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Diagnosis And Treatment of Familial SharShar-Pei Fever Every Shar-Pei owner should become proficient at taking a rectal temperature on their dog. A glass rectal thermometer or the new electronic thermometers can be used. Lubricate the thermometer with petroleum jelly or K-Y jelly and insert in the rectum about 1 inch. Read the temperature after about 1 minute. The normal dog temperature is 101102o F. Dogs with FSF usually have a temperature around 104-107o F. I then start aspirin therapy immediately, preferrably using Ascriptin®. I usually dose every 6 hours for the first 24 then every 12 hours thereafter for an additional 3 days. Generally, in cases of FSF, the temperature will begin to drop and will normalize in 12-24 hours. If the temperature doesn’t drop, then the dog should be seen by a veterinarian and a work-up done. As was stated before, treatment for renal amyloidosis is usually difficult and more successful if started in the early stages of the disease. To facilitate early detection, I advise checking a urinalysis every 3-4 months in Shar-Pei over 1-2 years of age. Primary tests I look at are the SPECIFIC GRAVITY and the URINE PROTEIN. The SPECIFIC GRAVITY is a measure of the concentration of the urine. One of the primary functions of the kidney is to conserve water and form a concentrated urine. In a young Shar-Pei with a dilute (watery) urine, kidney disease must be suspected and further testing pursued. Likewise, another function of the kidney is to prevent the loss of important substances such as protein, glucose,etc. If large amounts of PROTEIN appear in the urine, a kidney problem is suspect. It is also very important in the Shar-Pei to periodically weigh your dog. As we said before, weight loss is a key sign of trouble. Don’t rely on your visual assessment of the dog - get an accurate weight. Serial weight measurements are very critical in early detection of disease. There is an experimental therapy currently being tested using a drug called COLCHICINE. It has shown some promise in decreasing episodes of FSF and preventing the development of amyloidosis. ©Jeffrey W. Vidt, DVM 10/2005 Make your donations to FSF research to the CSPCA Charitable Trust Lee Arnold, Chairman P.O. Box 7007 Bedminster, NJ 07921 Familial Shar-Pei Fever Familial Shar-Pei Fever (FSF) is a condition seen primarily in Shar-Pei. It is characterized by 1 or more bouts of unexplained fever, usually 105-107oF. Fevers usually start prior to 18 months of age with many Shar-Pei having their first episode at 6-8 months of age. Fever attacks tend to become less frequent with age and generally last between 12-36 hours without treatment. Fever episodes may be accompanied by one or more of the following signs: - swelling around a joint particularly the “hock” joint or ankle. This is called HOCK FEVER or SWOLLEN HOCK SYNDROME by Shar-Pei breeders. This will manifest as lameness. Sometimes it will involve the wrist of the front leg. - sometimes a swollen, painful muzzle (Swollen Lip Syndrome). - abdominal pain, relunctance to move, rapid , shallow breathing. - “sick dog” look - tail down, ears down, depression, roached back. - loss of appetite Web site: www.drjwv.com What Is the Cause of Familial Shar-Pei Fever? While the cause of FSF in the Shar-Pei is not known, the following points are reasonably certain: 1. Shar-Pei seem to have a problem regulating their immune system. This has been called IMMUNE DYSREGULATION. 2. Shar-Pei with FSF have high levels of Interleukin-6, a substance which TURNS ON various parts of the immune system. 3. Those individuals with FSF also have an abnormal inflammatory response resulting from a hyperactive immune system. 4. It appears that Shar-Pei with FSF are prone to develop amyloidosis. This is discussed elsewhere in the pamphlet. 5. Pedigree studies indicate that FSF is an inherited disorder in the Shar-Pei, probably as an autosomal recessive trait. 6. Shar-Pei with FSF seem to have a higher incidence of other diseases such as immunemediated kidney disease, thromboembolic phenemona (throwing blood clots), certain types of cancer or tumors, etc. 7, Survey results have indicated that as many as 23% of Shar-Pei experience fevers of unknown origin. 8. Unfortunately past research projects have been unable to develop a DNA blood test to screen for this condition in Shar-Pei. Currently, the NIH is involved in trying to help the fancy. If you want to help with FSF research send your donation to the CSPCA Charitable Trust. 9. Current research is focusing on the AUTOINFLAMMATORY DISEASES seen in humans. These include the HEREDITARY RECURRENT FEVER syndromes (HRFs), CASPASE-ASSOCIATED PERIODIC SYNDROMES (CAPS), TUMOR NECROSIS FACTOR RECEPTOR-ASSOCIATED PERIODIC SYNDROMES (TRAPS), and others. These are due to mutations in genes which regulate the production of inflammatory cytokines such as the those of the interleukin family especially IL-1b. FSF is very similar to a disease seen in humans called Familial Mediterranean Fever. This human disease affects certain ethnic groups near the southeastern 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 in people as well.. 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 and the mutation in SharPei has not been isolated at this time. Amyloidosis.... A Killer Because Shar-Pei with FSF have an immune system which is constantly TURNED ON their bodies produce high levels of ACUTE PHASE PROTEINS. Some Shar-Pei can’t break these proteins down and instead form a protein called AMYLOID AA which is deposited in the kidneys and liver. This amyloid “clogs” up the kidneys resulting in kidney failure and death. Deaths from renal (kidney) amyloidosis have been reported in dogs as young as 8 months of age and as old as 12 years of age. Most deaths occur in the 3-5 year age group. Signs of amyloidosis are usually those of kidney failure: 1. Unexplained weight loss 2. Increased water consumption and urination. 3. Vomiting, loss of appetite 4. “Bad breath” due to uremic poisoning. 5. Depression and weakness. 6. Decrease in muzzle size. Diagnosis of amyloidosis can only be accomplished by doing kidney and/or liver biopsies. Kidney failure can be diagnosed by obtaining a urinalysis, and doing blood tests to evaluate kidney function. Since most patients with amyloidosis don’t show symptoms until the disease is well-advanced, treatment is usually difficult if not impossible. Early stages of kidney failure can often be managed utilizing prescription low-protein diets and symptomatic therapy to control vomiting, infection, etc. Owners need to be aware that there can be complications to FSF episodes such as Streptococcal Toxic Shock Syndrome (STSS), bleeding problems such as Disseminated Intravascular Coagulation (DIC), organ failure, etc. Your vet should be notified with every FSF attack and your dog should be checked if anything unusual is seen.