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Medical Science Monitor Basic Research


Kikuchi-Fujimoto Disease: Report of a Case with Progression to Lupus Nephritis

Challenging differential diagnosis, Rare coexistence of disease or pathology

Jorge Hurtado-Díaz, María Lucero Espinoza-Sánchez, Eduardo Rojas-Milán, Erik Cimé-Aké, María de los Ángeles Macias, Lizeth Romero-Ibarra, Olga Lidia Vera-Lastra

Mexico Department of Internal Medicine, High Specialty Medical Unit, Specialty Hospital, Dr. Antonio Fraga Mouret, La Raza National Medical Center, México City, Mexico

Am J Case Rep 2021; 22:e927351

DOI: 10.12659/AJCR.927351

Available online: 2021-01-26

Published: 2021-03-07


BACKGROUND: Kikuchi-Fujimoto disease (KFD) is an enigmatic disease, with a distinctive histopathology and a benign and self-limited course. It is more frequent in young Asian women. Autoimmune diseases are identified as one of its triggers; primarily SLE, which may precede, be concomitant with, or develop after the diagnosis of KFD. Patients with KFD should receive periodic follow-up for several years to detect possible evolution of SLE. The main feature of KFD is lymphadenopathy, and cervical lymph nodes are involved in 50% to 98% of cases. Other symptoms such as fever, fatigue, weight loss, and arthralgias are also reported. Differential diagnosis between KFD and SLE is a challenge. When KFD and SLE coexist, a lymph node biopsy may be diagnostic. Treatment should be symptomatic with analgesics and anti-inflammatories, with complete resolution in 3 to 4 months. Corticosteroids and immunosuppressive therapy are justified only in cases concomitant with SLE.
CASE REPORT: We report a case of KFD in a 28-year-old woman who was initially negative for anti-nuclear antibodies (ANA) and anti-double-stranded deoxyribonucleic acid antibodies (anti-dsDNA), but who became antibody-positive and presented with lupus nephritis 2 months later.
CONCLUSIONS: We present a case of a patient with KFD who developed SLE 2 months later; highlighting the importance of recognizing its association and its possible progression to monitor for future development of SLE and provide timely treatment to avoid complications. We also compared the clinical, laboratory, and histological similarities between the 2 entities.

Keywords: Histiocytic Necrotizing Lymphadenitis, Lupus Erythematosus, Systemic, Pathology, Molecular