Cogan Syndrome Vasculitis - Cogan Syndrome

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

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 – anti-neutrophil cytoplasmic antibody (ANCA)
      • Connective tissue disease – anti-nuclear antibody (ANA), CBC
    • 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).

Epidemiology

  • Incidence – rare; ~250 cases reported
  • Age – children (rare) and young adults in 20s-30s
  • Sex – M:F, equal
  • Ethnicity – more common in Caucasians

Pathophysiology

  • Inflammation in all vessel sizes (small, medium, large)
  • Primary ocular target – small vessels in the vascularized layers of the anterior globe, episclera, sclera and uveitis

Clinical Presentation

  • Constitutional – headache, fever, arthralgia, arthritis
  • Ophthalmologic – interstitial keratitis, iritis, uveitis, episcleritis (generally reversible)
  • Audiovestibular – Ménière-like syndrome (vertigo, tinnitus, ataxia) with or without sudden sensorineural hearing loss; frequently results in deafness
  • Vasculitis – occurs in 10-20% of patients
  • Cardiovascular – aortic disease; life threatening in small percent  of cases

Indications for Laboratory Testing

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

C-Reactive Protein 0050180
Method: Quantitative Immunoturbidimetry

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

Limitations

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

Additional Tests Available

CBC with Platelet Count and Automated Differential 0040003
Method: Automated Cell Count/Differential

Comments

May help in ruling out infectious process

Urinalysis, Complete 0020350
Method: Reflectance Spectrophotometry/Microscopy

Comments

Screen for hematuria, proteinuria, and RBC casts

Sedimentation Rate, Westergren (ESR) 0040325
Method: Visual Identification

Comments

Evaluate for suspected vasculitis

Urea Nitrogen, Serum or Plasma 0020023
Method: Quantitative Spectrophotometry

Comments

Assess for renal disease

Creatinine, Serum or Plasma 0020025
Method: Quantitative Enzymatic

Comments

Assess for renal disease

Guidelines

American Society for Clinical Pathology. Choosing Wisely - Five Things Physicians and Patients Should Question. An initiative of the ABIM Foundation. [Last revision Feb 2015; Accessed: Jan 2016]

Jennette J, Falk R, Bacon P, Basu N, Cid M, Ferrario F, Flores-Suarez L, Gross W, Guillevin L, Hagen E, Hoffman G, Jayne D, Kallenberg C, Lamprecht P, Langford C, Luqmani R, Mahr A, Matteson E, Merkel P, Ozen S, Pusey C, Rasmussen N, Rees A, Scott D, Specks U, Stone J, Takahashi K, Watts R. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65(1): 1-11. PubMed

General References

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

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

Medical Reviewers

Last Update: February 2016