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Kimura disease

MedGen UID:
46183
•Concept ID:
C0033838
•
Disease or Syndrome
Synonyms: Angiolymphoid hyperplasia with eosinophilia; Eosinophilic granuloma of soft tissue; Eosinophilic hyperplastic lymphogranuloma; Eosinophilic lymphofollicular granuloma; Eosinophilic lymphofolliculosis; Kimura's disease
SNOMED CT: Kimura's disease (399894006); Kimura disease (399894006)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
Monarch Initiative: MONDO:0018830
Orphanet: ORPHA482

Definition

Kimura disease is a benign and chronic inflammatory disorder of unknown etiology, occurring mainly in Asian countries (very rarely in Western countries) and predominantly affecting young men, that usually presents with solitary or multiple non-tender subcutaneous masses in the head and neck region (in particular the preauricular and submandibular area) and/or generalized painless lymphadenopathy, often with salivary gland involvement. Characteristic laboratory findings include blood eosinophilia and markedly elevated serum immunoglobulin E (IgE) levels. It is often associated with autoinflammatory disorders (i.e. ulcerative colitis, bronchial asthma) and a co-existing renal disease. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKimura disease
Follow this link to review classifications for Kimura disease in Orphanet.

Professional guidelines

PubMed

Treatment of Kimura Disease With Dupilumab.
Teraki Y, Terao A
JAMA Dermatol 2022 Mar 1;158(3):329-330. doi: 10.1001/jamadermatol.2021.5885. PMID: 35138349
Comparison of Local Recurrence Rate of Three Treatment Modalities for Kimura Disease.
Ye P, Wei T, Yu GY, Wu LL, Peng X
J Craniofac Surg 2016 Jan;27(1):170-4. doi: 10.1097/SCS.0000000000002337. PMID: 26674917
Benign non-infectious causes of lymphadenopathy: A review of cytomorphology and differential diagnosis.
Monaco SE, Khalbuss WE, Pantanowitz L
Diagn Cytopathol 2012 Oct;40(10):925-38. Epub 2012 Feb 20 doi: 10.1002/dc.21767. PMID: 22351626

Recent clinical studies

Etiology

Oropharyngeal Kimura's disease: a diagnostic dilemma and therapeutic challenge.
Saravanam PK, Gajendran A, Dinakaran N, Jayaraman D
BMJ Case Rep 2020 Oct 30;13(10) doi: 10.1136/bcr-2020-236366. PMID: 33127696Free PMC Article
Kimura's disease: effects of age on clinical presentation.
Kakehi E, Kotani K, Otsuka Y, Fukuyasu Y, Hashimoto Y, Sakurai S, Hirotani A, Simizu K, Fujita R, Shoji K, Adachi S, Matsumura M
QJM 2020 May 1;113(5):336-345. doi: 10.1093/qjmed/hcz312. PMID: 31800058
Comparison of Local Recurrence Rate of Three Treatment Modalities for Kimura Disease.
Ye P, Wei T, Yu GY, Wu LL, Peng X
J Craniofac Surg 2016 Jan;27(1):170-4. doi: 10.1097/SCS.0000000000002337. PMID: 26674917
Kimura disease: diagnosis and prognostic factors.
Iwai H, Nakae K, Ikeda K, Ogura M, Miyamoto M, Omae M, Kaneko T, Yamashita T
Otolaryngol Head Neck Surg 2007 Aug;137(2):306-11. doi: 10.1016/j.otohns.2007.03.027. PMID: 17666261
Eosinophilic angiocentric fibrosis.
Clauser L, Mandrioli S, Polito J, Marchetti E
J Craniofac Surg 2006 Jul;17(4):812-4. doi: 10.1097/00001665-200607000-00040. PMID: 16877941

Diagnosis

Kimura Disease: A Case Series and Systematic Review of Clinico-radiological Features.
Sangwan A, Goyal A, Bhalla AS, Kumar A, Sharma R, Arava S, Dawar R
Curr Probl Diagn Radiol 2022 Jan-Feb;51(1):130-142. Epub 2020 Nov 17 doi: 10.1067/j.cpradiol.2020.10.003. PMID: 33250297
Kimura disease.
Zhou P, Zhang W, Shi W, Zhang Y
Dermatol Online J 2017 Oct 15;23(10) PMID: 29469792
Intraparotid Kimura disease.
Bonfils P, Moya-Plana A, Badoual C, Nadéri S, Malinvaud D, Laccourreye O
Eur Ann Otorhinolaryngol Head Neck Dis 2013 Apr;130(2):87-9. Epub 2012 Sep 26 doi: 10.1016/j.anorl.2012.05.001. PMID: 23021002
Kimura disease.
Karaman E, Isildak H, Ozdilek A, Sekercioglu N
J Craniofac Surg 2008 Nov;19(6):1702-5. doi: 10.1097/SCS.0b013e31818ac2a5. PMID: 19098588
Kimura disease.
Abuel-Haija M, Hurford MT
Arch Pathol Lab Med 2007 Apr;131(4):650-1. doi: 10.5858/2007-131-650-KD. PMID: 17425383

Therapy

Efficacy and safety of dupilumab in the treatment of Kimura's disease.
Liu YL, Ran YT, Zhang YF, Peng XT, Xia YM, Yan HL
QJM 2024 Aug 1;117(8):575-580. doi: 10.1093/qjmed/hcae048. PMID: 38492561
Two cases of dupilumab-responsive Kimura disease.
Battesti G, Jachiet M, Lepelletier C, Petit A, Vignon-Pennamen MD, Cassius C, de Masson A, Battistella M, Bagot M, Bouaziz JD, Mahévas T
Clin Exp Dermatol 2024 Apr 23;49(5):502-506. doi: 10.1093/ced/llad455. PMID: 38149974
Anti-immunoglobulin E provides an additional therapy to conventional steroids for Kimura's disease.
Ao S, Huang G, Tang X, Zhu Z, Han J, Wang F, Zhai W
J Dermatol 2024 Apr;51(4):602-606. Epub 2023 Nov 10 doi: 10.1111/1346-8138.17021. PMID: 37950550
Mepolizumab-responsive Kimura disease.
Kinoshita M, Ogawa Y, Onaka M, Shimada S, Kawamura T
J Allergy Clin Immunol Pract 2021 Jul;9(7):2928-2930. Epub 2021 Mar 5 doi: 10.1016/j.jaip.2021.02.049. PMID: 33684634
Comparison of Local Recurrence Rate of Three Treatment Modalities for Kimura Disease.
Ye P, Wei T, Yu GY, Wu LL, Peng X
J Craniofac Surg 2016 Jan;27(1):170-4. doi: 10.1097/SCS.0000000000002337. PMID: 26674917

Prognosis

Kimura Disease: A Case Series and Systematic Review of Clinico-radiological Features.
Sangwan A, Goyal A, Bhalla AS, Kumar A, Sharma R, Arava S, Dawar R
Curr Probl Diagn Radiol 2022 Jan-Feb;51(1):130-142. Epub 2020 Nov 17 doi: 10.1067/j.cpradiol.2020.10.003. PMID: 33250297
Extranodal hematopoietic neoplasms and mimics in the head and neck: an update.
Thakral B, Zhou J, Medeiros LJ
Hum Pathol 2015 Aug;46(8):1079-100. Epub 2015 Jun 1 doi: 10.1016/j.humpath.2015.05.007. PMID: 26118762
Kimura disease: review of the literature.
Sun QF, Xu DZ, Pan SH, Ding JG, Xue ZQ, Miao CS, Cao GJ, Jin DJ
Intern Med J 2008 Aug;38(8):668-72. doi: 10.1111/j.1445-5994.2008.01711.x. PMID: 18808562
Kimura disease: diagnosis and prognostic factors.
Iwai H, Nakae K, Ikeda K, Ogura M, Miyamoto M, Omae M, Kaneko T, Yamashita T
Otolaryngol Head Neck Surg 2007 Aug;137(2):306-11. doi: 10.1016/j.otohns.2007.03.027. PMID: 17666261
Kimura disease.
Abuel-Haija M, Hurford MT
Arch Pathol Lab Med 2007 Apr;131(4):650-1. doi: 10.5858/2007-131-650-KD. PMID: 17425383

Clinical prediction guides

Clinical effects of dupilumab: A novel treatment for Kimura disease.
Shang BS, Hsiao CH, Tsao TF, Liao YY, Lin WL, Lee WI, Lue KH
Immun Inflamm Dis 2023 Nov;11(11):e1084. doi: 10.1002/iid3.1084. PMID: 38018601Free PMC Article
Distinct disease-specific Tfh cell populations in 2 different fibrotic diseases: IgG(4)-related disease and Kimura disease.
Munemura R, Maehara T, Murakami Y, Koga R, Aoyagi R, Kaneko N, Doi A, Perugino CA, Della-Torre E, Saeki T, Sato Y, Yamamoto H, Kiyoshima T, Stone JH, Pillai S, Nakamura S
J Allergy Clin Immunol 2022 Aug;150(2):440-455.e17. Epub 2022 May 11 doi: 10.1016/j.jaci.2022.03.034. PMID: 35568079Free PMC Article
Kimura's disease successively affecting multiple body parts: a case-based literature review.
Yang B, Liao H, Wang M, Long Q, Zhong H, Luo L, Liu Z, Cheng X
BMC Ophthalmol 2022 Apr 2;22(1):154. doi: 10.1186/s12886-022-02378-y. PMID: 35366827Free PMC Article
Treatment algorithm for Kimura's disease: A systematic review and meta-analysis of treatment modalities and prognostic predictors.
Lee CC, Feng IJ, Chen YT, Weng SF, Chan LP, Lai CS, Lin SD, Kuo YR
Int J Surg 2022 Apr;100:106591. Epub 2022 Mar 5 doi: 10.1016/j.ijsu.2022.106591. PMID: 35259522
Kimura Disease Presenting with Buccal Mass: A Case Report and Literature Review.
Sato R, Bandoh N, Goto T, Ichikawa H, Uemura A, Suzuki S, Yamaguchi T, Aimono E, Nishihara H, Katada A, Harabuchi Y
Head Neck Pathol 2021 Jun;15(2):657-662. Epub 2020 Jul 25 doi: 10.1007/s12105-020-01206-3. PMID: 32712881Free PMC Article

Recent systematic reviews

Treatment algorithm for Kimura's disease: A systematic review and meta-analysis of treatment modalities and prognostic predictors.
Lee CC, Feng IJ, Chen YT, Weng SF, Chan LP, Lai CS, Lin SD, Kuo YR
Int J Surg 2022 Apr;100:106591. Epub 2022 Mar 5 doi: 10.1016/j.ijsu.2022.106591. PMID: 35259522
Kimura Disease: A Case Series and Systematic Review of Clinico-radiological Features.
Sangwan A, Goyal A, Bhalla AS, Kumar A, Sharma R, Arava S, Dawar R
Curr Probl Diagn Radiol 2022 Jan-Feb;51(1):130-142. Epub 2020 Nov 17 doi: 10.1067/j.cpradiol.2020.10.003. PMID: 33250297
The role of radiotherapy in Kimura's disease: a multicenter systematic review of literature.
Fionda B, Loperfido A, Bussu F, Lancellotta V, Casà C, Vavassori A, Vicenzi L, Re A, Deodato F, Morganti AG, Gambacorta MA, Tagliaferri L
Eur Rev Med Pharmacol Sci 2021 Jun;25(12):4205-4210. doi: 10.26355/eurrev_202106_26124. PMID: 34227087

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