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Barrier Animal Care Clinic
32 Hardens Manor Way
Eastmoor Place
Charlton
London
SE7 8LP
FAMILIAL SHAR-PEI FEVER
Last Update March 2010
Introduction
 Familial Shar-Pei Fever (FSF) is an autoinflammatory syndrome, characterized by one or more bouts of
unexplained fever
 It has also been known as Hock Fever or Swollen Hock Syndrome
 Fevers usually start before 18 months of age, with many Shar Peis having their first episode at 6-8 months
 Fever attacks tend to become less frequent with age and generally last between 12-36 hours without treatment
 Fever events are a marker for the presence of autoinflammatory disease and treatment should be started early
to prevent complications
 The number and frequency of fever events does not correlate with the severity of underlying chronic
inflammation, which is what puts them at risk of developing amyloidosis
• Every Shar Pei owner should have a digital thermometer at home and be proficient at taking a rectal
temperature. The thermometer should be lubricated with KY jelly or Vaseline and inserted about 1 inch into the
rectum. The temperature is taken after the thermometer beeps. Normal temperature is about 38.5 C.
What is the cause?
 The cause of FSF in Shar Peis is not known
 However, the following points are reasonably certain:
 Shar Peis appear to have a problem regulating their immune system (called immune dysregulation)
 Shar Peis with FSF have high levels of ‘Interleukin 6’, a substance which turns on various parts of the immune
system
 Those individuals with FSF also have an abnormal inflammatory response resulting from a hyperactive immune
system
 Shar Peis with FSF are prone to developing amyloidosis
 Pedigree studies indicate that FSF is an inherited disorder in the Shar Pei, probably as an autosomal recessive
trait. The underlying genetic defect is most likely a defect in how the messengers of inflammation are controlled.
It appears to be a complex disorder involving multiple interactive genetic mutations.
 Shar Peis with FSF appear to have a higher incidence of other diseases such as immune-mediated kidney
disease, thromboembolic phenomena (throwing blood clots) and certain types of cancer
 Survey results indicate up to 23% of Shar Peis experience fevers of unknown origin
 Past research projects have been unable to develop a DNA blood test to screen for this condition in the Shar
Pei. Research is ongoing.
 There may be ‘triggers’ involved in the initiation of an FSF episode. Stress may be such a trigger e.g. dog
training class, another illness, a dog in heat, excessive exercise.
 Diet does not appear to be helpful in prevention of FSF or kidney disease
Amyloidosis
 A Shar Pei with FSF has an immune system which is constantly ‘turned on’. Their bodies produce high levels of
acute phase proteins. If the Shar Pei cannot break these proteins down, or there are large amounts
overwhelming the body’s ability to excrete if, a protein called Amyloid AA is formed which is deposited in the
kidneys and liver.
 This amyloid ‘clogs’ up the kidneys or liver, compressing the adjacent cell walls resulting in damage and death of
the normal cells
 Deaths from renal (kidney) amyloidosis have been reported in Shar Peis ranging from 8 months to 12 years of
age (most in the range 3-5 years of age)
 Signs of amyloidosis are usually those of kidney failure – i.e. unexplained weight loss, increased water
consumption and urine production, vomiting, loss of appetite, bad breath, depression, weakness and decrease in
muzzle size
 Diagnosis of kidney failure is done by blood and urine testing. Diagnosis of amyloidosis can only be achieved by
kidney/liver biopsy.
Barrier Animal Care Clinic LLP
Registered in England Partnership No. OC303416
Members: B Spolander BVSc MRCVS & S Rennie BA VetMB MRCVS
Tel: 020 8293 6580 Fax: 020 8858 5609 Email: [email protected]
www.wellpetclinic.co.uk
VAT Reg: 691 3368 16
Barrier Animal Care Clinic
32 Hardens Manor Way
Eastmoor Place
Charlton
London
SE7 8LP
Human disease
 FSF is very similar to a disease seen in humans called Familial Mediterranean Fever (FMF)
 This human disease affects certain ethnic groups near the south-eastern region of the Mediterranean Sea
 It is characterized by recurrent bouts of fever, joint pain and inflammation and many patients go on to develop
amyloidosis
 It is an inherited disorder
 The FMF gene in man has been isolated and sequenced. FMF is due to mutations in the FMF gene. Genetic
analysis has shown that FSF is not the same as FMF in man
Clinical Signs
• Fever (usually 39.4-41.7 C)
• Swelling around a joint, especially the hock/ankle joint. Also seen in the carpus/wrist of the front leg. The dog is
often lame.
• Swollen painful muzzle (Swollen Lip Syndrome)
• Abdominal pain, reluctance to move
• Rapid, shallow breathing
• Loss of appetite
• ‘Sick dog’ look – tail down, ears down, depression, roached back
• Complications to FSF – Streptococcal Toxic Shock Syndrome (STSS), bleeding problems such as Disseminated
Intravascular Coagulation (DIC), thromboembolic disease and high blood pressure associated with kidney failure
Diagnosis
• Diagnosis of FSF is made by clinical signs and excluding other possibilities. Blood and urine testing may need to
be performed and testing for tick-borne diseases (in high-risk areas). Other tests, including those for autoimmune
disorders, may be required.
• The most reliable test for amyloidosis is biopsy of the kidney/liver
There is no specific test for FSF. Note that there is no DNA test that can differentiate between normal, affected and
carrier animals.
Treatment
 The aim of treatment is to reduce the severity and frequency of fever outbreaks and to try and block the
development of amyloidosis
 Fever episodes are treated with Non-Steroidal Anti-Inflammatory Drugs (NSAIDs) such as Metacam
 Treatment for renal amyloidosis is difficult and more successful if started in the early stages of the disease. A
drug called colchicine is used in people with FMF, although the effectiveness of this drug in animals has not
been proven. The dose in dogs is 0.025-0.03mg/kg twice daily. Side effects include vomiting, diarrhoea and
abdominal pain. Initially start on a low dose and try to gradually increase to the maximum recommended amount
or the most that can be tolerated without side effects. Colchicine treats the underlying pathology by blocking the
movement of neutrophils (one of the white blood cells), decreasing levels of cytokines (the messengers of
inflammation) and blocking the formation of amyloid protein (a waste-product of inflammation). Treatment is for
life.
Barrier Animal Care Clinic LLP
Registered in England Partnership No. OC303416
Members: B Spolander BVSc MRCVS & S Rennie BA VetMB MRCVS
Tel: 020 8293 6580 Fax: 020 8858 5609 Email: [email protected]
www.wellpetclinic.co.uk
VAT Reg: 691 3368 16
Barrier Animal Care Clinic
32 Hardens Manor Way
Eastmoor Place
Charlton
London
SE7 8LP
What should I do if my dog has had a bout of FSF?
 Ensure you have a digital thermometer at home so if your dog becomes ill you can take the temperature
 Ensure you have a supply of NSAIDs (such as Metacam) at home so that if your dog does have a temperature
you can administer a dose
 Discuss with the vet about starting colchicines therapy
 Monitoring for the complications which often accompany FSF should be a major goal
 It is important to periodically weigh your dog (weight loss is a sign of trouble). An accurate weight should be
obtained (don’t just rely on a visual assessment).
 Urine testing for specific gravity and protein loss
 The Specific Gravity (SG) is a measure of the concentration of the urine. One of the main functions of the
kidneys is to conserve water and form a concentrated urine.
 Likewise, another function of the kidney is to prevent the loss of important substances such as protein. This is
tested for on a dipstick, and if there is concern we would perform a Urine Protein:Creatinine (UPC) ratio.
 If your dog has had a bout of FSF, we advise weighing and urine testing for SG and protein every 3 months (on
the first urine in the morning after the dog has had water withheld overnight). Blood testing should be done every
6-12 months and if the urine analysis is abnormal.
 Dogs with FSF should not be used in breeding programmes and should be neutered
 Dogs with FSF should be maintained as stress-free as possible
What should I do if my dog has never had a bout of FSF?
 Ensure you have a digital thermometer at home so that if your dog becomes ill you can take the temperature
 If your dog does have a temperature you should seek veterinary attention urgently
 Annual blood testing and urine testing is sensible due to the Shar Pei’s predisposition to disease
 Note also that any dogs with a family history of FSF should be closely monitored and discuss with the vet about
usage of colchicine
Antioxidants and Supplements
 Add to the dog’s diet to offset the chronic inflammation
• 50-100 mg alpha-lipoic acid once daily (maximum 100mg/day)
• Multi-vitamins (with A, D, E and complete B-complex vitamins)
• 2000mg omega-3 fish oil once daily
• 100-500mg curcumin with bioperine once daily
• 100-300mg boswellia twice daily
• Vitamin B12 1000mcg daily
• 200mg magnesium (not oxide) daily
• 100-200mg resveratrol daily
• Vitamin K2 (menaquinone, MK7)

Diet – recent research has suggested rancid fatty acids and oxidised cholesterol in cell membranes
have been lined to chronic inflammation and the deposition of reactive amyloid. Feed a good quality
diet and avoid rancid polyunsaturated, hydrogenated or trans-fats.
Barrier Animal Care Clinic LLP
Registered in England Partnership No. OC303416
Members: B Spolander BVSc MRCVS & S Rennie BA VetMB MRCVS
Tel: 020 8293 6580 Fax: 020 8858 5609 Email: [email protected]
www.wellpetclinic.co.uk
VAT Reg: 691 3368 16