Generic placeholder image

Current Rheumatology Reviews

Editor-in-Chief

ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

Systematic Review Article

Kikuchi-Fujimoto Disease: A Distinct Pathological Entity but also an "Overlap" Autoimmune Syndrome: A Systematic Review

Author(s): Fabiana Zaccarelli, Marco de Vincentiis, Giovanni D’Erme, Antonio Greco, Francesco Natalucci and Massimo Fusconi*

Volume 19, Issue 2, 2023

Published on: 17 October, 2022

Page: [159 - 167] Pages: 9

DOI: 10.2174/1573397118666220903123128

Price: $65

Abstract

Background: The association between KFD and autoimmune diseases, not only with systemic lupus erythematosus, has been repeatedly described.

Objective: The aim of this review is to evaluate whether an overlap syndrome is present between KFD and autoimmune diseases, whether there is a chronological and a casual relationship between the pathologies.

Methods: The databases used for the overlap case search were Medline and Embase from which we extrapolated the studies of interest. The search queries used were: Kikuchi-Fujimoto Syndrome and juvenile idiopathic arthritis or systemic lupus erythematosus or Systemic Sclerosis or Antiphospholipid Syndrome or Sjogren's Syndrome. All study types were considered (n = 103).

Results: Total number of included studies are 43. We have shown that there is an "overlap" syndrome between KFD and other autoimmune diseases. The chronology of disease onset was variable; autoimmune disease may be "preceding" (n = 11 cases) or "simultaneous" (n = 20 cases) or "post" (n = 8 cases). Kikuchi-Fujimoto Syndrome. Also, the autoimmune disease can present with a complete clinical picture or only with the presence of autoantibodies.

Conclusion: the different pathologies associated with KFD with different chronologies would suggest that there is an alteration of the immune system that allows the pathologies to occur in different temporal relationships.

Keywords: Kikuchi-Fujimoto disease, Juvenile idiopathic arthritis, Systemic Lupus Erythematosus, Systemic Sclerosis, Antiphospholipid Syndrome , Sjogren's Syndrome, Histiocytes.

Graphical Abstract

[1]
Pepe F, Disma S, Teodoro C, Pepe P, Magro G. Kikuchi-Fujimoto disease: A clinicopathologic update. Pathologica 2016; 108(3): 120-9.
[PMID: 28195263]
[2]
Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease: a comprehensive review. Am J Clin Pathol 2004; 122(1): 141-52.
[http://dx.doi.org/10.1309/YF081L4TKYWVYVPQ] [PMID: 15272543]
[3]
Lin H, Su CY, Huang CC, Hwang CF, Chien CY. Kikuchi’s disease: a review and analysisof 61 cases. Otolaryngol Head Neck Surg 2003; 128(5): 650-3.
[http://dx.doi.org/10.1016/S0194-5998(02)23291-X] [PMID: 12748557]
[4]
Halawa ARR, Ahmad MK, Nashwan AJ. An atypical presentation of Kikuchi‐Fujimoto disease: A case report & literature review. Clin Case Rep 2020; 8(12): 3514-8.
[http://dx.doi.org/10.1002/ccr3.3373] [PMID: 33363963]
[5]
Gionanlis L, Katsounaros M, Bamihas G, Fragidis S, Veneti P, Sombolos K. Kikuchi-Fujimoto disease and systemic lupus erythematosus: the EBV connection? Ren Fail 2009; 31(2): 144-8.
[http://dx.doi.org/10.1080/08860220802595518] [PMID: 19212912]
[6]
Sharma V, Rankin R. Fatal Kikuchi-like lymphadenitis associated with connective tissue disease: a report of two cases and review of the literature. Springerplus 2015; 4(1): 167.
[http://dx.doi.org/10.1186/s40064-015-0925-7] [PMID: 25897412]
[7]
Hurtado-Díaz J, Espinoza-Sánchez ML, Rojas-Milán E, et al. Kikuchi-Fujimoto disease: report of a case with progression to lupus nephritis. Am J Case Rep 2021; 22: e927351.
[http://dx.doi.org/10.12659/AJCR.927351] [PMID: 33677464]
[8]
Ghadiri N, Stanford M. Case of vaso-occlusive retinopathy in Kikuchi-Fujimoto and lupus overlap syndrome. BMJ Case Rep 2021; 31; 14(5): e240752.
[http://dx.doi.org/10.1136/bcr-2020-240752]
[9]
Pradhan S, Sirka CS, Dash G, Sahu K, Rout AN. A challenging case of Kikuchi-Fujimoto disease with systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis in a young man. Indian J Dermatol Venereol Leprol 2021; 87(1): 78-82.
[http://dx.doi.org/10.25259/IJDVL_893_18] [PMID: 33580926]
[10]
Müller CSL, Vogt T, Becker SL. Kikuchi-Fujimoto disease triggered by systemic lupus erythematosus and mycoplasma pneu_moniae infection-A report of a case and a review of the literatu. Am J Dermatopathol 2021; 43(3): 202-8.
[http://dx.doi.org/10.1097/DAD.0000000000001764] [PMID: 32809980]
[11]
Danai MM, Kaye SP, Gong R, McKnight HJ. A rare pediatric case of persistent kikuchi-fujimoto disease and underlying systemic lupus erythematosus. Clin Rheumatol 2021; 27(3): e111-2.
[http://dx.doi.org/10.1097/RHU.0000000000001287]
[12]
Pham AK, Castillo SA, Barton DT, et al. Kikuchi-Fujimoto disease preceded by lupus erythematosus panniculitis: do these findings together herald the onset of systemic lupus erythematosus? Dermatol Online J 2020; 26(8): 13030/qt6tx957m2.
[http://dx.doi.org/10.5070/D3268049885]
[13]
Canadas S, Fernandes R, Santos M, Vera-Cruz A, Moreira AT. Cervical lymphadenopathy in two young women the same rare diagnosis with different presentations. Eur J Case Rep Intern Med 2020; 7(4): 001516.
[http://dx.doi.org/10.12890/2020_001516]
[14]
Vithoosan S, Karunarathna T, Shanjeeban P, et al. Kikuchi-Fujimoto disease associated with systemic lupus erythematosus complicated with hemophagocytic lymphohistiocytosis: a case report. J Med Case Reports 2019; 13(1): 173.
[http://dx.doi.org/10.1186/s13256-019-2100-1]
[15]
Hwang JH, Yoo WH, An AR, Choi YJ. Coexistence of systemic lupus erythematosus with Kikuchi-Fujimoto disease involving the salivary gland, initially disguised as lymphoma. Rheumatology (Oxford) 2019; 58(3): 550-3.
[http://dx.doi.org/10.1093/rheumatology/key353] [PMID: 30521051]
[16]
Bari A, Zeeshan F, Hanif G, Jabeen U, Bano I, Rathore AW. A rare case of kikuchi-fujimoto disease associated with systemic lupus erythematosus. J Coll Physicians Surg Pak 2018; 28(6): S143-5.
[http://dx.doi.org/10.29271/jcpsp.2018.06.S143] [PMID: 29866252]
[17]
Găman M, Vlădăreanu AM, Dobrea C, et al. A challenging case of Kikuchi-Fujimoto disease associated with systemic lupus erythematosus and review of the literature. Case Rep Hematol 2018; 2018: 1-5.
[18]
Kinouchi R, Kinouchi M, Ishibazawa A, Yoshida A. Macular capillary recovery in systemic lupus erythematosus complicated by Kikuchi-Fujimoto disease. Int Ophthalmol 2018; 38(4): 1797-801.
[http://dx.doi.org/10.1007/s10792-017-0650-z] [PMID: 28695380]
[19]
Laeng RH, Stamm B. Kikuchi’s histiocytic necrotizing lymphade_nitis driven by activated cytolytic T-cells: an example associated with systemic scleroderma. Histopathology 1999; 34(4): 373-4.
[http://dx.doi.org/10.1046/j.1365-2559.1999.0669a.x] [PMID: 10231407]
[20]
Jiménez SJM, Lorente AEM, Fuentes SF, Miguel GF, Álvarez AR. Kikuchi-Fujimoto disease and its association to systemic lupus erythematosus. An Med Interna 2001; 18(8): 429-31.
[http://dx.doi.org/10.4321/S0212-71992001000800007] [PMID: 11589082]
[21]
Dumas G, Prendki V, Haroche J, et al. Kikuchi-Fujimoto disease. Medicine 2014; 93(24): 372-82.
[http://dx.doi.org/10.1097/MD.0000000000000220] [PMID: 25500707]
[22]
Montigny PM, Camboni A, Houssiau FA. Unusual cause of lymphadenopathy in a patient with systemic sclerosis. Clin Exp Rheumato 2020; 38 Suppl 125(3): 178-9.
[23]
Dalkiliç E, Karakoç Y, Tolunay S, Yurtkuran M. Systemic lupus erythematosus presenting as Kikuchi-Fujimoto disease. Clin Exp Rheumatol 2001; 19(2): 226.
[PMID: 11326493]
[24]
Santana A, Lessa B, Galrão L, Lima I, Santiago M. Kikuchi-Fujimoto’s disease associated with systemic lupus erythematosus: case report and review of the literature. Clin Rheumatol 2005; 24(1): 60-3.
[http://dx.doi.org/10.1007/s10067-004-0923-6] [PMID: 15517448]
[25]
Chen HC, Lai JH, Huang GS, et al. Systemic lupus erythematosus with simultaneous onset of Kikuchi-Fujimoto’s disease complicated with antiphospholipid antibody syndrome: a case report and review of the literature. Rheumatol Int 2005; 25(4): 303-6.
[http://dx.doi.org/10.1007/s00296-004-0507-4] [PMID: 15645234]
[26]
Shusang V, Marelli L, Beynon H, et al. Autoimmune hepatitis associated with Kikuchi–Fujimotoʼs disease. Eur J Gastroenterol Hepatol 2008; 20(1): 79-82.
[http://dx.doi.org/10.1097/MEG.0b013e32825a6a9e] [PMID: 18090996]
[27]
Goldblatt F, Andrews J, Russell A, Isenberg D. Association of Kikuchi-Fujimoto’s disease with SLE. Rheumatology (Oxford) 2007; 47(4): 553-4.
[http://dx.doi.org/10.1093/rheumatology/ken008] [PMID: 18304938]
[28]
Frikha F, Marzouk S, Frigui M, et al. Kikuchi-Fujimoto’s disease and connective tissue disease: a report of three cases. Rev Med Interne 2008; 29(2): 129-34.
[http://dx.doi.org/10.1016/j.revmed.2007.07.012] [PMID: 17981372]
[29]
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): 301-5.
[http://dx.doi.org/10.3109/s10165-009-0269-7] [PMID: 20082108]
[30]
Abraham M, Lazareth I, Bonardel G, et al. Kikuchi disease and lupus: case report, literature review and FDG PET/TC interest. J Mal Vasc 2011; 36(4): 274-9.
[http://dx.doi.org/10.1016/j.jmv.2011.05.004] [PMID: 21757306]
[31]
Sopeña B, Rivera A, Vázquez-Triñanes C, et al. Autoimmune manifestations of Kikuchi disease. Semin Arthritis Rheum 2012; 41(6): 900-6.
[http://dx.doi.org/10.1016/j.semarthrit.2011.11.001]
[32]
Smith LW, Petri M, Petri M. Diffuse lymphadenopathy as the presenting manifestation of systemic lupus erythematosus. J Clin Rheumatol 2013; 19(7): 397-9.
[http://dx.doi.org/10.1097/RHU.0b013e3182a6a924] [PMID: 24048114]
[33]
Rue KS, Rodger DC, Rao NA, Rodger DC, Narsing A, Rao NA. Retinopathy in lupus transitioned to Kikuchi-Fujimoto disease. Am J Ophthalmol Case Rep 2016; 3(3): 43-6.
[http://dx.doi.org/10.1016/j.ajoc.2016.06.004] [PMID: 29503907]
[34]
Jayawickreme KP, Subasinghe S, Weerasinghe S, Perera L, Dissanayaka P. A young girl with chronic isolated cervical lymphadenopathy found to have lupus lymphadenopathy, progressing to develop lupus nephritis. J Med Case Reports 2021; 15(1): 328.
[http://dx.doi.org/10.1186/s13256-021-02949-5]
[35]
Akhavanrezayat A, Cooper JD, Hassan M, Pham BH, Nguyen QD, Farr AK. Bilateral preretinal hemorrhage associated with Kikuchi-Fujimoto disease. Am J Ophthalmol Case Rep 2021; 22: 101041.
[http://dx.doi.org/10.1016/j.ajoc.2021.101041] [PMID: 33732946]
[36]
Miyashita Y, Yamaguchi M, Fujimoto W. Painful indurated erythema suggestive of Kikuchi-Fujimoto disease in a patient with primary Sjögren’s syndrome. J Dermatol 2003; 30(8): 608-11.
[http://dx.doi.org/10.1111/j.1346-8138.2003.tb00443.x] [PMID: 12928530]
[37]
Soy M, Peynirci H, Bilgi S, Adali MK, Güresci S. Kikuchi-Fujimoto disease coexisted with Sjogren’s syndrome. Clin Rheumatol 2007; 26(4): 607-8.
[http://dx.doi.org/10.1007/s10067-005-0184-z] [PMID: 16465479]
[38]
Bogusz AM, Bhargava P. Recurrent histiocytic necrotizing lymphadenitis with a long latency in a patient with autoimmunity: a case report and review of literature. Int J Surg Pathol 2013; 21(3): 287-96.
[http://dx.doi.org/10.1177/1066896912467369] [PMID: 23204033]
[39]
Zhang J, Yang J, 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.
[PMID: 26770297]
[40]
Al-Allaf AW, Yahia Y. Kikuchi-Fujimoto disease associated with sjögren’s syndrome: A case report. Eur J Case Rep Intern Med 2018; 25; 5(5): 000856.
[http://dx.doi.org/10.12890/2018_000856] [PMID: 30756036]
[41]
Wiśniewska K, Pawlak-Buś K, Leszczyński P. Kikuchi-Fujimoto disease associated with primary Sjögren’s syndrome - literature review based on a case report. Reumatologia 2020; 58(4): 251-6.
[http://dx.doi.org/10.5114/reum.2020.98438] [PMID: 32921833]
[42]
Kim TY, Ha KS, Kim Y, Lee J, Lee K, Lee J. Characteristics of Kikuchi–Fujimoto disease in children compared with adults. Eur J Pediatr 2014; 173(1): 111-6.
[http://dx.doi.org/10.1007/s00431-013-2131-3] [PMID: 23955486]
[43]
Marsili M, Nozzi M, Onofrillo D, Sieni E, Chiarelli F, Breda L. Kikuchi disease, macrophage activation syndrome, and systemic juvenile arthritis: A new case associated with a mutation in the perforin gene. Scand J Rheumatol 2015; 44(5): 429-30.
[http://dx.doi.org/10.3109/03009742.2015.1033009] [PMID: 25974073]
[44]
Ramanan AV, Wynn RF, Kelsey A, Baildam EM. Systemic juvenile idiopathic arthritis, Kikuchi’s disease and haemophagocytic lymphohistiocytosis-is there a link? Case report and literature review. Br J Rheumatol 2003; 42(4): 596-8.
[http://dx.doi.org/10.1093/rheumatology/keg167] [PMID: 12649409]
[45]
Eisner MD, Amory J, Mullaney B, Tierney L Jr, Browner WS. Necrotizing lymphadenitis associated with systemic lupus erythematosus. Semin Arthritis Rheum 1996; 26(1): 477-82.
[http://dx.doi.org/10.1016/S0049-0172(96)80028-X] [PMID: 8870115]
[46]
Cramer JP, Schmiedel S, Alegre NG, Schäfer H, Burchard GD, Merz H. Necrotizing lymphadenitis: Kikuchi-Fujimoto disease alias lupus lymphadenitis? Lupus 2010; 19(1): 89-92.
[http://dx.doi.org/10.1177/0961203309345793] [PMID: 19933723]
[47]
Gordon JK, Magro C, Lu T, et al. Overlap between systemic lupus erythematosus and Kikuchi Fujimoto disease: a clinical pathology conference held by the Department of Rheumatology at Hospital for Special Surgery. HSS J 2009; 5(2): 169-77.
[http://dx.doi.org/10.1007/s11420-009-9123-x] [PMID: 19609622]
[48]
Imamura M, Ueno H, Matsuura A, et al. An ultrastructural study of subacute necrotizing lymphadenitis. Am J Pathol 1982; 107(3): 292-9.
[PMID: 6282130]
[49]
Dima A, Jurcut C, Chasset F, Felten R, Arnaud L. Hydroxychloroquine in systemic lupus erythematosus: overview of current knowledge. Ther Adv Musculoskelet Dis 2022; 14: 1759720X211073001.
[50]
Noursadeghi M, Aqel N, Gibson P, Pasvol G. Successful treatment of severe Kikuchi’s disease with intravenous immunoglobulin. Rheumatology (Oxford) 2006; 45(2): 235-7.
[http://dx.doi.org/10.1093/rheumatology/kei074] [PMID: 16352644]
[51]
Go EJ, Jung YJ, Han SB, Suh BK, Kang JH. A case of Kikuchi-Fujimoto disease with autoimmune thyroiditis. Korean J Pediatr 2012; 55(11): 445-8.
[http://dx.doi.org/10.3345/kjp.2012.55.11.445] [PMID: 23227065]
[52]
Rosado FGN, Tang YW, Hasserjian RP, McClain CM, Wang B, Mosse CA. Kikuchi-Fujimoto lymphadenitis: role of parvovirus B19, Epstein-Barr virus, human herpesvirus 6, and human herpesvirus 8. Hum Pathol 2013; 44(2): 255-9.
[http://dx.doi.org/10.1016/j.humpath.2012.05.016] [PMID: 22939574]
[53]
Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M. Kikuchi-Fujimoto Disease: analysis of 244 cases. Clin Rheumatol 2007; 26(1): 50-4.
[http://dx.doi.org/10.1007/s10067-006-0230-5] [PMID: 16538388]

Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy