Lupus and Kikuchi-Fujimoto Disease: A Combination for Catastrophe

Abstract

Kikuchi-Fujimoto disease (KFD) is a self-limiting disease of idiopathic origin affecting young women characterized by unexplained fever and lymphadenopathy. It has been usually found to be associated with autoimmunity, of which systemic lupus erythematosus (SLE) is the most outstanding. Fever and lymphadenopathy carry a broad differential, and a missed diagnosis of a rare condition such as Kikuchi can lead to inappropriate treatment in an otherwise benign condition. Therefore, careful examination and histologic confirmation of the diagnosis are critical.

A Rare Case of Idiopathic Lymphadenopathy

“…3 Interestingly, KFD has also occurred in conjunction with SLE, preceding its onset in 30% of cases. 4 The FNA biopsy is not specific in diagnosing KFD, with an accuracy of 56.7%. 5 Therefore, excisional biopsy of the affected lymph nodes is often required.…”

“…Individuals with KFD have a significantly higher incidence of human leukocyte antigen class II genes (specifically, the DPA1*01 and DPB1*0202 alleles, which are more common in Asian individuals) . Interestingly, KFD has also occurred in conjunction with SLE, preceding its onset in 30% of cases …”

Kikuchi fujimoto disease in a young male post-COVID -19 vaccination

“…The positive expression of immunohistochemical markers such as CD 68 and CD 4 suggests Kikuchi disease. 12 The treatment of Kikuchi disease is supportive management along with steroid therapy. Though the disease resolves on its own, regular follow up must be done to know the condition of the patient and for disease recurrence.…”

A Case Report of Kikuchi Fujimoto Disease as an Antecedent Illness of Systemic Lupus Erythematosus in a Male

“…Other than SLE, adult-onset Still's disease, SARS-CoV-2 infection, and Sjogren disease are a few disease entities reported to coexist with Kikuchi disease in the literature [8][9][10]. Serology for Epstein-Barr virus, cytomegalovirus, HIV, toxoplasmosis, Yersinia enterocolitica, and cat scratch disease is often done to exclude infection-related fever and lymphadenopathy [4]. The difference between Kikuchi and SLE is that Kikuchi presents with prominently cervical lymphadenopathy with rarely positive ANA and a self-limiting nature, whereas in SLE, ANA positivity predominates and needs specific treatment.…”

“…Other than systemic lupus erythematosus (SLE), Kikuchi disease is also associated with several autoimmune and infectious diseases. The published reviews estimate that in 30% of cases, KFD proceeds with SLE, whereas in 23% of cases, it follows SLE [4].…”