial round helped quite a bit, but the second round did not have any effect on the lingering symptoms. In my opinion, prednisone is miserable to be on long term and I do not recommend it. The daily AEs that came with it included mood swings, insomnia, weight gain, and more. I have been on hydroxychloroquine now for almost 2 months and although it has some AEs of its own, it is nowhere near as rough as the prednisone and has helped manage my remaining symptoms quite a bit.
This certainly has not been a fun experience, but I was under great care during my time in the hospital and continue to be under good care through the rheumatology clinic. The one thing that could have made a huge difference would have been the issues involved in getting my surgery scheduled while I was still inpatient, which took quite a while. The pain during that time was so intense and unlike anything I have ever experienced before, and it was only the surgery that finally brought me some relief. To paint you a picture, I have broken bones, split my leg open, and have roughly 40 to 50 hours of tattoo work on me, and I have never experienced the level of pain like I felt in my neck and stomach. I remember feeling like someone had wound up and hit me with a baseball bat. The surgery brought me immense relief and if it had occurred when it was originally supposed to, I would have been spared 3 or so days of this type of pain.
It has been almost 10 months since my surgery and diagnosis, and life has mostly returned to normal for me. I am still on long-term medication as I mentioned, and I still deal with fatigue, spleen pain, and several other symptoms, but it is much more under control these days. I feel very fortunate to have been under and continue to be under such great care.
Conclusions
This case report highlights the importance of recognizing KFD as a rare but possible cause of fever and necrotizing cervical lymphadenopathy. KFD often mimics malignant lymphoproliferative disorders, autoimmune diseases such as SLE lymphadenitis, and infectious conditions such as HIV and tuberculous lymphadenitis. While KFD is seen with higher prevalence in Asian countries and was previously thought to be more predominant in females, the diagnosis should still be considered irrespective of geographic location or patient sex. Lymph node biopsy is the preferred diagnostic approach for patients with suspected KFD. Treatment is typically supportive but may consist of glucocorticoids in severe cases. Hydroxychloroquine may be used in refractory cases or as a steroid-sparing regimen when steroid AEs are poorly tolerated. Long-term follow-up is critical for patients with KFD to monitor for both disease recurrence and the development of autoimmune disease, especially SLE.
Acknowledgments
The authors thank Dr. Jacob Pilley for his detailed review of the patient’s pathology results. The authors also extend their gratitude to the patient, who deepened our understanding of this condition and what it is like to live with it.
