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Cogan syndrome is a rare autoimmune multisystem disease that typically manifests as a non-syphilitic interstitial keratitis 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.
Tests generally appear in the order most useful for common clinical situations
| Test name: Sedimentation Rate, Westergren (ESR) |
| ARUP #: 0040325 |
| Methodology: Westergren |
| Use: Use in initial assessment Determine if inflammation is present |
| Limitations: Not specific for diagnosis; positive in only ~50% of patients with Cogan syndrome |
| Test name: C-Reactive Protein |
| ARUP #: 0050180 |
| Methodology: Quantitative Immunoturbidimetric |
| Use: Use in initial assessment Determine if inflammation is present |
| Limitations: Not specific for diagnosis; positive in only ~50% of patients with Cogan syndrome |
| Test name: Urinalysis, Complete |
| ARUP #: 0020350 |
| Methodology: Reflective Photometry/Microscopic by Yellow IRIS |
| Use: Evaluate for hematuria and potential glomerulonephritis |
| Limitations: Not specific for diagnosis of Cogan syndrome |
| Test name: Anti-Neutrophil Cytoplasmic Antibody with Reflex to Titer and MPO/PR-3 Antibodies |
| ARUP #: 2002068 |
| Methodology: Semi-Quantitative Indirect Fluorescent Antibody/Semi-Quantitative Multi-Analyte Fluorescent Detection |
| Use: Rule out other ANCA+ vasculitis processes If screen is positive, titer and MPO/PR-3 antibodies testing will be added to aid in antibody determination |
| Limitations: Not specific for diagnosis of Cogan syndrome |
| Test name: Anti-Nuclear Antibodies (ANA), IgG by ELISA with Reflex to ANA, IgG by IFA |
| ARUP #: 0050080 |
| Methodology: Qualitative Enzyme-Linked Immunosorbent Assay (ELISA)/Semi-Quantitative Indirect Fluorescent Antibody (IFA) |
| Use: Rule out connective tissue disease as a cause of vasculitic symptoms All ELISA results reported as "Detected" are further tested by IFA ANA ELISA screen is designed to detect 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 |
| Limitations: Not specific for diagnosis of Cogan syndrome ANA ELISA assays have been reported to have lower sensitivities for antibodies associated with nucleolar and specked ANA-IFA patterns |
| Test name: Urea Nitrogen, Serum or Plasma |
| ARUP #: 0020023 |
| Methodology: Quantitative Spectrophotometry |
| Use: Rule out underlying disease processes and assess renal function |
| Limitations: Not specific for diagnosis of Cogan syndrome |
| Test name: Creatinine, Serum or Plasma |
| ARUP #: 0020025 |
| Methodology: Quantitative Enzymatic |
| Use: Rule out underlying disease processes and assess renal function |
| Limitations: Not specific for diagnosis of Cogan syndrome |
| Test name: CBC with Platelet Count & Automated Differential |
| ARUP #: 0040003 |
| Methodology: Automated Cell Count with Flow Cell Differential |
| Use: Evaluate for infection, eosinophilia, anemia and thrombocytosis |
| Limitations: Not specific for diagnosis of Cogan syndrome |
| Test name: Treponema pallidum (Rapid Plasma Reagin) with Reflex to Titer & TP-PA Confirmation |
| ARUP #: 0050478 |
| Methodology: Semi-Quantitative Charcoal Agglutination/Semi-Quantitative Particle Agglutination (PA) |
| Use: Rule out syphilis as a cause of eye and ear disease processes |
| Limitations: Not specific for diagnosis of Cogan syndrome |
| Test name: Human Immunodeficiency Virus Types 1 and 2 (HIV-1, HIV-2) Antibodies with Reflex to Human Immunodeficiency Virus Type 1 (HIV-1) Antibody Confirmation by Western Blot |
| ARUP #: 2005377 |
| Methodology: Qualitative Chemiluminescent Immunoassay/Qualitative Western Blot |
| Use: Rule out HIV as a cause of eye and ear disease processes |
| Limitations: Not specific for diagnosis of Cogan syndrome |