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Cogan's syndrome; diffuse interstitial keratitis
A clinical diagnosis.
Etiology:
- autoimmune (putative)
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]
Special laboratory:
- slit-lamp examination
Management:
- prednisone, ENT referral [2]
Related
keratitis
General
vasculitis
syndrome
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 846
- 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
- ARUP Consult: Cogan Syndrome Vasculitis
The Physician's Guide to Laboratory Test Selection & Interpretation
https://www.arupconsult.com/content/cogan-syndrome-vasculitis
- 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
PMID: 29490187
http://www.nejm.org/doi/full/10.1056/NEJMicm1709103