SHAR-PEI RECURRENT FEVER SYNDROME
Also called “SWOLLEN HOCK SYNDROME”
or “FSF” (“Familial Shar Pei Fever)
A fever of 106º F is a medical emergency. It is a good practice to know
The fevers are unpleasant and can be dangerous if the fever rises to 106º F but what makes this syndrome a serious problem is the accompanying kidney damage. An abnormal protein called “amyloid” is laid down in the kidney destroying the kidneys ability to filter protein. The valuable blood proteins are thus lost in urine along with waste chemicals. The dog becomes thin from the loss of body proteins, develops a propensity to throw abnormal blood clots throughout the body (from urinating out the proteins that would normally prevent this), and high blood pressure results.
All Shar pei should be regularly screened for urinary protein loss with a urinalysis.
WRINKLES GONE WRONG
The characteristic skin wrinkles that make the Shar pei what it is are caused by the excessive production of hyaluronan. Hyaluronan is a structural protein in everyone’s skin but a mutation in the Shar pei leads to multiple copies of the genes regulating production of hyaluronan. The result is a whole lot of extra hyaluronan puffing up the skin.
All Shar pei have this mutation; without this mutation the dog cannot really be a Shar pei but not all Shar peis have this mutation in the same way. Some have a mutation that leads to variable qualities of hyaluronan. In other words, not all the hyaluronan produced is of a healthy quality. Poor quality hyaluronin creates inflammation. This unhealthy type of mutation is often called the “meatmouth” mutation.
The “meat mouth” (on the left) Shar pei has a puffy muzzle
It might seem that one might simply look at a Shar pei’s face to determine if he or she is a candidate for Fever Syndrome but the situation is more complicated.
HOW DO I KNOW IF MY SHAR PEI HAS THE SYNDROME?
Obviously, a fever can develop in a Shar pei from a wound or other source of infection just as it can in any other breed of dog. Some effort should be made to find another source of the fever and this generally requires a complete physical examination and some blood testing.
At the present time there is no test for this disease though since the genetics of this condition have recently been worked out, a test will likely soon become available. Until then, diagnosis is made based on the clinical findings in the patient. One episode of fever is enough to make the diagnosis. Fevers classically begin before age 18 months but can begin at any age.
TREATMENT OF FEVER SYNDROME
During a fever episode anti-inflammatory medications provided by the veterinarian can be used to control high fevers. The real challenge, however, is to prevent kidney damage in the long term. It is possible for a dog to have substantial kidney damage before the first fever episode even happens therefore it is important to begin therapy after the first episode and to regularly screen for urinary protein loss in any Shar pei whether fevers have occurred or not.
The medication central to the prevention of amyloid deposition in the kidney is colchicine.
Cell division, a process more scientifically known as “mitosis,” requires microscopic protein fibers, acting like structural cables, to pull dividing cells apart. These cables are called “mitotic spindles” and colchicine interferes with their formation. The ability of colchicine to interfere with this sort of structural protein formation has led to its use in abnormal protein depositions such as amyloidosis. This means that colchicine can prevent the kidney damage that occurs in Recurrent Fever Syndrome.
For more details on colchicine click here.
Recently, legal issues have forced generic colchicine off the market leaving only the brand name product “Colcrys”®. This medication is problematically expensive for most pet owners. A special arrangement has been made for the owners of Recurrent Fever Syndrome dogs. An application can be obtained at www.needymeds.com, a valid prescription is required, and your veterinarian will need to fill out part of the application. Colchicine can also be obtained through a compounding pharmacy. A prescription is still necessary but the other paperwork is not.
Antioxidants are important in amyloid prevention in that they help preserve cell membrane fatty acids. Current recommendations include supplements in omega 3 fatty acids (fish oil) and a quality multi-vitamin. Herbal antioxidants have also been recommended. Normalizing proper hyaluronan metabolism may require magnesium supplementation. Consult your veterinarian for specifics.
Page posted: 5/29/2011