The detail information of Familial Shar-Pei Fever
Basic Information

Another Name: Periodic Fever Syndrome

Abbreviated Name: FSF

Disease Description: Familial Shar-Pei Fever (FSF) dogs are in a constant state of inflammation.

Inherit Mode: -

Disease Symptom: If your pet has a fever and swollen joints, it is highly recommended to visit the veterinarian promptly in order to investigate the reason for the illness. Other symptoms that may accompany an episode of Familial Shar-Pei Fever are as follows: Swollen, painful muzzle; Swelling in wrists and lips, which may feel hot ;Reluctance to move ;Pain in the abdomen; Vomiting; Diarrhea; Shallow breathing; A stance that shows obvious discomfort, such as an arched back

Disease Cause: The cause of FSF is various genetic factors: A genetic defect in the immune system can lead to an overactive immune response. The mutated genes upset the regulation of inflammatory chemical messengers in the body. A genetic mutation resulting in excessive wrinkles, causing overproduction of hyaluronan, which can deregulate normal inflammatory processes (fever, redness and swelling). This means, in general, that dogs with extra wrinkling are suspected to be more apt to have FSF.

Disease Diagnose: To begin, the veterinarian will perform diagnostic tests to rule out other illnesses by exclusion. Checking for tick-borne diseases is common, along with testing for autoimmune disorders. Urinalysis and blood chemistry will be included. Unfortunately, at present there are no DNA blood tests definitive to FSF. Studies are currently underway with the hopes of developing a screening method. Blood test results will show an increased white cell count and a hike in liver enzymes during a fever episode.

Treat Method: Keeping your dog’s fever down is paramount to this illness. Aspirin may be given upon recommendation of the veterinarian. Be careful to administer only the amount prescribed. Aspirin may be given for several days in order to combat the fever, and to prevent a rebound of symptoms. If your pet is in the midpoint of a severe episode, IV support may be required. Anti-inflammatories will be given if pain is noted.

Breeder Advice: -

Disease Description Source: Link

Associated Diseases
Disease Name Other Name Mode of inheritance Link ID Possible OMIM ID Gene
Periodic Fever Syndrome Hyaluronanosis; Familial Shar-Pei fever; Hereditary cutaneous mucinosis; Canine Autoinflammatory Disease (AID); Shar-Pei Autoinflammatory Disease (SPAID) Autosomal HAS2 MTBP
Associated Breeds
iDog Breed Number Breed Name Personality Height Weight Breed Source
CB73 Chinese Shar-Pei Steadfastly devoted to loved ones, but standoffish and lordly toward strangers 45.7-50.8 cm 20.4-27.2 kg China
References
2017 Metzger,J.,Nolte,A.,Uhde,A.K.,Hewicker-Trautwein,M.,Distl,O.: :
Whole genome sequencing identifies missense mutation in MTBP in Shar-Pei affected with Autoinflammatory Disease (SPAID). BMC Genomics 18:348, 2017.
Pubmed reference: 28472921 . DOI: 10.1186/s12864-017-3737-z .
2016 Olsson,M.,Kierczak,M.,Karlsson,Å.,Jabłońska,J.,Leegwater,P.,Koltookian,M.,Abadie,J.,DeCitres,C.D.,Thomas,A.,Hedhammar,Å.,Tintle,L.,Lindblad-Toh,K.,Meadows,J.R.: :
Absolute quantification reveals the stable transmission of a high copy number variant linked to autoinflammatory disease. BMC Genomics 17:299, 2016.
Pubmed reference: 27107962 . DOI: 10.1186/s12864-016-2619-0 .
2014 Metzger,J.,Distl,O.: :
A study of Shar-Pei dogs refutes association of the 'meatmouth' duplication near HAS2 with Familial Shar-Pei Fever. Anim Genet 45:763-4, 2014.
Pubmed reference: 25040095 . DOI: 10.1111/age.12193 .
2013 Olsson,M.,Tintle,L.,Kierczak,M.,Perloski,M.,Tonomura,N.,Lundquist,A.,Murén,E.,Fels,M.,Tengvall,K.,Pielberg,G.,DufauredeCitres,C.,Dorso,L.,Abadie,J.,Hanson,J.,Thomas,A.,Leegwater,P.,Hedhammar,A.,Lindblad-Toh,K.,Meadows,J.R.: :
Thorough Investigation of a Canine Autoinflammatory Disease (AID) Confirms One Main Risk Locus and Suggests a Modifier Locus for Amyloidosis. PLoS One 8:e75242, 2013.
Pubmed reference: 24130694 . DOI: 10.1371/journal.pone.0075242 .
2011 Docampo,M.J.,Zanna,G.,Fondevila,D.,Cabrera,J.,López-Iglesias,C.,Carvalho,A.,Cerrato,S.,Ferrer,L.,Bassols,A.: :
Increased HAS2-driven hyaluronic acid synthesis in shar-pei dogs with hereditary cutaneous hyaluronosis (mucinosis). Vet Dermatol 22:535-45, 2011.
Pubmed reference: 21718367 . DOI: 10.1111/j.1365-3164.2011.00986.x .
2011 Olsson,M.,Meadows,J.R.,Truvé,K.,RosengrenPielberg,G.,Puppo,F.,Mauceli,E.,Quilez,J.,Tonomura,N.,Zanna,G.,Docampo,M.J.,Bassols,A.,Avery,A.C.,Karlsson,E.K.,Thomas,A.,Kastner,D.L.,Bongcam-Rudloff,E.,Webster,M.T.,Sanchez,A.,Hedhammar,A.,Remmers,E.F.,Andersson,L.,Ferrer,L.,Tintle,L.,Lindblad-Toh,K.: :
A novel unstable duplication upstream of HAS2 predisposes to a breed-defining skin phenotype and a periodic fever syndrome in Chinese Shar-Pei dogs. PLoS Genet 7:e1001332, 2011.
Pubmed reference: 21437276 . DOI: 10.1371/journal.pgen.1001332 .
2010 Akey,JM.,Ruhe,AL.,Akey,DT.,Wong,AK.,Connelly,CF.,Madeoy,J.,Nicholas,TJ.,Neff,MW.: :
Tracking footprints of artificial selection in the dog genome. Proc Natl Acad Sci U S A 107:1160-5, 2010.
Pubmed reference: 20080661 . DOI: 10.1073/pnas.0909918107 .
2009 Zanna,G.,Docampo,M.J.,Fondevila,D.,Bardagí,M.,Bassols,A.,Ferrer,L.: :
Hereditary cutaneous mucinosis in shar pei dogs is associated with increased hyaluronan synthase-2 mRNA transcription by cultured dermal fibroblasts. Vet Dermatol 20:377-82, 2009.
Pubmed reference: 20178474 . DOI: 10.1111/j.1365-3164.2009.00799.x .
1992 Rivas,AL.,Tintle,L.,Kimball,ES.,Scarlett,J.,Quimby,FW.: :
A canine febrile disorder associated with elevated interleukin-6. Clin Immunol Immunopathol 64:36-45, 1992.
Pubmed reference: 1606750 .