Skip to main content
SpringerLink
Log in
Book cover

Systemic Vasculitis pp 123–135Cite as

Cogan’s Syndrome

  • Chapter
  • First Online:

  • 1848 Accesses

Part of the book series: Medical Radiology ((Med Radiol Diagn Imaging))

Abstract

Cogan’s syndrome is thought to be an autoimmune disease of uncertain etiology which causes inflammation of the eye, most typically interstitial kcratitis, and hearing loss. It typically occurs in the third and fourth decades of life. About half of patients have systemic disease manifestations, including constitutional symptoms of fever and weight loss, arthritis, neuropathy, rash, pericarditis, pleuritis, and vasculitis. The vasculitis typically affects large vessels, particularly the aorta and other large vessels, but may also affect medium and small vessels as well. Vascular complications may lead to limb claudication and aortic aneurysm aortic heart disease and failure of other involved organs. Vasular imaging, which can include angiography using conventional imaging computed tomography, ultrasound, magnetic resonance scanning, and use of positron emission tomography, is required to assess the extent and activity vascular involvement. Treatment is directed to the involved organs, and can include glucocorticosteroids, cytotoxic agents, and vascular repair.

This is a preview of subscription content, log in via an institution.

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   249.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   349.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  • Albayram MS, Wityk R, Yousem DM et al (2001) The cerebral angiographic findings in Cogan syndrome. AJNR Am J Neuroradiol 22(4):751–754

    PubMed  CAS  Google Scholar 

  • Allen NB, Cox CC, Cobo M et al (1990) Use of immunosuppressive agents in the treatment of severe ocular and vascular manifestations of Cogan’s syndrome. Am J Med 88(3):296–301

    Article  PubMed  CAS  Google Scholar 

  • Balink H, Bruyn GA (2007) The role of PET/CT in Cogan’s syndrome. Clin Rheumatol 26(12):2177–2179

    Article  PubMed  CAS  Google Scholar 

  • Bastug DE, Dominic A, Ortiz O et al (1997) Popliteal artery thrombosis in a patient with Cogan syndrome: treatment with thrombolysis and percutaneous transluminal angioplasty. Cardiovasc Intervent Radiol 20(1):57–59

    Article  PubMed  CAS  Google Scholar 

  • Benitez JT, Arsenault MD, Licht JM et al (1990) Evidence of central vestibulo-auditory dysfunction in atypical Cogan’s syndrome: a case report. Am J Otol 11(2):131–134

    Article  PubMed  CAS  Google Scholar 

  • Bisdas TE, Teebken OE, Wilhelmi M et al (2009) Surgical treatment of a thoracoabdominal aneurysm in Cogan’s syndrome. Ann Thorac Surg 88(5):1668–1670

    Article  PubMed  Google Scholar 

  • Casselman JW, Majoor MH, Albers FW (1994) MR of the inner ear in patients with Cogan syndrome. AJNR Am J Neuroradiol 15(1):131–138

    PubMed  CAS  Google Scholar 

  • Cochrane AD, Tatoulis J (1991) Cogan’s syndrome with aortitis, aortic regurgitation, and aortic arch vessel stenoses. Ann Thorac Surg 52(5):1166–1167

    Article  PubMed  CAS  Google Scholar 

  • Cogan DG (1945) Syndrome of nonsyphilitic interstitial keratitis and vestibuloauditory symptoms. Arch Ophthal 33:144–149

    Google Scholar 

  • Eisenstein B, Taubenhaus M (1958) Nonsyphilitic interstitial keratitis and bilateral deafness (Cogan’s syndrome) associated with cardiovascular disease. N Engl J Med 258(22):1074–1079

    Article  PubMed  CAS  Google Scholar 

  • Fisher ER, Hellstrom HR (1961) Cogan’s syndrome and systemic vascular disease. Analysis of pathologic features with reference to its relationship to thromboangiitis obliterans (Buerger). Arch Pathol 72:572–592

    PubMed  CAS  Google Scholar 

  • Garcia Berrocal JR, Vargas JA, Vaquero M et al (1999) Cogan’s syndrome: an oculo-audiovestibular disease. Postgrad Med J 75(883):262–264

    PubMed  CAS  Google Scholar 

  • Gaubitz M, Lubben B, Seidel M et al (2001) Cogan’s syndrome: organ-specific autoimmune disease or systemic vasculitis? A report of two cases and review of the literature. Clin Exp Rheumatol 19(4):463–469

    PubMed  CAS  Google Scholar 

  • Gloddek B, Gloddek J, Arnold W (1999) A rat T-cell line that mediates autoimmune disease of the inner ear in the Lewis rat. ORL J Otorhinolaryngol Relat Spec 61(4):181–187

    Article  PubMed  CAS  Google Scholar 

  • Gluth MB, Baratz KH, Matteson EL et al (2006) Cogan syndrome: a retrospective review of 60 patients throughout a half century. Mayo Clin Proc 81(4):483–488

    Article  PubMed  Google Scholar 

  • Grasland A, Pouchot J, Hachulla E et al (2004) Typical and atypical Cogan’s syndrome: 32 cases and review of the literature. Rheumatology (Oxford) 43(8):1007–1015

    Article  CAS  Google Scholar 

  • Harris JP, Weisman MH, Derebery JM et al (2003) Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trial. Jama 290(14):1875–1883

    Article  PubMed  CAS  Google Scholar 

  • Haynes BF, Kaiser-Kupfer MI, Mason P et al (1980) Cogan syndrome: studies in thirteen patients, long-term follow-up, and a review of the literature. Med (Baltimore) 59(6):426–441

    CAS  Google Scholar 

  • Haynes BF, Pikus A, Kaiser-Kupfer M et al (1981) Successful treatment of sudden hearing loss in Cogan’s syndrome with corticosteroids. Arthr Rheum 24(3):501–503

    Article  CAS  Google Scholar 

  • Helmchen C, Arbusow V, Jager L et al (1999) Cogan’s syndrome: clinical significance of antibodies against the inner ear and cornea. Acta Otolaryngol 119(5):528–536

    Article  PubMed  CAS  Google Scholar 

  • Ho AC, Roat MI, Venbrux A et al (1999) Cogan’s syndrome with refractory abdominal aortitis and mesenteric vasculitis. J Rheumatol 26(6):1404–1407

    PubMed  CAS  Google Scholar 

  • Hughes GB, Barna BP, Kinney SE et al (1988) Clinical diagnosis of immune inner-ear disease. Laryngoscope 98(3):251–253

    Article  PubMed  CAS  Google Scholar 

  • Ikeda M, Okazaki H, Minota S (2002) Cogan’s syndrome with antineutrophil cytoplasmic autoantibody. Ann Rheum Dis 61(8):761–762

    Article  PubMed  CAS  Google Scholar 

  • Jansen M, Salch S, Bolster M et al (2010) Thoracic vasculitis presenting as surgical problems. Virchows Archive 456:91–96

    Article  Google Scholar 

  • Kondo Y, Ito S, Ohi Y et al (2009) Atypical Cogan’s syndrome with aortitis. Intern Med 48(12):1093–1097

    Article  PubMed  Google Scholar 

  • Low AH, Su JW, Sin KY et al (2007) Cogan’s syndrome with recurrent carotid and aortic aneurysms: a potentially fatal disorder mimicking Marfan syndrome. Scand J Rheumatol 36(1):71–73

    Article  PubMed  CAS  Google Scholar 

  • Lunardi C, Bason C, Leandri M et al (2002) Autoantibodies to inner ear and endothelial antigens in Cogan’s syndrome. Lancet 360(9337):915–921

    Article  PubMed  CAS  Google Scholar 

  • Majoor MH, Albers FW, Casselman JW (1993) Clinical relevance of magnetic resonance imaging and computed tomography in Cogan’s syndrome. Acta Otolaryngol 113(5):625–631

    Article  PubMed  CAS  Google Scholar 

  • Matteson EL, Choi HK, Poe DS et al (2005) Etanercept therapy for immune-mediated cochleovestibular disorders: a multi-center, open-label, pilot study. Arthr Rheum 53(3):337–342

    Article  CAS  Google Scholar 

  • Mazlumzadeh M, Matteson EL (2007) Cogan’s syndrome: an audiovestibular, ocular, and systemic autoimmune disease. Rheum Dis Clin North Am 33(4):855–874 vii-viii

    Article  PubMed  Google Scholar 

  • Mazlumzadeh M, Lowe VJ, Mullan BP et al (2003) The utility of positron emission tomography in the evaluation of autoimmune hearing loss. Otol Neurotol 24(2):201–204

    Article  PubMed  Google Scholar 

  • McCabe BF (1979) Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol 88(5 Pt 1):585–589

    PubMed  CAS  Google Scholar 

  • McCallum RM (1993) Cogan’s syndrome. In: Frauenfelder FT, Hampton R (eds) Current ocular therapy, 4th edn. WB Saunders, Philadelphia, p 410

    Google Scholar 

  • McCallum RM, Haynes BF (1996) Cogan’s syndrome. In: Pepose JS, Holland GN, Wilhelmus KR (eds) Ocular infection and immunity, 1st edn. Mosby, St. Louis, p 446

    Google Scholar 

  • McCallum RM, Allen NB, Cobo LM et al (1992) Cogan’s syndrome: clinical features and outcomes [abstract]. Arthr Rheum 35(Suppl):S51

    Google Scholar 

  • McDonald TJ, Vollertsen RS, Younge BR (1985) Cogan’s syndrome: audiovestibular involvement and prognosis in 18 patients. Laryngoscope 95(6):650–654

    Article  PubMed  CAS  Google Scholar 

  • Negroni L, Tiberio G (1969) Cogan’s syndrome. Riv Otoneurooftalmol 44(3):199–224

    PubMed  CAS  Google Scholar 

  • Ochonisky S, Chosidow O, Kuentz M et al (1991) Cogan’s syndrome. An unusual etiology of urticarial vasculitis. Dermatol 183(3):218–220

    Article  CAS  Google Scholar 

  • Orsoni JG, Zavota L, Vincenti V et al (2004) Cogan syndrome in children: early diagnosis and treatment is critical to prognosis. Am J Ophthalmol 137(4):757–758

    PubMed  Google Scholar 

  • Pasanisi E, Vincenti V, Bacciu A et al (2003) Cochlear implantation and Cogan syndrome. Otol Neurotol 24(4):601–604

    Article  PubMed  Google Scholar 

  • Quaranta N, Bartoli R, Giagnotti F et al. (2002) Cochlear implants in systemic autoimmune vasculitis syndromes. Acta Otolaryngol Suppl(548): 44–8

    Google Scholar 

  • Rarey KE, Bicknell JM, Davis LE (1986) Intralabyrinthine osteogenesis in Cogan’s syndrome. Am J Otolaryngol 7(6):387–390

    Article  PubMed  CAS  Google Scholar 

  • Raza K, Karokis D, Kitas GD (1998) Cogan’s syndrome with Takayasu’s arteritis. Br J Rheumatol 37(4):369–372

    Article  PubMed  CAS  Google Scholar 

  • Sandner A, Neumann K, Kosling S, et al. (2009) [Cogan’s disease: report of two cases.]. Hno

    Google Scholar 

  • Schuknecht HF, Nadol B Jr (1994) Temporal bone pathology in a case of Cogan’s syndrome. Laryngoscope 104(9):1135–1142

    Article  PubMed  CAS  Google Scholar 

  • Shimura M, Yasuda K, Fuse N et al (2000) Effective treatment with topical cyclosporin A of a patient with Cogan syndrome. Ophthalmol 214(6):429–432

    Article  CAS  Google Scholar 

  • Stone JH, Francis HW (2000) Immune-mediated inner ear disease. Curr Opin Rheumatol 12(1):32–40

    Article  PubMed  CAS  Google Scholar 

  • Thomas HG (1992) Case report: clinical and radiological features of Cogan’s syndrome–non-syphilitic interstitial keratitis, audiovestibular symptoms and systemic manifestations. Clin Radiol 45(6):418–421

    Article  PubMed  CAS  Google Scholar 

  • Tseng JF, Cambria RP, Aretz HT et al (1999) Thoracoabdominal aortic aneurysm in Cogan’s syndrome. J Vasc Surg 30(3):565–568

    Article  PubMed  CAS  Google Scholar 

  • Udayaraj UP, Hand MF, Shilliday IR et al (2004) Renal involvement in Cogan’s syndrome. Nephrol Dial Transpl 19(9):2420–2421

    Article  Google Scholar 

  • Vella JP, O’Callaghan J, Hickey D et al (1997) Renal artery stenosis complicating Cogan’s syndrome. Clin Nephrol 47(6):407–408

    PubMed  CAS  Google Scholar 

  • Verhagen C, Mor F, Kipp JB et al (1999) Experimental autoimmune keratitis induced in rats by anti-cornea T-cell lines. Invest Ophthalmol Vis Sci 40(10):2191–2198

    PubMed  CAS  Google Scholar 

  • Vollertsen RS, McDonald TJ, Younge BR et al (1986) Cogan’s syndrome: 18 cases and a review of the literature. Mayo Clin Proc 61(5):344–361

    PubMed  CAS  Google Scholar 

  • Weyn T, Haine S, Conraads V (2009) Cogan’s syndrome with left main coronary artery occlusion. Cardiol J 16(6):573–576

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Rheumatology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN, 55905, USA

    Eric L. Matteson

  2. Department of Radiology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN, 55905, USA

    Anthony W. Stanson

Authors
  1. Eric L. Matteson
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Anthony W. Stanson
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Eric L. Matteson .

Editor information

Editors and Affiliations

  1. Medical School of Tunis, Department of Medical Imaging, Hôpital Mongi Slim, La Marsa, 2046, Tunisia

    Lotfi Hendaoui

  2. Mayo Graduate School of Medicine, Gonda Vascular Center, Mayo Clinic, Rochester, 55902, Minnesota, USA

    Anthony W. Stanson

  3. Medical School of Tunis, Department of Medical Imaging, Interior Security Forces Teaching Hospit, La Marsa, 2070, Tunisia

    M. Habib Bouhaouala

  4. Medical School of Toulouse University, Department of Medical Imaging, Rangueil Hospital, Toulouse CX 9, 31059, France

    Francis Joffre

Rights and permissions

Reprints and permissions

Copyright information

© 2011 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Matteson, E.L., Stanson, A.W. (2011). Cogan’s Syndrome. In: Hendaoui, L., Stanson, A., Bouhaouala, M., Joffre, F. (eds) Systemic Vasculitis. Medical Radiology(). Springer, Berlin, Heidelberg. https://doi.org/10.1007/174_2011_148

Download citation

  • DOI: https://doi.org/10.1007/174_2011_148

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-02483-2

  • Online ISBN: 978-3-642-02484-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation