Vortex Keratopathy: Fabry Related or Amiodarone Induced?




The report entitled “PP-116 vortex keratopathy associated with long term use of amiadarone,” written by Altun et al and published in a recent issue of American Journal of Cardiology , was quite interesting. Here, we would like to emphasize some relevant points.


Amiodarone is widely used for treatment of atrial fibrillation. It has varying side effects and vortex keratopathy is 1 of them. We would like to point out that the differential diagnosis for this clinical entity should also include Fabry disease (FD). FD is an X-linked inherited, rare, progressive, multisystem disorder of glycosphingolipid metabolism affecting multiple organs and causing varying degrees of dysfunction. Cornea verticillata (vortex keratopathy) is one of the pathognomonic features of the disease. Indistinguishable pattern of amiodarone-induced vortex keratopathy from FD related cornea verticillata may confuse the diagnosis if solely considered. It is also claimed that corneal opacities of FD usually do not affect vision. Because of the wide spectrum of the disease, patients with FD are generally diagnosed at late ages. Vortex keratopathy in a patient with potential Fabry-related chronic heart disease, using amiodarone, may obscure the diagnosis if we narrow the differential diagnosis. Beyond FD, drugs such as chloroquine, hydroxychloroquine, gentamicine, nonsteroidal anti-inflammatory drugs may also induce vortex keratopathy. Other possible causes for vortex keratopathy should be kept in mind at clinical practice.

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Nov 28, 2016 | Posted by in CARDIOLOGY | Comments Off on Vortex Keratopathy: Fabry Related or Amiodarone Induced?

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