Download Familial Shar-Pei Fever

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.