Inheritance of renal amyloidosis in Chinese Shar-pei dogs

Renal amyloidosis (RA) and recurrent fever of unknown origin (RFUO) are characteristics of familial Mediterranean fever (FMF), a human disorder inherited as an autosomal-recessive trait. Although no animal model has been established for FMF, a similar syndrome of RFUO and RA has been reported in Chinese Shar-pei (CSP) dogs. This report addresses two questions: (1) Is RA inherited in CSP dogs? (2) If it is, is it possible to hypothesize the type of inheritance involved? Two studies were conducted to answer these questions: a historical cross-sectional comparison, which included CSP and non-CSP dogs with RA; and a prospective study that included CSP dogs with RA, RFUO, or both. The cross-sectional comparison resulted in an odds ratio of 10 for RA in CSP dogs under 7 years of age. The prospective study of 28 dogs with RA or RFUO identified 20 that had RFUO and RA, three with RA alone, and five with RFUO alone. RFUO preceded RA in all cases with both conditions. The RFUO/RA combination was observed in both sexes. Four dogs with RFUO with or without RA were born to parents that either were alive at age 7 or had died because of conditions other than kidney failure/RA. When one parent was known to express one of these conditions, the prevalence of RA was between 25% and 50% among littermates.(ABSTRACT TRUNCATED AT 250 WORDS)