Amyloidosis is a hereditary disease that exists is varying degrees in ALL lines of the Chinese Shar Pei breed.  Most, if not all, of the original imported, foundation stock Shar Pei were affected by this recessive hereditary disease when they arrived in the United States.  And since every Shar Pei line goes back directly to the same small genetic pool of dogs, this disease can be found in every Shar Pei line.  If you talk to a Shar Pei breeder who does not know what amyloidosis is, they obviously do not have enough knowledge of the Shar Pei breed to be producing puppies.  If you talk to a Shar Pei breeder who claims, "it is not in my lines" - they are not being honest.  Amyloidosis is in every line in varying degrees.  There are many lines where the incidence of amyloidosis is low and there are many lines with high incidence and high risk factors.  Only breeders who have knowledge of the various lines and research pedigrees to reduce the risk factors involved should be producing puppies.  People who do not know about the hereditary problems and simply "breed" dogs are only adding to the decline of the Shar Pei breed by perpetuating problems that can only be managed by careful and selective breeding practices to minimize risks.  There is no test for amyloidosis except through tissue samples taken at the time of autopsy.  So at this time only careful and selective breeding will protect the Shar Pei breed and hopefully maintain some degree of health until tests are developed so that breeders can eliminate the disease through selective breeding.

Here at True Gem, we are dedicated to the Chinese Shar Pei breed and to maintaining its health for future generations.  We only place pet and companion Shar Pei in homes on a spay/neuter contract.  And we are extremely selective on placing any Shar Pei in a home with full breeding rights.

So what is this hereditary disease in the Chinese Shar Pei breed?  Amyloidosis is the abnormally large deposit of a substance called amyloid in the tissues of the body.  The kidney is the primary organ affected in the Shar Pei breed, but the liver and spleen can also be affected, though less common.  Think of amyloid as a wax that can build up on cells, interfering with the nutrition of the cell and slowly crushing the cell.  Once the cell is crushed and dies, it is replaced with scar tissue that is nonfunctional in the organs of the body.  Kidney cells are virtually non-replaceable by the organ and once a number of these cells have been destroyed the kidney stops functioning and kidney failure results.  The liver can more easily replace dead and damaged cells, so the progression to amyloidosis is slower.  You can not assume that every Shar Pei that dies of kidney failure has amyloidosis.  There are other causes of kidney failure in dogs, but amyloidosis is the primary cause of premature death in the Chinese Shar Pei breed. A tissue biopsy and Congo Red Stain test is necessary to diagnose amyloidosis and differentiate it from other disorders.  Kidney amyloidosis causes excessive protein loss in the urine.  And one of the proteins being excessively excreted is responsible for preventing blood clots from forming.  These clots tend to lodge in the lungs and may cause labored breathing and respiratory distress in Shar Pei with kidney amyloidosis.

Amyloid production by the body is a normal and natural occurrence in response to tissue injury and inflammation.  Many diseases, traumatic injuries, cancer disorders, stresses, etc can stimulate the production of amyloid.  Normally there is a balance between the amount of amyloid produced and its degradation and excretion from the body.  Amyloid production is a normal protective response to inflammation and injury and only develops into a disease when this normal process goes out of control.  Chinese Shar Pei with amyloidosis can not break down the amyloid and excrete it from the body.  It is not known if amyloidosis in the Chinese Shar Pei is due to an excessive and prolonged production of amyloid, which overwhelms the degradation process, or if there is a defect in the degradation process itself.  But if the amyloid is not excreted naturally, it deposits on the cells.  And once amyloid is deposited in the tissues, nothing can remove it.  As the amyloid builds up on the outside of the cells, the cells are crushed and die. 

So obviously, in Chinese Shar Pei who have amyloidosis, the progression of the disease can be slow or rapid, depending on the amount of amyloid being produced by the body.  Small amounts of naturally produced amyloid can build up on the cells for many years as a result of the natural inflammation process and not result in amyloidosis until the Chinese Shar Pei is 10 years or older.  The hereditary form of amyloidosis tends to strike earlier, affecting Shar Pei between 3 – 5 years of age and resulting in premature death.

Amyloidosis is a recessive hereditary disease.  There is ongoing research to find a test for amyloidosis, but as with many diseases, the only "cure" may be through selective breeding.  It is known that both the sire and dam must either carry the disease or have the disease to pass it on to offspring.  Shar Pei dogs who carry the disease, but do not develop the disease have one normal gene and one abnormal gene and are referred to as heterozygous.  Shar Pei dogs who develop amyloidosis have two abnormal genes and are referred to as homozygous.  Breeding two homozygous Shar Pei (both having two abnormal genes) will only produce homozygous puppies that have amyloidosis.  Breeding two heterozygous Shar Pei (both having one normal gene and one abnormal gene) will produce in a mathematical ratio, some puppies that have no abnormal genes, puppies with only one abnormal gene (heterozygous) and some puppies with two abnormal genes (homozygous).  If one parent is free of amyloidosis (two normal genes) none of the puppies will develop amyloidosis, but depending on the status of the other parent (one or two abnormal genes) the puppies may be heterozygous (one abnormal gene) or free from amyloidosis (two normal genes).  So obviously the ongoing research to develop a test to determine whether a Shar Pei dog is homozygous, heterozygous or free from amyloid is extremely important to the future of the Chinese Shar Pei breed. But in the mean time, knowledge of the various lines, pedigree research and selectively breeding is the only way breeders can fight the disease.  And of course, until the test is developed, there is no way to absolutely guarantee that any individual puppy from a litter will not develop amyloidosis.  Even the most carefully planed and researched breedings can produce some Shar Pei puppies that develop amyloidosis. 

Here at True Gem, we strive to only produce quality, healthy puppies, but we also realize that amyloidosis can appear despite our best efforts.  We will replace any puppy/dog that dies before 5 years of age and tests positive for amyloidosis using the Congo Red Stain test.  This guarantee only applies if the terms of the contract are met and positive Congo Red Stain test results are provided using tissue biopsy acquired at the time of autopsy.

Familial Shar Pei fever is important to know about and understand if you own a Chinese Shar Pei and especially if you are a first time owner of a Chinese Shar Pei.  Many veterinarians who are unfamiliar with the Chinese Shar Pei breed will run extensive and unnecessary tests when presented with a Shar Pei puppy or young adult who is experiencing fever and lameness of unknown origin.  This can result in a very expensive bill, especially if the puppy/dog is taken to an emergency clinic, when simple aspirin therapy was all that was needed.  But it is also extremely important to be completely honest with yourself and your veterinarian if your dog or puppy is suddenly showing signs of lethargy, fever, decreased appetite, lameness, joint swelling, and reluctance to move.  Do NOT lie to yourself or your veterinarian if your puppy or dog suffered a traumatic injury, ingested a poisonous plant or chemical, etc.  FSF will usually last 24 – 48 hours with minimal or no treatment and the puppy/dog will act as if nothing has happened.  But some traumas and especially ingestion of a poisonous material or pneumonia should be treated immediately if the puppy/dog is going to survive.  

Familial Shar Pei Fever (FSF) is also referred to as Shar Pei Fever and Swollen Hock Syndrome.  It is an autosomally recessive fever disorder that resembles Familial Mediterranean Fever in humans.  It is characterized by recurrent, unexplained fever episodes and may be accompanied by joint inflammation.  Fevers are usually 105 – 107 degrees F, but in rare cases may go higher.  If the Shar Pei dog does not have a fever, it is not FSF.  And remember that we are talking about sporadic "unexplained" fevers.  Joint injuries, trauma injuries, viral infections, Lyme disease and even puppies teething can also cause fevers, but these are "explained" fevers.  It is important to not mistake a Shar Pei dog with a ruptured or torn cruciate or other condition as having FSF just because there is an associated fever.

Fever episodes associated with FSF usually last 24 – 48 hours without treatment.  Approximately 50% of Chinese Shar Pei who experience FSF will also have swelling around joints – most commonly the hock joint, which is equivalent to the human ankle joint.  This is sometimes referred to as Swollen Hock Syndrome (SHS).  Do not mistake the normal "socks" (excessive wrinkling on the hocks of some Shar Pei dogs) for Swollen Hock Syndrome.  SHS refers to swelling of the joint (not skin) and causes lameness.  Shar Pei dogs may be stiff or lame and reluctant to move.

Familial Shar Pei Syndrome usually begins when dogs are under 18 months of age, but it can develop in older dogs.  Fever episodes usually become less frequent with age.  There is no association between the number, frequency or severity of FSF episodes and the development or degree of amyloidosis.  However, FSF is an inflammatory disorder.  Amyloid production is a natural and normal occurrence in response to tissue injury or inflammation.  The body's inability to break down and excrete amyloid causing build up and deposit of amyloid on tissues of the body is what causes amyloidosis.  So Shar Pei dogs who have recurrent FSF, but can process and excrete the amyloid will not develop amyloidosis.  Shar Pei dogs who can not process and excrete the amyloid will end up with amyloid deposits in their tissue, which can progress to amyloidosis.  So it can be assumed that Shar Pei dogs who can not excrete the amyloid and have frequent episodes of FSF will develop amyloidosis much quicker due to the amyloid build up on the cells.  But it can also be assumed that Shar Pei dogs who can excrete the amyloid, can also have frequent episodes of FSF and never develop amyloidosis.  A Shar Pei dog who experiences Familial Shar Pei Fever (more often in frequent episodes) is at higher risk of developing amyloidosis, but it does NOT mean every Shar Pei will develop amyloidosis.  The less frequent the episodes, the less likely the dog will develop amyloidosis, if at all.  Incidence of FSF in Shar Pei is about 23 – 28%.  About 25% of the Shar Pei who experience FSF will develop renal amyloidosis and prematurely die of renal failure.  When this happens, it usually results in premature death between the ages of 2 – 5 years.  And it should be noted that some Shar Pei will develop renal amyloidosis without ever having an episode of FSF. 

Typically a Chinese Shar Pei who is experiencing FSF will suddenly (unexplained) have a fever, be lethargic, experience lameness or a stiff gait, be painful and reluctant to move, and have a decreased appetite.  The fever is usually 105 – 107 degrees F.  Buffered aspirin (full strength, 325 mg) can be given every 8 – 12 hours to help with fever reduction and pain for older puppies and adults.  Young Shar Pei puppies should only be given – aspirin (full strength, 325 mg) every 8 – 12 hours.  I have found that the best way to give any type of pill or tablet is to put it in a dab of soft butter, open the dog's mouth and slip it towards the back of their tongue.  Then gently hold their mouth closed.  They like the taste of the butter and the pill usually just slides right down.  Allow your puppy/dog to rest undisturbed until they feel better (24 – 48 hours), but monitor body temperature and fluid intake.  In very rare cases, the fever may not respond to aspirin therapy, become extremely high and require IV fluid therapy to prevent dehydration.  During fever episodes, if your puppy/dog is not consuming any water or fluids, you can offer something more tempting such as chicken broth, or use a squeeze bottle or baster to gently force fluids into their mouth so they will drink.  Elevate their head slightly and squeeze a small amount of water into the side of their mouth so they can swallow it.  Large amounts of water given too quickly can cause them to choke and gag.  There is no infection, so antibiotics are not normally necessary or prescribed unless there is concern that the stressed dog may be secondarily infected.

As with amyloidosis, there is no test for FSF.  Once a test is developed for amyloidosis and FSF, a screening program can be developed to determine which Shar Pei dogs are normal, carriers or affected.  Then Shar Pei breeders can effectively eliminate this genetic disease from the breed through selective breeding practices.