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Cogan syndrome

MedGen UID:
82871
•Concept ID:
C0271270
•
Disease or Syndrome
Synonym: Cogan's syndrome
SNOMED CT: Cogan's syndrome (405810005); Oculovestibuloauditory syndrome (405810005); Cogan syndrome (405810005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0015453
Orphanet: ORPHA1467

Definition

A rare inflammatory/autoimmune disorder of unknown origin characterized by interstitial keratitis (IK) and audiovestibular dysfunctions. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCogan syndrome

Professional guidelines

PubMed

Cogan syndrome: Characteristics, outcome and treatment in a French nationwide retrospective study and literature review.
Durtette C, Hachulla E, Resche-Rigon M, Papo T, Zénone T, Lioger B, Deligny C, Lambert M, Landron C, Pouchot J, Kahn JE, Lavigne C, De Wazieres B, Dhote R, Gondran G, Pertuiset E, Quemeneur T, Hamidou M, Sève P, Le Gallou T, Grasland A, Hatron PY, Fain O, Mekinian A; SNFMI and CRI
Autoimmun Rev 2017 Dec;16(12):1219-1223. Epub 2017 Oct 14 doi: 10.1016/j.autrev.2017.10.005. PMID: 29037902
Cogan syndrome--pathogenesis, clinical variants and treatment approaches.
Kessel A, Vadasz Z, Toubi E
Autoimmun Rev 2014 Apr-May;13(4-5):351-4. Epub 2014 Jan 10 doi: 10.1016/j.autrev.2014.01.002. PMID: 24418297
New treatment strategies in large-vessel vasculitis.
Unizony S, Stone JH, Stone JR
Curr Opin Rheumatol 2013 Jan;25(1):3-9. doi: 10.1097/BOR.0b013e32835b133a. PMID: 23114585

Recent clinical studies

Etiology

Rare primary vasculitis: update on multiple complex diseases and the new kids on the block.
Dantas JG, Biegelmeyer E, Zarur EB, Pinheiro FAG
Adv Rheumatol 2024 Oct 9;64(1):79. doi: 10.1186/s42358-024-00421-8. PMID: 39385260
Autosomal Recessive Cerebellar Ataxias With Elevated Alpha-Fetoprotein: Uncommon Diseases, Common Biomarker.
Renaud M, Tranchant C, Koenig M, Anheim M
Mov Disord 2020 Dec;35(12):2139-2149. Epub 2020 Oct 12 doi: 10.1002/mds.28307. PMID: 33044027
Endovascular treatment of multiple aneurysms complicating Cogan syndrome.
Angiletta D, Wiesel P, Pulli R, Marinazzo D, Bortone AS, Regina G
Ann Vasc Surg 2015 Feb;29(2):361.e9-361.e12. Epub 2014 Nov 4 doi: 10.1016/j.avsg.2014.08.016. PMID: 25462550
Otolaryngologic manifestations of systemic vasculitis.
Parra-García GD, Callejas-Rubio JL, Ríos-Fernández R, Sainz-Quevedo M, Ortego-Centeno N
Acta Otorrinolaringol Esp 2012 Jul-Aug;63(4):303-10. Epub 2011 Dec 15 doi: 10.1016/j.otorri.2011.09.002. PMID: 22177421
Otolaryngological manifestations of rheumatic diseases in children.
Zulian F, Sari M, de Filippis C
Int J Pediatr Otorhinolaryngol 2009 Dec;73 Suppl 1:S56-60. doi: 10.1016/S0165-5876(09)70011-4. PMID: 20114157

Diagnosis

Cogan's Syndrome: Clinical Presentations and Update on Treatment.
Espinoza GM, Wheeler J, Temprano KK, Keller AP
Curr Allergy Asthma Rep 2020 Jun 16;20(9):46. doi: 10.1007/s11882-020-00945-1. PMID: 32548646
Interstitial keratitis diagnosis and treatment.
Gauthier AS, Noureddine S, Delbosc B
J Fr Ophtalmol 2019 Jun;42(6):e229-e237. Epub 2019 May 15 doi: 10.1016/j.jfo.2019.04.001. PMID: 31103357
Imaging spectrum of CNS vasculitis.
Abdel Razek AA, Alvarez H, Bagg S, Refaat S, Castillo M
Radiographics 2014 Jul-Aug;34(4):873-94. doi: 10.1148/rg.344135028. PMID: 25019429
Ocular manifestations of systemic inflammatory diseases.
Mohsenin A, Huang JJ
Conn Med 2012 Oct;76(9):533-44. PMID: 23155672
Otolaryngologic manifestations of systemic vasculitis.
Parra-García GD, Callejas-Rubio JL, Ríos-Fernández R, Sainz-Quevedo M, Ortego-Centeno N
Acta Otorrinolaringol Esp 2012 Jul-Aug;63(4):303-10. Epub 2011 Dec 15 doi: 10.1016/j.otorri.2011.09.002. PMID: 22177421

Therapy

A rare case of atypical Cogan's syndrome presenting as encephalitis.
Maikap D, Pradhan A, Padhan P
Mod Rheumatol Case Rep 2022 Jun 24;6(2):305-308. doi: 10.1093/mrcr/rxab055. PMID: 34957524
Successful treatment of Cogan's syndrome with tocilizumab.
Kougkas N, Bertsias G, Stratoudaki R, Avgoustidis N
Scand J Rheumatol 2021 Jul;50(4):330-331. Epub 2020 Oct 13 doi: 10.1080/03009742.2020.1818822. PMID: 33047628
COGAN'S SYNDROME.
Iliescu DA, Timaru CM, Batras M, De Simone A, Stefan C
Rom J Ophthalmol 2015 Jan-Mar;59(1):6-13. PMID: 27373108Free PMC Article
Rheumatic inflammatory eye diseases of childhood.
Reiff A, Kadayifcilar S, Özen S
Rheum Dis Clin North Am 2013 Nov;39(4):801-32. Epub 2013 Jul 13 doi: 10.1016/j.rdc.2013.05.005. PMID: 24182856
Recommendations of the Italian Society of Rheumatology for the treatment of the primary large-vessel vasculitis with biological agents.
Pipitone N, Olivieri I, Salvarani C; Italian Society of Rheumatology
Clin Exp Rheumatol 2012 Jan-Feb;30(1 Suppl 70):S139-61. Epub 2012 May 11 PMID: 22640657

Prognosis

Rare primary vasculitis: update on multiple complex diseases and the new kids on the block.
Dantas JG, Biegelmeyer E, Zarur EB, Pinheiro FAG
Adv Rheumatol 2024 Oct 9;64(1):79. doi: 10.1186/s42358-024-00421-8. PMID: 39385260
Aortic valve perforation in the setting of Cogan's syndrome.
Beltagy A, Eshak N, Abdelnabi MH, Almaghraby A, Magdy S, Shehata H
Echocardiography 2019 Aug;36(8):1590-1593. Epub 2019 Jun 27 doi: 10.1111/echo.14428. PMID: 31246322
Interstitial keratitis diagnosis and treatment.
Gauthier AS, Noureddine S, Delbosc B
J Fr Ophtalmol 2019 Jun;42(6):e229-e237. Epub 2019 May 15 doi: 10.1016/j.jfo.2019.04.001. PMID: 31103357
Otolaryngological manifestations of rheumatic diseases in children.
Zulian F, Sari M, de Filippis C
Int J Pediatr Otorhinolaryngol 2009 Dec;73 Suppl 1:S56-60. doi: 10.1016/S0165-5876(09)70011-4. PMID: 20114157
Varied clinical spectrum of necrobiotic xanthogranuloma.
Cornblath WT, Dotan SA, Trobe JD, Headington JT
Ophthalmology 1992 Jan;99(1):103-7. doi: 10.1016/s0161-6420(92)32031-7. PMID: 1741120

Clinical prediction guides

Clinical outcomes of epithelial basement membrane dystrophy after keratorefractive lenticule extraction.
Kim BK, Chung YT
J Cataract Refract Surg 2024 Jun 1;50(6):558-564. doi: 10.1097/j.jcrs.0000000000001409. PMID: 38270250Free PMC Article
Integrating visual search, eye movement training and reversing prism exposure in the treatment of Balint-Holmes syndrome: a single case report.
Panico F, Arini A, Cantone P, Crisci C, Trojano L
Top Stroke Rehabil 2022 May;29(4):280-285. Epub 2021 May 7 doi: 10.1080/10749357.2021.1923319. PMID: 33960916
The utility of a normal tear osmolarity test in patients presenting with dry eye disease like symptoms: A prospective analysis.
Brissette AR, Drinkwater OJ, Bohm KJ, Starr CE
Cont Lens Anterior Eye 2019 Apr;42(2):185-189. Epub 2018 Sep 17 doi: 10.1016/j.clae.2018.09.002. PMID: 30236650
Mutational analysis of SYNJ1 gene (PARK20) in Parkinson's disease in a Taiwanese population.
Chen KH, Wu RM, Lin HI, Tai CH, Lin CH
Neurobiol Aging 2015 Oct;36(10):2905.e7-8. Epub 2015 Jun 12 doi: 10.1016/j.neurobiolaging.2015.06.009. PMID: 26149920
Rituximab ameliorated severe hearing loss in Cogan's syndrome: a case report.
Orsoni JG, Laganà B, Rubino P, Zavota L, Bacciu S, Mora P
Orphanet J Rare Dis 2010 Jun 16;5:18. doi: 10.1186/1750-1172-5-18. PMID: 20550723Free PMC Article

Recent systematic reviews

Report of 2 pediatric cases with atypical Cogan's syndrome and a systematic review.
Kasap Cuceoglu M, Basaran O, Batu ED, Kaya Akca U, Atalay E, Sener S, Balik Z, Bayindir Y, Aliyev E, Gocmen R, Kadayifcilar S, Akyol U, Bilginer Y, Ozen S
Int J Rheum Dis 2023 Mar;26(3):544-550. Epub 2022 Dec 11 doi: 10.1111/1756-185X.14531. PMID: 36502531
Cogan's syndrome: State of the art of systemic immunosuppressive treatment in adult and pediatric patients.
Mora P, Calzetti G, Ghirardini S, Rubino P, Gandolfi S, Orsoni J
Autoimmun Rev 2017 Apr;16(4):385-390. Epub 2017 Feb 14 doi: 10.1016/j.autrev.2017.02.009. PMID: 28232169
COGAN'S SYNDROME.
Iliescu DA, Timaru CM, Batras M, De Simone A, Stefan C
Rom J Ophthalmol 2015 Jan-Mar;59(1):6-13. PMID: 27373108Free PMC Article
Cogan's syndrome: an autoimmune inner ear disease.
Greco A, Gallo A, Fusconi M, Magliulo G, Turchetta R, Marinelli C, Macri GF, De Virgilio A, de Vincentiis M
Autoimmun Rev 2013 Jan;12(3):396-400. Epub 2012 Jul 28 doi: 10.1016/j.autrev.2012.07.012. PMID: 22846458

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