Cogan's syndrome; diffuse interstitial keratitis
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Introduction
A clinical diagnosis.
Etiology
Epidemiology
- rare
- most common in adults in 20's & 30's
- may occur in children
Pathology
- vasculitis of the cornea & inner ear resulting in interstitial keratitis & vestibular dysfunction
- vasculitis may affect other organs as well
Clinical manifestations
- fever, fatigue, & weight loss
- episodes of dizziness & sensorineural hearing loss
- Weber test will lateralize to unaffected ear
- complete deafness usually occurs within a couple of years
- keratitis
- peripheral corneal opacification
- crystalline deposits on the corneal stroma
- conjunctival hyperemia
- vision difficulty
- photophobia
* image[7]
Laboratory
- erythrocyte sedimentation rate (ESR) may be abnormal
- serum C-reactive protein may be abnormal
- complete blood count (CBC)
- may be leukocytosis, thrombocytosis, anemia
- see ARUP consult[6]
Diagnostic procedures
Management
- prednisone, ENT referral[2]
More general terms
Additional terms
References
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 846
- ↑ 2.0 2.1 Medical Knowledge Self Assessment Program (MKSAP) 14, 18. American College of Physicians, Philadelphia 2006, 2018.
- ↑ Wikipedia: Cogan syndrome http://en.wikipedia.org/wiki/Cogan_syndrome
- ↑ Cogan's syndrome Wrongdiagnosis.com http://www.wrongdiagnosis.com/c/cogans_syndrome/intro.htm
- ↑ Vinceneux P, 2005 PDF Orphanet Cogan's syndrome http://www.orpha.net/data/patho/Pro/en/Cogan-FRenPro3333.pdf
- ↑ 6.0 6.1 ARUP Consult: Cogan Syndrome Vasculitis The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/cogan-syndrome-vasculitis
- ↑ 7.0 7.1 N Engl J Med Image Challenge. Feb 22, 2018 http://www.nejm.org/image-challenge
Rodrigues-Barros S, Parreira S Interstitial Keratitis in Cogan's syndrome N Engl J Med 2018; 378:852 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/29490187 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMicm1709103
Patient information
Cogan's syndrome (diffuse interstitial keratitis) patient information