Late recurrence of Kikuchi-Fujimoto disease nine years after initial diagnosis: a case-based review

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare, benign, and self-limited inflammatory lymphadenitis. It predominantly affects young adults and typically presents with tender cervical lymphadenopathy, low-grade fever, and constitutional symptoms such as malaise, night sweats, and weight loss. Though initially recognized in Asian populations, cases are now reported worldwide. The etiology remains uncertain, but KFD has recognized overlap with autoimmune diseases. Systemic lupus erythematosus (SLE) and Sjögren's disease (SjD) account for most coexisting disorders. We present the case of a woman in her early forties with recurrent cyclical fevers and cervical lymphadenopathy. She had biopsy-proven KFD but showed relapse after a nine-year asymptomatic period after her first manifestation. A case-based review about KFD recurrence was conducted using PubMed/MEDLINE databases following CaBArET guidelines and identified a total of 21 reports for review of the clinical manifestations and treatment approaches of recurrent KFD. Our findings suggest that although KFD is typically self-limited, recurrence may be associated with autoimmune features in selected cases, emphasizing the need for renewed diagnostic vigilance even years after apparent resolution. Although treatment responses in recurrent Kikuchi-Fujimoto disease are heterogeneous, literature evidence and our case suggest that corticosteroids remain first-line, while agents such as hydroxychloroquine or anakinra may offer benefit in steroid-dependent or relapsing cases. However, these remain individualized interventions rather than standardized protocols, and controlled evidence is lacking. Key Points • Kikuchi-Fujimoto disease, although typically self-limited, can relapse after unusually long latency, highlighting the need for continued clinical awareness. • Recurrent presentations may mimic infectious or malignant conditions, often leading to extensive diagnostic work-ups. • Reactive histologic findings at recurrence do not exclude KFD and should be interpreted in the context of prior confirmed disease and clinical evolution.