AI Article Synopsis

  • Kikuchi-Fujimoto's disease (KFD) and adult-onset Still's disease (AOSD) are uncommon inflammatory conditions with unclear causes, and previous reports indicate instances of them occurring together.
  • This study introduces a new case of KFD and AOSD in a middle-aged Hispanic woman, providing fresh insights into demographic and diagnostic characteristics not seen before in overlapping cases.
  • The findings suggest an overlap syndrome due to shared symptoms and treatment responses, pointing to a potentially similar underlying biological mechanism for both diseases.

Article Abstract

Kikuchi-Fujimoto's disease (KFD) and adult-onset Still's disease (AOSD) are rare idiopathic inflammatory conditions of unknown etiology. Ten prior instances of KFD and AOSD occurring together have been reported in the medical literature. These overlaps, together with certain distinguishing clinical and laboratory characteristics in these co-occurrences, offer insight into the pathophysiology of both of these rare disorders. Too, examination of these cases may help improve the diagnostic evaluation and care of patients afflicted with these rare diseases. We therefore report an additional patient with KFD and AOSD occurring in a middle-aged Hispanic female patient and perform a systematic literature review using the PubMed/MEDLINE and Embase databases to further analyze and compare prior identified cases. Our observations in our index case complement and expand previous reports, including new demographic and diagnostic features not seen in prior cases of overlap. Indeed ours is the first in a patient of Hispanic ethnicity, with retroperitoneal lymphadenopathy, as well as with a skin biopsy consistent with AOSD. Each of the reviewed cases of co-occurrence met the diagnostic criteria for both KFD and AOSD. This finding, in the setting of unique clinical and diagnostic manifestations that are not typically seen in either disease entity alone, suggests the presence of an overlap syndrome. Also, many of the shared clinical features and symptomatic responses to targeted therapies implies a similar, yet still poorly understood, pathophysiologic pathway for the two diseases.

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http://dx.doi.org/10.1007/s10067-021-05769-6DOI Listing

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Kikuchi-Fujimoto disease (KFD) and adult-onset Still disease (AOSD) are two rare conditions whose association poses a significant diagnostic challenge. KFD is characterized by subacute necrotizing lymphadenitis of unknown etiology, primarily affecting young adults, and often presents with fever and posterior cervical lymphadenopathy. AOSD is a systemic inflammatory disorder of unclear origin, defined by high-spiking fever, lymphadenopathy, hepatosplenomegaly, hyperferritinemia, and leukocytosis.

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Article Synopsis
  • Kikuchi-Fujimoto's disease (KFD) and adult-onset Still's disease (AOSD) are uncommon inflammatory conditions with unclear causes, and previous reports indicate instances of them occurring together.
  • This study introduces a new case of KFD and AOSD in a middle-aged Hispanic woman, providing fresh insights into demographic and diagnostic characteristics not seen before in overlapping cases.
  • The findings suggest an overlap syndrome due to shared symptoms and treatment responses, pointing to a potentially similar underlying biological mechanism for both diseases.
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Background: To evaluate the impact of macrophage activation syndrome (MAS) on clinical features in patients with Kikuchi-Fujimoto disease (KFD) and to compare the features of MAS in KFD with those of adult-onset Still's disease (AOSD) and systemic lupus erythematosus (SLE).

Methods: The medical records of febrile patients hospitalised with KFD between November 2005 and April 2017 were reviewed. Patients fulfilling the 2016 classification criteria for MAS were classified as having MAS.

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Kikuchi-Fujimoto's disease (KFD) and adult-onset Still's disease (AOSD) are rare inflammatory conditions with some overlapping features. We encountered a 22-year-old male patient who presented with daily fevers, neck discomfort, and sore throat and subsequently developed rash, arthritis, and cervical lymphadenopathy. Biopsy of the skin rash was consistent with KFD skin involvement.

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