Familial Shar-Pei fever (FSF) is a disease that belongs to the autoinflammatory disease complex "SPAID" (Shar-Pei Autoinflammatory Disease). The clinical signs of this disease present with high fever, inflammation of the ears, joints and skin. Often only some of the described symptoms occur in the affected animals. The characteristic recurrent episodes of fever resemble familial Mediterranean fever in humans. The recurrent febrile episodes are accompanied by swollen joints and/or a thickened snout in some animals. Analogous to humans, the recurrent febrile episodes are considered possible precursors for secondary amyloid A amyloidosis (AA amyloidosis). However, other processes such as persistent infections, inflammation, or neoplastic disease may also promote secondary AA amyloidosis due to chronic accumulation of inflammatory products. In AA amyloidosis, abnormally altered protein (amyloid) is deposited extracellularly in the kidney or, in the systemic form, in various organs such as the kidney, heart, liver, pancreas, adrenal gland, or intestinal submucosa and can lead to multiple organ damage. Deposits in the kidneys of Shar-Pei have the peculiarity that the amyloid is predominantly found in the renal medulla (medullary interstitium), while in other dog breeds it is more common in the area of the renal cortex (glomeruli). Affected dogs show non-specific symptoms such as anorexia, nausea, lethargy and weight loss. In particular, the so-called "meatmouth" Shar-Pei type, which exhibits increased skin wrinkling and heavily padded fangs, appears to have a predisposition to recurrent fevers similar to familial Mediterranean fever in humans.
The genetic cause of SPAID in the Shar-Pei was identified during a research project conducted by the Institute of Animal Breeding and Genetics with the support of Shar-Pei breeders and Shar-Pei breed clubs (1st German Shar-Pei Club 1985 e.V., Shar-Pei Freunde Deutschland and World of Shar-Pei). This work led to completely new results, which were not known before and especially not found in earlier work from Sweden. For the first time, an explanation for the development of the inflammatory symptoms and thus the trigger for SPAID could be found. These completely new results represent a major step forward in SPAID research, as they provide for the first time a functional explanation for the development of SPAID disease. The new test procedure, which was developed at the Institute of Animal Breeding and Genetics at the University of Veterinary Medicine Hanover Foundation and differs fundamentally from the test previously offered in Sweden and in the USA, therefore does not include the test based on copy number variations (CNVs) in a region on dog chromosome 13 published by Olsson et al. (2016).
This new test is now available to all breeders and dog owners to help reduce the risk of SPAID.
In addition, you can continue to support Shar-Pei research by donating samples- whether or not you want to do the paid test in addition is up to you. Please mark the desired service on the submission form and send an EDTA blood sample to the address indicated.
If you send a larger amount of samples (in one shipping unit), the price per test will be reduced:
from 10 samples: 10% discount
from 30 samples: 20% discount
For members of the 1st DSPC the test is free of charge until 31.05.2017, after that we charge according to the standard price.
The results to date represent an important step in elucidating the disease in Shar-Pei and should help breeders make breeding decisions in the future. Research on the Shar-Pei continues. The new findings can be used to explore the mechanisms of SPAID and potential avenues for therapeutic approaches.
Dog owners or veterinarians who have already sent in a sample are kindly asked to fill in the questionnaire and send it to us (also by e-mail or fax)!
POAG/PLL in Shar-Pei: You can also request this test via the SPAID submission form.
You can find the documents to fill out here:
For samples already sent in, for which the questionnaire is to be submitted later: