Background/Purpose: Kikuchi-Fujimoto disease (KD), also known as histiocytic necrotizing lymphadenitis, is a self-limited disease of unknown etiology. An association between KD and systemic lupus erythematosus (SLE) has been reported, in which KD diagnosis precedes or follows that of SLE. Dysregulation of type 1 interferon signaling was also observed in lymph node tissues from KD patients. We aimed to explore the clinical characteristics of SLE patients with a preexisting history of KD.

Methods: From 2005 to 2024, patients diagnosed with SLE were identified from electronic medical records. All patients were satisfied with the 2019 EULAR/ACR classification criteria for SLE. Among them, patients with documented KD history in electronic medical records were identified. Patients whose SLE diagnosis predated the KD diagnosis were excluded. Epidemiology and clinical findings, including organ involvement and prognosis, were reviewed.

Results: A total of 1,039 patients with SLE were identified (male: 9.5%). Among them, 37 patients (3.8%) had a preexisting history of KD (male: 8.1%), with a median follow-up of 81 months (IQR: 23-155). For the time interval to SLE following KD diagnosis, nearly 40% of patients (14/37) were diagnosed less than 30 days. The other 23 patients were diagnosed at least 1 month after KD, with a median age at KD diagnosis of 25 years (18-40) and at SLE diagnosis of 31 years (22-45).
Among these 23 patients, seven patients (30.1%) were diagnosed with KD before the age of 19 years old (childhood-onset KD). Their median age at KD diagnosis was 15 years (14-17), and at SLE diagnosis, 17 years (17-21). The median interval to SLE following KD diagnosis was 24 months (10-77), which was longer than the 5 months (1-75) in the adult-onset KD group (16 patients), but the difference was not statistically significant.
Overall, six patients (6/37, 16.2%), including two male patients with childhood-onset KD, presented with neuropsychiatric lupus; of note, all of whom were diagnosed with SLE at least 1 month after KD. Nineteen patients (19/37, 51.4%) presented with lupus nephritis, six patients progressed to chronic kidney disease stage 3 or higher, and two of them developed end-stage renal disease.

Conclusion: The history of KD may be found in a small proportion of newly diagnosed SLE patients. With further studies to analyze the natural course of these patients in detail, guidance on long-term monitoring of childhood-onset KD patients is needed.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/kikuchi-fujimotos-disease-in-systemic-lupus-erythematosus-single-center-experience/