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Cogan’s Syndrome and Other Ocular Vasculitides

  • Vasculitis (LR Espinoza, Section Editor)
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Abstract

The clinical presentation of Cogan’s syndrome has been classified as typical and atypical. Like other forms of ocular vasculitis, Cogan’s syndrome has been found to have autoimmune origins with antibodies against the cornea, inner ear, and endothelial antigens. Antineutrophil cytoplasmic antibody (ANCA) and rheumatoid factor (RF) have been associated with Cogan’s syndrome as well as ocular-involving vasculitides not as strongly associated with the audiovestibular manifestations such as granulomatosis with polyangiitis and rheumatoid arthritis. The mainstay of therapy has been corticosteroids although other methods have been described in recalcitrant disease and to prevent development of systemic sequelae.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. Cogan DG. Syndrome of non-syphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthalmol. 1945;33:144–9.

    Article  Google Scholar 

  2. Haynes BF, Kaiser-Kupfer MI, Mason P, Fauci AS. Cogan syndrome: studies in thirteen patients, long-term follow-up, and a review of the literature. Medicine. 1980;59:426–41.

    Article  CAS  PubMed  Google Scholar 

  3. Vollersten R. Vasculitis and Cogan’s syndrome. Rheum Dis Clin N Am. 1990;16:433–8.

    Google Scholar 

  4. Lunardi C, Bason C, Leandri M, et al. Autoantibodies to inner ear and endothelial antigens in Cogan’s syndrome. Lancet. 2002;360:915–21.

    Article  CAS  PubMed  Google Scholar 

  5. Van Doornum S, McColl G, Walter M, et al. Prolonged prodrome, systemic vasculitis, and deafness in Cogan’s syndrome. Ann Rheum Dis. 2001;60:69–71.

    Article  PubMed Central  PubMed  Google Scholar 

  6. Cheson BD, Bluming AZ, Alroy J. Cogan’s syndrome: a systemic vasculitis. Am J Med. 1976;60:549–55.

    Article  CAS  PubMed  Google Scholar 

  7. Grasland A, Pouchot J, Hachulla E, et al. Typical and atypical Cogan’s syndrome: 32 cases and review of the literature. Rheumatology. 2004;43:1007–15.

    Article  CAS  PubMed  Google Scholar 

  8. Branislava I, Marijana T, Nemanja D, et al. Atypical Cogan’s syndrome associated with coronary disease. Chin Med J. 2011;124:3192–4.

    PubMed  Google Scholar 

  9. Gaubitz M, Lubben B, Seidel M, et al. Cogan’s syndrome: Organ-specific autoimmune disease or systemic vasculitis? A report of two cases and review of the literature. Clin Exp Rheumatol. 2001;19:463–9.

    CAS  PubMed  Google Scholar 

  10. Gasparovic H, Djuric Z, Bosnic D, et al. Aortic root vasculitis associated with Cogan’s syndrome. Ann Thorac Surg. 2011;92:340–1.

    Article  PubMed  Google Scholar 

  11. Hautefort C, Loundon N, Montchilova M, et al. Mycophenolate mofetil as a treatment of steroid dependent Cogan’s syndrome in childhood. Int J Pediatr Otorhinolaryngol. 2009;73:1477–9.

    Article  PubMed  Google Scholar 

  12. Lepur D, Vranjican Z, Himbele J, et al. Chlamydia pneumonia infection as a trigger for a Cogan’s syndrome. J Infect. 2006;52:223–6.

    Article  PubMed  Google Scholar 

  13. Pagnini I, Zannin ME, Vittadello F, et al. Clinical features and outcome of Cogan Syndrome. J Pediatr. 2012;160:303–7. Review of the pediatric implications of Cogan syndrome with focus on different manifestations in children with major impact on function.

    Article  PubMed  Google Scholar 

  14. Gluth MB, Baratz KH, Matteson EL, Driscoll CL. Cogan syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc. 2006;81:483–8.

    Article  PubMed  Google Scholar 

  15. Darougar S, John AC, Viswalingam M, et al. Isolation of Chlamydia psittaci from a patient with interstitial keratitis and uveitis associated with ontological and cardiovascular lesions. Br J Ophthalmol. 1978;62:709–14.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  16. Ljungstrom L, Franzen C, Schlaug M, et al. Reinfection with Chlamydia pneumonia may induce isolated and systemic vasculitis in small and large vessels. Scand J Infect Dis Suppl. 1997;104:37–40.

    CAS  PubMed  Google Scholar 

  17. Laitinen K, Laurila A, Pyhala L, Leinonen M, Saikku P. Chlamydia pneumoniae infection induces inflammatory changes in the aortas of rabbits. Infect Immun. 1997;65:4832–5.

    PubMed Central  CAS  PubMed  Google Scholar 

  18. Helmchen C, Arbusow V, Jager L, et al. Cogan’s syndrome: clinical significance of antibodies against the inner ear and cornea. Acta Otolaryngol (Stockh). 1999;119:528–36.

    Article  CAS  Google Scholar 

  19. Hughes GB, Kinney SE, Barna BP, et al. Autoimmune reactivity in Cogan’s syndrome: a preliminary report. Otolaryngol Head Neck Surg. 1983;91:24–32.

    CAS  PubMed  Google Scholar 

  20. Majoor MH, Albers FW, van der Gaag R, et al. Corneal autoimmunity in Cogan’s syndrome? Report of two cases. Ann Otol Rhinol Laryngol. 1992;101:679–84.

    Article  CAS  PubMed  Google Scholar 

  21. Garcia Callejo FJ, Costa Alcacer I, Blay Galaud L, et al. Inner ear autoimmune disorder. Cogan’s syndrome. An Esp Pediatr. 2001;55:87–91.

    Article  CAS  PubMed  Google Scholar 

  22. Bonaguri C, Orsoni J, Russo A, et al. Cogan’s Syndrome: Anti-Hsp70 Antibodies are a serological marker in the typical form. IMAJ. 2014;16:285–8. Important autoantibody finding that may serve as a serological marker for typical Cogan’s syndrome.

    PubMed  Google Scholar 

  23. Ikeda M, Okazaki H, Minota S. Cogan’s syndrome with antineutrophil cytoplasmic autoantibody. Ann Rheum Dis. 2002;61:761–2.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  24. Tarney CM, Wilson K, Sewell MF. Cogan Syndrome in pregnancy. Obstet Gynecol. 2014;124:428–31.

    Article  PubMed  Google Scholar 

  25. Tervaert JW, Mulder L, Stegeman C, et al. Occurrence of autoantibodies to human leucocyte elastase in Wegener’s granulomatosis and other inflammatory disorders. Ann Rheum Dis. 1993;52:115–20.

    Article  PubMed Central  CAS  Google Scholar 

  26. Yamanishi Y, Ishioka S, Takeda M, et al. Atypical Cogan’s syndrome associated with antineutrophil cytoplasmic autoantibodies. Br J Rheumatol. 1996;35:601–3.

    Article  CAS  Google Scholar 

  27. Suzuki M, Arimura Y, Minoshima S, et al. A case of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA)-related glomerulonephritis associated with Cogan’s syndrome. Nippon Jinzo Gakkai Shi. 1996;38:423–7.

    CAS  PubMed  Google Scholar 

  28. Brijker F, Magee CC, Tervaert J, et al. Outcome analysis of patients with vasculitis associated with antineutrophil cytoplasmic antibodies. Clin Nephrol. 1999;52:344–51.

    CAS  PubMed  Google Scholar 

  29. Lee LY, Akhtar MM, Kirresh O, Gibson T: Interstitial keratitis and sensorineural hearing loss as a manifestation of rheumatoid arthritis: clinical lessons from a rare complication. BMJ Case Rep 2012.

  30. Harper SL, Letko E, Samson CM, et al. Wegener’s Granulomatosis: the relationship between ocular and systemic disease. J Rheumatol. 2001;28:1025–32.

    CAS  PubMed  Google Scholar 

  31. Bakthavachalam S, Driver MS, Cox C, et al. Hearing loss in Wegener’s granulomatosis. Otol Neurotol. 2004;25:833–7.

    Article  PubMed  Google Scholar 

  32. Sriskandarajah V, Bansal RA, Yeoh R, Bansal AS. Early intervention in localized Wegener’s granulomatosis with sensorineural hearing loss preserves hearing. Am J Audiol. 2012;21:121–6.

    Article  PubMed  Google Scholar 

  33. Gran JT, Nordvag BY, Storesund B. An overlap syndrome with features of atypical Cogan syndrome and Wegener’s granulomatosis. Scand J Rheumatol. 1999;28:62–4.

    Article  CAS  PubMed  Google Scholar 

  34. Finkielman JD, Lee AS, Hummel AM, et al. ANCA are detectable in nearly all patients with active severe Wegener’s granulomatosis. Am J Med. 2007;120:643.e9–14.

    Article  Google Scholar 

  35. Zlatanovic G, Veselinovic D, Cekic S, et al. Ocular manifestation of rheumatoid arthritis-different forms and frequency. Bosn J Basic Med Sci. 2010;10:323–7.

    PubMed  Google Scholar 

  36. Fujita M, Igarachi T, Kurai T, et al. Correlation between dry eye and rheumatoid arthritis activity. Am J Ophthalmol. 2005;140:808–13.

    Article  PubMed  Google Scholar 

  37. Knox Cartwright NE, Tole DM, Georgoudis P, Cook SD. Peripheral ulcerative keratitis and corneal melt: a 10-year single center review with historical comparison. Cornea. 2014;33:27–31. Important review showing that current therapeutic interventions are improving outcomes in immune mediated ocular disease.

    Article  PubMed  Google Scholar 

  38. Takatsu M, Higaki M, Kinoshita H, et al. Ear involvement in patients with Rheumatoid Arthritis. Otol Neurotol. 2005;26:755–61.

    Article  PubMed  Google Scholar 

  39. Pascual-Ramos V, Contreras-Yanez I, Enriquez L, et al. Hearing impairment in a tertiary-care-level population of Mexican rheumatoid arthritis patients. J Clin Rheumatol. 2012;18:393–8.

    Article  Google Scholar 

  40. Pascual-Ramos V, Contreras-Yanez I, Rivera-Hoyos P, et al. Cumulative disease activity predicts incidental hearing impairment in patient with rheumatoid arthritis. Clin Rheumatol. 2014;33:315–21.

    Article  PubMed  Google Scholar 

  41. Deane KD. Preclinical rheumatoid arthritis (autoantibodies): an updated review. Curr Rheumatol Rep. 2014;16:419.

    Article  PubMed  Google Scholar 

  42. Kessel A, Vadasz Z, Toubi E. Cogan syndrome—Pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 2014;13:351–4. A nice review of Cogan’s syndrome stressing more of the plethora of systemic findings associated with the disease.

    Article  PubMed  Google Scholar 

  43. Beccastrini E, Emmi G, Squatrito D, et al. Infliximab and Cogan’s syndrome. Clin Otolaryngol. 2010;35:441–2.

    Article  CAS  PubMed  Google Scholar 

  44. Shahid FL, Mukherjee R, Knapp C. Cogan’s Syndrome associated with orbital inflammation. Orbit. 2013;32:206–7.

    Article  PubMed  Google Scholar 

  45. Shibuya M, Fijio K, Morita K, et al. Successful treatment with tocilizumab in a case of Cogan’s syndrome complicated with aortitis. Mod Rheumatol. 2013;23:577–81.

    Article  PubMed  Google Scholar 

  46. Bovo R, Ciorba A, Trevisi P, et al. Cochlear implant in Cogan syndrome. Acta Otolaryngol (Stockh). 2011;131:494–7.

    Article  Google Scholar 

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Gabriela M. Espinoza and Angela Prost declare that they have no conflicts of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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Correspondence to Gabriela M. Espinoza.

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This article is part of the Topical Collection on Vasculitis

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Espinoza, G.M., Prost, A. Cogan’s Syndrome and Other Ocular Vasculitides. Curr Rheumatol Rep 17, 24 (2015). https://doi.org/10.1007/s11926-015-0499-y

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  • DOI: https://doi.org/10.1007/s11926-015-0499-y

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