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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
Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases.
Stone JR, Bruneval P, Angelini A, Bartoloni G, Basso C, Batoroeva L, Buja LM, Butany J, d'Amati G, Fallon JT, Gittenberger-de Groot AC, Gouveia RH, Halushka MK, Kelly KL, Kholova I, Leone O, Litovsky SH, Maleszewski JJ, Miller DV, Mitchell RN, Preston SD, Pucci A, Radio SJ, Rodriguez ER, Sheppard MN, Suvarna SK, Tan CD, Thiene G, van der Wal AC, Veinot JP
Cardiovasc Pathol 2015 Sep-Oct;24(5):267-78. Epub 2015 May 16 doi: 10.1016/j.carpath.2015.05.001. PMID: 26051917
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

Recent clinical studies

Etiology

Paediatric Cogan Syndrome masquerading as IgA vasculitis.
Ramani PK, Grigorian F, Lightle H, Joshi SV
Mod Rheumatol Case Rep 2023 Dec 29;8(1):229-236. doi: 10.1093/mrcr/rxad060. PMID: 37902167
Spectrum and Outcome of Noninfectious Aortitis.
Ferfar Y, Morinet S, Espitia O, Agard C, Vautier M, Comarmond C, Desbois AC, Domont F, Resche-Rigon M, Cacoub P, Biard L, Saadoun D; French Study Group for Large Vessel Vasculitis (GEFA)
J Rheumatol 2021 Oct;48(10):1583-1588. Epub 2021 Jul 1 doi: 10.3899/jrheum.201274. PMID: 34210830
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'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
Clinical features and outcome of Cogan syndrome.
Pagnini I, Zannin ME, Vittadello F, Sari M, Simonini G, Cimaz R, Zulian F
J Pediatr 2012 Feb;160(2):303-307.e1. Epub 2011 Sep 13 doi: 10.1016/j.jpeds.2011.07.051. PMID: 21920537

Diagnosis

Selected Otologic Disorders Causing Dizziness.
Ishiyama G
Continuum (Minneap Minn) 2021 Apr 1;27(2):468-490. doi: 10.1212/CON.0000000000000977. PMID: 34351115
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
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 and Behcet syndromes.
Singer O
Rheum Dis Clin North Am 2015;41(1):75-91, viii. doi: 10.1016/j.rdc.2014.09.007. PMID: 25399941

Therapy

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 and other ocular vasculitides.
Espinoza GM, Prost A
Curr Rheumatol Rep 2015 Apr;17(4):24. doi: 10.1007/s11926-015-0499-y. PMID: 25854487
Cogan and Behcet syndromes.
Singer O
Rheum Dis Clin North Am 2015;41(1):75-91, viii. doi: 10.1016/j.rdc.2014.09.007. PMID: 25399941
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
Cogan syndrome.
Orsoni JG, Zavota L, Pellistri I, Piazza F, Cimino L
Cornea 2002 May;21(4):356-9. doi: 10.1097/00003226-200205000-00005. PMID: 11973382

Prognosis

Paediatric Cogan´s syndrome - review of literature, case report and practical approach to diagnosis and management.
Rücklová K, von Kalle T, Koitschev A, Gekeler K, Scheltdorf M, Heinkele A, Blankenburg F, Kötter I, Hospach A
Pediatr Rheumatol Online J 2023 Jun 8;21(1):54. doi: 10.1186/s12969-023-00830-x. PMID: 37291629Free PMC Article
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
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
Ocular inflammation in neurorheumatic disease.
Vodopivec I, Lobo AM, Prasad S
Semin Neurol 2014 Sep;34(4):444-57. Epub 2014 Nov 4 doi: 10.1055/s-0034-1390393. PMID: 25369440
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

Clinical prediction guides

The genetic spectrum of congenital ocular motor apraxia type Cogan: an observational study, continued.
Schröder S, Yigit G, Li Y, Altmüller J, Büttel HM, Fiedler B, Kretzschmar C, Nürnberg P, Seeger J, Serpieri V, Valente EM, Wollnik B, Boltshauser E, Brockmann K
Orphanet J Rare Dis 2023 May 2;18(1):101. doi: 10.1186/s13023-023-02706-5. PMID: 37131188Free PMC Article
Spectrum and Outcome of Noninfectious Aortitis.
Ferfar Y, Morinet S, Espitia O, Agard C, Vautier M, Comarmond C, Desbois AC, Domont F, Resche-Rigon M, Cacoub P, Biard L, Saadoun D; French Study Group for Large Vessel Vasculitis (GEFA)
J Rheumatol 2021 Oct;48(10):1583-1588. Epub 2021 Jul 1 doi: 10.3899/jrheum.201274. PMID: 34210830
Clinical Presentation of Ataxia-Telangiectasia.
Alyasin S, Esmaeilzadeh H, Ebrahimi N, Nabavizadeh SH, Nemati H
Arch Iran Med 2019 Dec 1;22(12):682-686. PMID: 31823618
Autoimmune Inner Ear Disease: Immune Biomarkers, Audiovestibular Aspects, and Therapeutic Modalities of Cogan's Syndrome.
Shamriz O, Tal Y, Gross M
J Immunol Res 2018;2018:1498640. Epub 2018 Apr 23 doi: 10.1155/2018/1498640. PMID: 29850616Free PMC Article
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

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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