Case report details KFD patient developing SLE treated with immunosuppression

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Frontiers in Medicine Published April 15, 2026 Medically reviewed April 25, 2026 DOI ↗ By Dr. Sofia Müller, MD · Lifespan & Whole-Person Care

Key Takeaway

Note diagnostic pitfalls of KFD with atypical GI prodrome and need for long-term surveillance.

This publication is a case report and literature review focusing on a single patient with Kikuchi-Fujimoto disease (KFD) who developed systemic lupus erythematosus (SLE). The report details the clinical trajectory of this individual, highlighting the diagnostic challenges associated with an atypical gastrointestinal prodrome in KFD. The authors discuss the association between these two conditions and emphasize the necessity for vigilant long-term surveillance in patients presenting with such features.

Treatment in this specific instance involved initial corticosteroid therapy, which achieved resolution of KFD symptoms, followed by targeted immunosuppressive therapy that resulted in sustained remission of SLE. The report does not provide pooled effect sizes, absolute numbers, or statistical measures such as p-values or confidence intervals, as these data are unavailable in a single-case narrative. Consequently, the effectiveness of these treatments beyond this isolated example cannot be definitively established.

The authors acknowledge significant limitations inherent to this study design. As a case report, it lacks a control group, and the findings cannot be generalized to the broader population. The text explicitly advises against overstating the causality between KFD and SLE or the broad effectiveness of the described therapies. No adverse events, discontinuations, or tolerability data were reported for this patient. Clinicians should interpret these findings with caution, recognizing that this evidence supports diagnostic vigilance rather than providing robust proof of therapeutic efficacy.

Study Details

Study typeSystematic review

EvidenceLevel 1

PublishedApr 2026

View Original Abstract ↓

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare idiopathic inflammatory disorder characterized by low incidence and a propensity for misdiagnosis. Although its etiology remains elusive, KFD is often linked to underlying autoimmune conditions. Definitive diagnosis requires lymph node biopsy. Classically, patients present with cervical lymphadenopathy and fever; however, this report describes an atypical, severe manifestation in a patient whose initial symptoms were dominated by prominent gastrointestinal involvement, including abdominal pain and vomiting. Histopathological evaluation of an excised lymph node confirmed the diagnosis. While corticosteroid therapy led to resolution of KFD symptoms, the patient's course was further complicated by the subsequent onset of systemic lupus erythematosus (SLE). Targeted immunosuppressive therapy for SLE ultimately achieved sustained remission. This case underscores the diagnostic pitfalls of KFD with an atypical gastrointestinal prodrome, reaffirms its established yet pivotal association with SLE, and advocates for vigilant long-term surveillance in affected individuals.