Cogan Syndrome Vasculitis

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).

Quick Answers for Clinicians

Which testing algorithms are related to this topic?

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 (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

Background

Epidemiology

  • 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

Pathophysiology

  • 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

ARUP Lab Tests

Primary Tests

Preferred reflex panel for the workup of suspected vasculitis

For patients with a history of vasculitis, refer to the ANCA reflex panel that includes a titer, and MPO and PR3 antibodies

For evaluation of autoimmune liver disease, use in conjunction with autoimmune liver disease evaluation with reflex to smooth muscle antibody, IgG

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

Panel includes ANCA IgG MPO antibodies, IgG ; and serine proteinase 3, IgG

Preferred reflex panel for managing patients with a known diagnosis of vasculitis; may be assistive in evaluating suspected vasculitis

For the workup of suspected vasculitis, the preferred panel is ANCA-associated vasculitis profile (ANCA/MPO/PR3) with reflex to ANCA titer

Panel includes ANCA, IgG; myeloperoxidase antibody; and serine proteinase 3 antibody

Aid in initial diagnosis of connective tissue disease

All ELISA results reported as "Detected" are further tested by IFA using HEp-2 substrate with an IgG-specific conjugate

Detect antibodies against double-stranded DNA (dsDNA), histones, SS-A (Ro), SS-B (La), Smith, Smith/RNP, Scl-70, Jo-1, centromere proteins, and other antigens extracted from the HEp-2 cell nucleus

ANA ELISA assays have been reported to have lower sensitivities than ANA IFA for systemic autoimmune rheumatic diseases

CDC recommended test for the screening and diagnosis of syphilis

For RPR test that follows reverse algorithm, refer to rapid plasma reagin (RPR) with reflex to RPR titer or T. pallidumantibody by particle agglutination

Evaluate for HIV

Related Tests

Preferred test to detect acute phase inflammation (eg, autoimmune diseases, connective tissue disease, rheumatoid arthritis, infection, or sepsis)

Nonspecific test used to detect inflammation associated with infections, cancers, and autoimmune diseases

May help in ruling out infectious process

Screen for various metabolic and kidney disorders

Screening test to evaluate kidney function

Screening test to evaluate kidney function

Assay interference (negative) may be observed when high concentrations of N-acetylcysteine (NAC) are present

Negative interference has also been reported with NAPQI (an acetaminophen metabolite) but only when concentrations are at or above those expected during acetaminophen overdose

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