Familial Shar-Pei Fever in Dogs

Familial Shar-Pei Fever (FSF) dogs are in a constant state of inflammation. Recurrent fever and pain, accompanied by tibiotarsal joint (hock) swelling occur as random events. In some cases, the muzzle may become enlarged as well. The fevers range between 103 - 107F (39.4 - 41.7C) and last 12 to 36 hours without treatment. It must be noted that your dog will require emergency treatment if the fever reaches 106F or higher. Do not delay contacting a qualified veterinarian if your Shar-Pei has a fever that is climbing rapidly. Amyloidosis is a definite concern, and must be diagnosed in early stages, due to the risk of kidney failure.

Surveys show that 23% of dogs of the Chinese Shar-Pei breed are known to have Familial Shar-Pei Fever. The syndrome is an inherited autosomal recessive condition, with episodic fever and swelling of the hock as the two main characterizations. This condition must be treated by a veterinarian in order to avoid or minimize the effects of amyloidosis, a serious complication of the syndrome.

As the owner of a Shar-Pei, it is important for you to know that not all dogs of this breed will develop FSF. However, if your pet has a fever and swollen joints, it is highly recommended to visit the veterinarian promptly in order to investigate the reason for the illness. Other symptoms that may accompany an episode of Familial Shar-Pei Fever are as follows:

• Swollen, painful muzzle
• Swelling in wrists and lips, which may feel hot
• Reluctance to move
• Pain in the abdomen
• Vomiting
• Diarrhea
• Shallow breathing
• A stance that shows obvious discomfort, such as an arched back

If your pet has developed amyloidosis, (which is a complication in about 5% of Shar-Pei’s who have FSF) you will see the additional symptoms of:

• Increased thirst
• Frequent urination
• Vomiting
• Halitosis (bad breath)
• Loss of appetite

Familial Shar-Pei Fever usually begins to present symptoms in dogs around the age of 18 months, but it is not unusual for the onset of the disease to occur in adulthood. In dogs with early onset of FSF, the episodes can become less frequent with age. The cause of FSF is various genetic factors:

• A genetic defect in the immune system can lead to an overactive immune response.
• The mutated genes upset the regulation of inflammatory chemical messengers in the body.
• A genetic mutation resulting in excessive wrinkles, causing overproduction of hyaluronan, which can deregulate normal inflammatory processes (fever, redness and swelling). This means, in general, that dogs with extra wrinkling are suspected to be more apt to have FSF.

Pets with FSF can develop the secondary complication of amyloidosis; the cause for this being that Shar-Pei dogs who have the fever syndrome will have increased serum concentrations of interleukin-6, which stimulates the production of amyloid protein (known to be associated with inflammatory disease).

To begin, the veterinarian will perform diagnostic tests to rule out other illnesses by exclusion. Checking for tick-borne diseases is common, along with testing for autoimmune disorders. Urinalysis and blood chemistry will be included.

Unfortunately, at present there are no DNA blood tests definitive to FSF. Studies are currently underway with the hopes of developing a screening method. Blood test results will show an increased white cell count and a hike in liver enzymes during a fever episode. Further blood tests may show the following issues:

• Neutrophilia
• Monocytosis
• Hypercholesterolemia
• Hypomagnesemia
• Hyperglobulinemia
• High blood platelet count

A Shar-Pei with Familial Shar-Pei Fever should automatically be red flagged for the possibility of amyloidosis occurrence. Upon examination, the veterinarian may see oral ulcerations, dehydration, and pale mucous membranes. If your dog is in the advanced stages of amyloidosis, further testing will show the following:

• Proteinuria (high levels of protein in the urine)
• Low albumin in the blood
• Fluid retention
• X-ray and ultrasound may show abnormalities of the kidneys and liver

Biopsy of organs may be done to determine the extent of the disease, but this is not without risk to your pet.

Keeping your dog’s fever down is paramount to this illness. Aspirin may be given upon recommendation of the veterinarian. Be careful to administer only the amount prescribed. Aspirin may be given for several days in order to combat the fever, and to prevent a rebound of symptoms.

If your pet is in the midpoint of a severe episode, IV support may be required. Anti-inflammatories will be given if pain is noted. Your pet is at risk for a septic-like shock with an extremely high fever, accompanied by necrotic skin sloughing associated with the mucin found in Shar-Pei skin, which could signal an infection at this time as opposed to a fever event. All of the above point to the importance of keeping the veterinarian involved in the care of your Shar-Pei. At any time, if your Shar-Pei is showing signs of unusual behavior, a veterinarian visit is in order. This is stated because some dogs may not display the symptoms of FSF, but still have amyloidosis which can eventually mean kidney or liver failure.

Colchicine is the treatment of choice for dogs with FSF because it has been known to reduce the frequency and severity of fevers, and can help to prevent amyloidosis. (It is interesting to note that colchicine is used on humans with the illness Familial Mediterranean Fever to prevent and treat amyloidosis.) This drug will be prescribed daily.

Any Shar-Pei, who has FSF, should be monitored with regular urinalysis (to check creatinine level), CBC and chemistry profile. It is important to consistently monitor blood pressure and body weight. Consistent high fever can produce a loss of wrinkles ( due to lack of production of hyaluronan). Prednisone is sometimes used to treat mucin issues.

Colchicine must be continued as a preventative of amyloidosis and a treatment for recurring fever events.

A high-quality diet is necessary, which can be determined by you based on your observations of your pet’s dietary health. It is critical to keep in mind that your Shar-Pei should have a diet that includes minerals, glucosamine, hyaluronan, and vitamins for optimal health and nutrition. Some Shar-Pei may benefit from a low carb and grain free diet. Omega 3’s are important, as are lecithin and probiotics.

Stress is known to bring on episodes of Familial Shar-Pei Fever. Excessive exercise, illness or boarding situations, for example, can lead to a fever event.

If your Shar-Pei is suffering from kidney or liver failure due to amyloidosis, supportive care is available. The long-term prognosis is not favorable due to the organ damage caused by this complication.