SHAR-PEI RECURRENT FEVER SYNDROME

Also called “SWOLLEN HOCK SYNDROME,”
"SPAID" or "SHAR PEI AUTOINFLAMMATORY DISEASE"
or “FSF” (“Familial Shar Pei Fever Syndrome)

A fever of 106º F is a medical emergency. It is a good practice to know
how to take your dog’s temperature and is especially important if your
Shar pei seems listless. Normal canine body temperature is 100-102º F

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.
Red flags include urinary protein and dilute urine (specific gravity less than 1.020).

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 hyaluronan breaks down rapidly in a process that generates inflammation which, in turn, creates fever and damages organs. The mutation that creates all this poor quality hyaluranan is often called the “meatmouth” mutation.

The “meat mouth” (on the left) Shar pei has a puffy muzzle
while the “bone mouth” (on the right) is sleeker.

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.

As for screening tests, there is a genetic test available at Cornell University, though testing has been temporarily suspended as of August 21st, 2020. The mutated genetic variant that creates the meat mouth appearance is typically present in multiple copies within an affected Shar Pei. The more copies are present, the more reactive the inflammation. The genetic test determines for the number of mutated copies present and reports the "copy number variant." The higher this number, the more affected the dog. In the absence of a test, 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.

The test requires a blood sample and a six page information form filled out by both the owner and the veterinary professional drawing the blood. A microchip number or tattoo is required to confirm the identity of the patient. Testing information can be viewed at:

https://ahdc.vet.cornell.edu/sects/Molec/spaid.cfm

TREATMENT OF FEVER SYNDROME

During a fever episode anti-inflammatory medications provided by the veterinarian can be used to control high fevers. Pain medication is often needed to control the discomfort during the episode. 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.

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. GoodRx.com is another program that provides discounts on costly prescription medications and their coupons are accepted at most major pharmacies. Colchicine can also be obtained through a compounding pharmacy. A prescription is still necessary regardless of which source is employed.

Cell in the process of division. The spindles are shown in green. (Photocredit: Public Domain Graphic via Wikimedia Commons)

OTHER THERAPY

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. Antioxidants such as vitamin C, Lecithin granules and glucosamine supplements to improve the hyaluronan quality are also recommended. Consult your veterinarian for a specific protocol for your dog. Early intervention is the goal with this condition.

Herbal antioxidants have also been recommended. Normalizing proper hyaluronan metabolism may require magnesium supplementation. Consult your veterinarian for specifics.

Page posted: 5/29/2011
Page last updated: 2/13/2021