Cogan Syndrome Vasculitis

Interstitial Keratitis

  • Diagnosis
  • Algorithms
  • Background
  • Lab Tests
  • References
  • Related Topics

Indications for Testing

Rapid onset of sensorineural hearing loss, eye inflammation, symptoms of vasculitis

Laboratory Testing

  • No formal criteria or confirmatory test for the diagnosis of Cogan syndrome (CS)
  • Nonspecific testing – helpful in excluding other diagnoses
    • Initial assessment
    • Rule out other disease processes
      • Renal disorders – urinalysis, urea nitrogen, creatinine
      • Other vasculitis – antineutrophil cytoplasmic antibody (ANCA)
      • Connective tissue disease – antinuclear antibody (ANA), CBC
        • CBC may reveal anemia, leukocytosis, thrombocytosis
    • Rule out infections associated with hearing loss
      • Treponema pallidum
        • Presence of interstitial keratitis and/or acute sensorineural hearing loss combined with negative testing for syphilis is highly suggestive of CS
      • HIV

Imaging Studies

Angiography – if symptoms present, use to identify aortitis

Differential Diagnosis

Cogan syndrome (CS) is a rare vasculitis that typically manifests as an ophthalmic disorder (interstitial keratitis) and/or an audiovestibular disorder. It is categorized as a variable vessel vasculitis (Chapel Hill, 2012).


  • Incidence – rare; ~250 cases reported (Singer, 2015)
  • Age – young adults in 20s-30s
    • A few cases in children and adults >50 years
  • Sex – M:F, equal
  • Ethnicity – none reported


  • Inflammation in all vessel sizes (small, medium, large)
    • Large vessel disease most common
  • Primary ocular target – small vessels in vascularized layers of anterior globe, episclera, sclera, and uveitis
  • ​Autoantibodies against inner ear and endothelial antigens have been reported (Singer, 2015)
    • Not consistently demonstrated
    • No correlation with disease activity for most patients
    • Antibodies include Anti-Hsp70, antineutrophil cytoplasmic antibody (ANCA) (uncommon)

Clinical Presentation

  • Constitutional – headache, fever, arthralgia, arthritis, weight loss
  • Ophthalmologic – interstitial keratitis, iritis, uveitis, episcleritis, choroiditis, retinal vasculitis
  • Audiovestibular – Ménière-like syndrome (vertigo, tinnitus, ataxia) with or without sudden sensorineural hearing loss; frequently results in deafness
  • Vasculitis – aortitis, aneurysms, glomerulonephritis, mesenteric vasculitis/thrombosis
  • Cardiovascular – coronary arteritis, aortic aneurysms
Tests generally appear in the order most useful for common clinical situations. Click on number for test-specific information in the ARUP Laboratory Test Directory.

ANCA-Associated Vasculitis Profile (ANCA/MPO/PR-3) with Reflex to ANCA Titer 2006480
Method: Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multiplex Bead Assay


Negative antibody testing does not rule out autoimmune liver disease

All interpretation of antibody patterns must be done in conjunction with clinical presentation

There may be overlap between diseases and antibodies detected

No single test shows absolute specificity

Anti-Neutrophil Cytoplasmic Antibody with Reflex to Titer and MPO/PR-3 Antibodies 2002068
Method: Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multiplex Bead Assay

Anti-Nuclear Antibodies (ANA), IgG by ELISA with Reflex to ANA, IgG by IFA 0050080
Method: Qualitative Enzyme-Linked Immunosorbent Assay/Semi-Quantitative Indirect Fluorescent Antibody


ANA ELISA assays have lower sensitivities for antibodies associated with nucleolar and specked ANA-IFA patterns

Rapid Plasma Reagin (RPR) with Reflex to Titer and TP-PA Confirmation 0050478
Method: Semi-Quantitative Charcoal Agglutination/Semi-Quantitative Particle Agglutination

Human Immunodeficiency Virus Types 1 and 2 (HIV-1, HIV-2) Antibodies by CIA with Reflex to HIV-1 Antibody Confirmation by Western Blot 2005377
Method: Qualitative Chemiluminescent Immunoassay/Qualitative Western Blot


Choosing Wisely. An initiative of the ABIM Foundation. [Accessed: Sep 2017]

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65(1): 1-11. PubMed

General References

Espinoza GM, Prost A. Cogan's syndrome and other ocular vasculitides Curr Rheumatol Rep. 2015; 17(4): 24. PubMed

Fugate JE, Smith JH, Claassen DO. Bilateral cochlear enhancement in Cogan syndrome. Neurology. 2009; 73(1): 75. PubMed

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(4): 483-8. PubMed

Kessel A, Vadasz Z, Toubi E. Cogan syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 2014; 13(4-5): 351-4. PubMed

Mazlumzadeh M, Matteson EL. Cogan's syndrome: an audiovestibular, ocular, and systemic autoimmune disease. Rheum Dis Clin North Am. 2007; 33(4): 855-74, vii-viii. PubMed

Singer O. Cogan and Behcet syndromes Rheum Dis Clin North Am. 2015; 41(1): 75-91, viii. PubMed

Medical Reviewers

Last Update: September 2017