Familial Shar-Pei Fever

This familial immunoreactive disorder is found only in Chinese Shar-Pei dogs, characterized by episodic fever and swollen hocks (back of the leg). If left untreated, it can lead to excessive amyloid accumulation throughout the body and subsequent kidney and liver failure. 

Symptoms and Types

• Fever (for up to 24-36 hours)
• Loss of appetite (anorexia)
• Lethargy
• Dehydration
• Swollen hocks
• Weight loss
• Fluid-filled soft tissue swellings involving one or more joints
• Joint and abdominal pain
• Reluctance to move
• Hunched posture
• Heavy breathing (tachypnea)  

Causes

Any chronic infection, inflammation, immune-mediated disease, or cancer can cause reactive or secondary amyloidosis. However, dysregulation of immune and inflammatory processes are also thought to predispose shar-pei dogs to this disorder.

Diagnosis

You will need to give a thorough history of your dog’s health, including the onset and nature of the symptoms, to the veterinarian. He or she will then perform a complete physical examination as well a biochemistry profile, urinalysis, complete blood count (CBC).

Other tests which can be done to rule out or identify the underlying disease causing the amyloidosis include Ehrlichia and Borrelia serology, Heartworm exams, Coombs' test, rheumatoid arthritis factor tests, and a clotting profile, which can help rule out liver disease. Chest X-rays and abdominal X-rays and ultrasounds are used by the veterinarian to look for liver and kidney abnormalities, and an analysis of synovial fluid may show acute inflammation.

X-rays of the joints will show a swelling of the soft tissues around the joint with no bony involvement. An abdominal ultrasound is useful to examine the consistency of the liver and kidneys.

Lastly, if amyloid is being deposited in the kidneys the urine protein:creatinine ratio can increase from less than one (normal) to greater than thirteen.

Treatment

The course of treatment will depend on the underlying cause of the disorder. If the dog is undergoing pain and fever responsive to NSAIDS, for example, it may be treated on an outpatient basis. Conversely, Shar-Pei dogs showing anorexia, fever, marked lameness or nonspecific pain, vomiting or diarrhea, fluid in the abdomen, or episodes of cholestasis (bile flow blockage in the liver) should be treated on an inpatient basis. And those undergoing organ failure or suffering from a blood clot or portal and renal vein thrombosis should be placed in intensive care immediately.

Antibiotics, fluid therapy, oxygen therapy, and blood transfusions are also provided on a case-by-case basis. For DIC or other coagulopathies fresh frozen plasma can be given. And severely hypoalbuminec patients with ascites may receive human serum albumin transfusions.

Living and Management

Unfortunately, there is no cure for familial shar-pei amyloidosis. Therapy may decrease the deposition of amyloid, but often the condition has progressed beyond the stage at which medication is beneficial. In addition, because of the genetic nature of the disorder, your veterinarian will recommend against breeding the affected Shar-Pei.