Disease overview

Periodic Fever Syndrome, also known as Shar-Pei Autoinflammatory Disease, is a inherited syndrome that causes recurrent episodes of fever and inflammation with no known autoimmune or infectious cause. It is thought that there is a buildup of a substance that acts as a trigger, similar to a pathogen, that over stimulates the immune system.

Gene name

HAS2

Inheritance

Autosomal dominant with incomplete penetrance

Signs and symptoms

Clinical signs may include fever, swollen limbs and snout, lack of appetite, and listlessness. Signs typically present before 18 months of age, but timing of presentation can vary.

How is it diagnosed

A veterinarian will conduct a thorough exam, including a review of health history and any clinical signs. Laboratory tests and other diagnostics may be required for a definitive diagnosis. Genetic testing is necessary to determine if there is an underlying genetic cause, which can assist veterinarians with diagnosis and help breeders identify affected and carrier dogs.

Breeds at higher risk

Citations

Olsson M, Meadows JR, Truvé K, Rosengren Pielberg G, Puppo F, Mauceli E, Quilez J, Tonomura N, Zanna G, Docampo MJ, Bassols A, Avery AC, Karlsson EK, Thomas A, Kastner DL, Bongcam-Rudloff E, Webster MT, Sanchez A, Hedhammar A, Remmers EF, 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. 2011 Mar;7(3):e1001332. doi: 10.1371/journal.pgen.1001332. Epub 2011 Mar 17. PMID: 21437276; PMCID: PMC3060080.

Disclaimer

Disease diagnosis and treatment should always be performed by a veterinarian. The following information is for educational purposes only.