Kikuchi-Fujimoto Disease Preceding Overlap Syndrome of Sjögren’s Syndrome and Systemic Lupus Erythematosus: Literature Review Based on a Case Report
Keywords:
Kikuchi-Fujimoto disease, Overlap Syndrome, Sjögren’s Syndrome, Systemic Lupus Erythematosus, Inflammatory MyositisAbstract
Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting histiocytic necrotizing lymphadenitis systemic disorder with unknown etiology. KFD has been known for half a century, but difficulties in distinguishing it remain. Its diagnostic significance is related to the increasing prevalence of KFD with autoimmune diseases in various timeframes. Systemic lupus erythematosus (SLE) is the most prevalent autoimmune connective tissue disease (AICTD) appearing alongside KFD. An 18-year-old female presented with acute muscle weakness, shortness of breath, fever, and significant weight loss for 5 months before admission. Pain and morning joint stiffness had been felt for 9 months. One year ago, she lumped her right neck and was diagnosed with KFD from the excision biopsy and immunohistochemical staining (CD68). Creatine-kinase enzymes and C-Reactive protein were elevated with a high anti-Ku and anti-Jo-1 negative level. There was a low level of complements, high anti-nuclear antibody titer, with positive anti-SS-A. Sialometry and Schirmer test showed reduced salivary and lacrimal gland production. We diagnosed this patient as having an overlap syndrome preceded by KFD. The AICTD involved was Sjögren’s syndrome and SLE. Although KFD is considered a self-limiting disease, its occurrence should be noticed regarding the possibility of other autoimmune conditions. KFD usually coincides with AICTD, although it could also precede or occur afterward. This case is reported to raise awareness of the overlap syndrome preceded by KFD.References
Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes: a clinicopathological study. Acta Hematol Jpn. 1972;35:379-80.
Fujimoto, Y. Kozima, Y. Yamaguchi K. Cervical subacute necrotizing lymphadenitis: a new clinicopathologic entity. Naika. 1972;20:920-7.
Xu S, Sun W, Liu J. Kikuchi-Fujimoto disease: A case report and the evaluation of diagnostic procedures. BMC Oral Health. 2019;19(1):1-5. doi:10.1186/s12903-019-0920-4
Zaccarelli F, de Vincentiis M, D’Erme G, Greco A, Natalucci F, Fusconi M. Kikuchi-Fujimoto disease: a distinct pathological entity but also an “overlap” autoimmune syndrome - a systematic review. Curr Rheumatol Rev. 2022;(3 Sep).
Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M. Kikuchi-Fujimoto disease: analysis of 244 cases. Clin Rheumatol. 2007;26(1):50-54. doi:10.1007/s10067-006-0230-5
Radfar L, Radfar M, Moser KL, Scofield RH. Kikuchi-Fujimoto disease in patients with Sjögren ’ s syndrome. 2013;2013(January):32-36.
Wilkinson CE, Nichol F. Kikuchi-Fujimoto disease associated with polymyositis. Rheumatology. 2000;39(11):1302-1304. doi:10.1093/rheumatology/39.11.1302
Sopeña B, Rivera A, Vázquez-Triñanes C, et al. Autoimmune manifestations of Kikuchi disease. Semin Arthritis Rheum. 2012;41(6):900-906. doi:10.1016/j.semarthrit.2011.11.001
Khan AM, Ahmad M, Muhammad O, Taj S, Shiza ST. Kikuchi-Fujimoto disease in a young female: A case report and literature review. Cureus. 2021;13(11):11–4.
AlShieban S, Masuadi E, Alghamdi R, et al. Pathological features and clinical characteristics of Kikuchi-Fujimoto disease: A tertiary hospital experience in Riyadh, Saudi Arabia. Cureus. 2023;15(1).
Barbar S, Noventa F, Rossetto V, et al. A risk assessment model for the identification of hospitalized medical patients at risk for venous thromboembolism: The Padua Prediction Score. J Thromb Haemost. 2010;8(11):2450-7. doi:10.1111/j.1538-7836.2010.04044.x
Bottai M, Tjärnlund A, Santoni G, et al. EULAR/ACR classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups: A methodology report. RMD Open. 2017;3(2):1-10. doi:10.1136/rmdopen-2017-000507
Sifuentes Giraldo W., Bouruncel Alaluna C, Roy Ariño G, García Villanueva M., de La Puente Bujidos C, Gámir Gámir M. Autoimmune diseases associated with anti-ku antibodies: A retrospective case series. Ann Rheum Dis. 2016;75(Suppl 2):544-5. doi:10.1136/annrheumdis-2016-eular.2478
Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren’s Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol. 2017;69(1):35-45. doi:10.1002/art.39859
Amin R. Review Article Kikuchi -Fujimoto Disease- a Comprehensive Review. Bangladesh J Med. 2013;24:70-7.
Salah A, Al-Fadhli A. Kikuchi-fujimoto disease. Kuwait Med J. 2014;46(4):340-1. doi:10.5858/134.2.289
Ogata S, Bando Y, Saito N, Katsuoka K, Ishii M. Kikuchi-Fujimoto disease developed into autoimmune disease: A report of two cases. Mod Rheumatol. 2010;20(3):301-305. doi:10.1007/s10165-009-0269-7
Ghadiri N, Stanford M. Case of vaso-occlusive retinopathy in Kikuchi-Fujimoto and lupus overlap syndrome. BMJ Case Rep. 2021;14(5).
Lu, S. Zhang, J. Zhou, W. Wang X. Histiocytic necrotic lymphadenitis as an initial presentation in two children with Sjogren’s syndrome and/or systemic lupus erythematous. Chinese J Contemp Pediatr. 2010;12(4):311-312.
Jun Z, Jun Y, Weng WW, Zhu YJ, Qiu H, Dong MJ. Kikuchi-Fujimoto disease associated with Sjogren’s syndrome: A case report and review of the literature. Int J Clin Exp Med. 2015;8(10):17061-6.
Cheng CY, Sheng WH, Lo YC, Chung CS, Chen YC, Chang SC. Clinical presentations, laboratory results and outcomes of patients with Kikuchi’s disease: Emphasis on the association between recurrent Kikuchi’s disease and autoimmune diseases. J Microbiol Immunol Infect. 2010;43(5):366-371. doi:10.1016/S1684-1182(10)60058-8
Campbell T, Auer I, Martin L. A case of concurrent Kikuchi-Fujimoto disease and neuropsychiatric lupus in a patient with rheumatoid arthritis and systemic lupus erythematosus overlap syndrome. McMaster Univ Med J. 2019;16(1):1-5. doi:10.15173/mumj.v16i1.2026
Baizabal-Carvallo JF, Jankovic J. Stiff-person syndrome: Insights into a complex autoimmune disorder. J Neurol Neurosurg Psychiatry. 2015;86(8):840-8. doi:10.1136/jnnp-2014-309201
Ashton C, Paramalingam S, Stevenson B, Brusch A, Needham M. Idiopathic inflammatory myopathies: a review. Intern Med J. 2021;51(6):845-852. doi:10.1111/imj.15358
Firestein GS. Firestein & Kelley’s Textbook of Rheumatology. 10th ed. Elsevier; 2017.
Pepmueller PH. Undifferentiated connective tissue disease, mixed connective tissue disease, and overlap syndromes in Rheumatology. Mo Med. 2016;113(2):136-40.
Ramos-Casals M, Brito-Zerón P, Bombardieri S, et al. EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies. Ann Rheum Dis. 2020;79(1):3-18. doi:10.1136/annrheumdis-2019-216114
Bosch X, Guilabert A. Kikuchi-Fujimoto disease. Orphanet J Rare Dis. 2006;1(1):3-5. doi:10.1186/1750-1172-1-18
Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol. 2019;71(9):1400-12. doi:10.1002/art.40930
Aguila LA, Lopes MRU, Pretti FZ, et al. Clinical and laboratory features of overlap syndromes of idiopathic inflammatory myopathies associated with systemic lupus erythematosus, systemic sclerosis, or rheumatoid arthritis. Clin Rheumatol. 2014;33(8):1093-8. doi:10.1007/s10067-014-2730-z
Mok CC, Hamijoyo L, Kasitanon N, et al. The Asia-Pacific league of associations for rheumatology consensus statements on the management of systemic lupus erythematosus. Lancet Rheumatol. 2021;9913(21):1-15. doi:10.1016/s2665-9913(21)00009-6
Downloads
Published
How to Cite
Issue
Section
License
Copyright
The authors who publish in this journal agree to the following requirements:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0) that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors can enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) before and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work. (See The Effect of Open Access)
Privacy Statement
The names and email addresses entered in this journal site will be used exclusively for the stated purposes of this journal and will not be made available for any other purpose or to any other party.
