The Physician's Guide to Laboratory Test Selection and Interpretation

ARUP LABORATORIES | 500 Chipeta Way | Salt Lake City, Utah 84108-1221 | (800) 522-2787 | www.arupconsult.com | www.aruplab.com
20062013 ARUP Laboratories. All Rights Reserved. Cogan Syndrome - p. 1 of 4
Cogan Syndrome
Diagnosis
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
Tests are used to rule out specific diseases and/or demonstrate inflammation
Initial assessment
Erythrocyte sedimentation rate and C-reactive protein elevated in ~50% of patients with active
disease
Rule out other disease processes
Renal disorders urinalysis, urea nitrogen, creatinine
Vasculitis anti-neutrophil cytoplasmic antibody (ANCA)
Connective tissue disease anti-nuclear antibody (ANA), CBC
Rule out specific infections
Treponema pallidum testing
Presence of interstitial keratitis and/or acute sensorineural hearing loss combined with
negative testing for syphilis is highly suggestive of Cogan syndrome
HIV testing
Imaging Studies
Angiogram identify aortitis if symptoms present
Differential Diagnosis
Autoimmune sensorineural hearing loss
Brain tumor meningioma
Mnire disease
Treponema pallidum
Other vasculitic disease
Microscopic polyangiitis
Polyarteritis nodosa
Clinical Background
Cogan syndrome is a rare vasculitis that typically manifests as an ophthalmic disorder (interstitial keratitis)
and/or an audiovestibular disorder. Although no definitive trigger has been identified, a history of Chlamydia
or influenza-like infection has been associated with the onset of Cogan syndrome.
Epidemiology
Incidence rare (~250 cases reported)
Age children (rare) and young adults in 20s-30s
Sex M:F, equal
Clinical Presentation
Constitutional headache, fever, arthralgia, arthritis
Ophthalmologic interstitial keratitis, iritis, uveitis, episcleritis (generally reversible)
Audiovestibular Mnire-like syndrome (vertigo, tinnitus, ataxia) with or without sudden sensorineural
hearing loss; frequently results in deafness
The Physician's Guide to Laboratory Test Selection and Interpretation
ARUP LABORATORIES | 500 Chipeta Way | Salt Lake City, Utah 84108-1221 | (800) 522-2787 | www.arupconsult.com | www.aruplab.com
20062013 ARUP Laboratories. All Rights Reserved. Cogan Syndrome - p. 2 of 4
Vasculitis occurs in 10-20% of patients
Cardiovascular aortic disease (life threatening in 10% of cases)
Treatment
Corticosteroids
Administration early in the course of disease may prevent deafness
Topical steroids for keratitis
Immunosuppressives
Methotrexate
Cyclophosphamide
Cochlear implant
60% of patients experience significant hearing loss
Implant therapy may be effective for severe loss
Lab Tests
Indications for Laboratory Testing
Tests generally appear in the order most useful for common clinical situations. For test-specific information, refer to the
test number in the ARUP Laboratory Test Directory on the ARUP Web site at www.aruplab.com.
Test Name and Number Recommended Use Limitations Follow Up
Sedimentation Rate,
Westergren (ESR)
0040325
Method:
Visual Identification
Aid in determining presence of
inflammation
C-Reactive Protein
0050180
Method:
Quantitative
Immunoturbidimetry
Aid in determining presence of
inflammation
Urinalysis, Complete
0020350
Method:
Reflectance
Spectrophotometry/
Microscopy
Evaluate for hematuria and potential
glomerulonephritis
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
Rule out ANCA+ vasculitis
processes
If screen is positive, titer and
MPO/PR-3 antibodies testing
will be added to aid in antibody
determination
The Physician's Guide to Laboratory Test Selection and Interpretation
ARUP LABORATORIES | 500 Chipeta Way | Salt Lake City, Utah 84108-1221 | (800) 522-2787 | www.arupconsult.com | www.aruplab.com
20062013 ARUP Laboratories. All Rights Reserved. Cogan Syndrome - p. 3 of 4
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
Rule out connective tissue disease
as a cause of vasculitic symptoms
All ELISA results reported as
"Detected" are further tested by IFA
Detects antibodies against dsDNA,
histone, SS-A (Ro), SS-B (La),
Smith, snRNP/Sm, Scl-70, Jo-1,
centromere, and an extract of lysed
HEp-2 cells
ANA ELISA assays
have lower sensitivities
for antibodies
associated with
nucleolar and specked
ANA-IFA patterns
Urea Nitrogen, Serum or
Plasma
0020023
Method:
Quantitative
Spectrophotometry
Rule out renal disease
Creatinine, Serum or
Plasma
0020025
Method:
Quantitative Enzymatic
Rule out renal disease
CBC with Platelet Count
and Automated Differential
0040003
Method:
Automated Cell
Count/Differential
Evaluate for infection, eosinophilia,
anemia, and thrombocytosis
Rapid Plasma Reagin
(RPR) with Reflex to Titer
and TP-PA Confirmation
0050478
Method:
Semi-Quantitative
Charcoal Agglutination/
Semi-Quantitative Particle
Agglutination
Rule out syphilis as a cause of
ophthalmic and/or auditory disorders
The Physician's Guide to Laboratory Test Selection and Interpretation
ARUP LABORATORIES | 500 Chipeta Way | Salt Lake City, Utah 84108-1221 | (800) 522-2787 | www.arupconsult.com | www.aruplab.com
20062013 ARUP Laboratories. All Rights Reserved. Cogan Syndrome - p. 4 of 4
Human Immunodeficiency
Virus Types 1 and 2
(HIV-1, HIV-2) Antibodies
with Reflex to HIV-1
Antibody Confirmation by
Western Blot
2005377
Method:
Qualitative
Chemiluminescent
Immunoassay/Qualitative
Western Blot
Rule out HIV as a cause of
ophthalmic and/or auditory disorders
General References
Fugate JE, Smith JH, Claassen DO. Bilateral cochlear enhancement in Cogan syndrome.Neurology. 2009; 73 (1) :75-.
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) :483-488.
Grasland A, Pouchot J, Hachulla E, Bletry O, Papo T, Vinceneux P. Typical and atypical Cogan's syndrome: 32 cases
and review of the literature.Rheumatology (Oxford). 2004; 43 (8) :1007-1015.
Mazlumzadeh M, Matteson EL. Cogan's syndrome: an audiovestibular, ocular, and systemic autoimmune
disease.Rheum Dis Clin North Am. 2007; 33 (4) :855-viii.
Reviewed by
Tebo, Anne E., PhD. Assistant Medical Director, Immunology at ARUP Laboratories; Assistant Professor of Pathology
(Clinical), University of Utah
Diagnostic Algorithm(s)
PDF algorithm(s) available at www.arupconsult.com.
Vasculitis in Adults Testing Algorithm
Related Content
Alport Syndrome
Autoimmune Inner Ear Disease
Hearing Loss, Hereditary Nonsyndromic - Connexin 26 or 30
Treponema pallidum - Syphilis
Vasculitis - ANCA
Last Update: March 2013