Abstract
A breed-specific polymyositis is frequently observed in the Hungarian Vizsla. Beneficial clinical response to immunosuppressive therapies has been demonstrated which points to an immune-mediated aetiology. Canine inflammatory myopathies share clinical and histological similarities with the human immune-mediated myopathies. As MHC class II associations have been reported in the human conditions we investigated whether an MHC class II association was present in the canine myopathy seen in this breed. 212 Hungarian Vizsla pedigree dogs were stratified both on disease status and degree of relatedness to an affected dog. This generated a group of 29 cases and 183 “graded” controls: 93 unaffected dogs with a first degree affected relative, 44 unaffected dogs with a second degree affected relative, and 46 unaffected dogs with no known affected relatives. Eleven DLA class II haplotypes were identified, of which, DLA-DRB1*02001/DQA1*00401/DQB1*01303, was at significantly raised frequency in cases compared to controls (OR = 1.92, p = 0.032). When only control dogs with no family history of the disease were compared to cases, the association was further strengthened (OR = 4.08, p = 0.00011). Additionally, a single copy of the risk haplotype was sufficient to increase disease risk, with the risk substantially increasing for homozygotes. There was a trend of increasing frequency of this haplotype with degree of relatedness, indicating low disease penetrance. These findings support the hypothesis of an immune-mediated aetiology for this canine myopathy and give credibility to potentially using the Hungarian Vizsla as a genetic model for comparative studies with human myositis.
Highlights
IntroductionA polymyositis (an immune-mediated form of generalised inflammatory myopathy) with specific characteristics is being increasingly observed in the Hungarian Vizsla dog [1,2]
A polymyositis with specific characteristics is being increasingly observed in the Hungarian Vizsla dog [1,2]
This study provides evidence for an association between an MHC class II haplotype with the development of polymyositis in Hungarian Vizslas
Summary
A polymyositis (an immune-mediated form of generalised inflammatory myopathy) with specific characteristics is being increasingly observed in the Hungarian Vizsla dog [1,2]. In one study, using three dogs, CD4+ T-cells were found in excess of CD8+ T-cells which advocates a divergent aetiology to other cases of canine polymyositis [1]. Other diagnostic tests, such as serum creatine kinase concentration, electromyography, fluoroscopy of the oesophagus, and Magnetic Resonance Imaging (MRI) of affected muscle groups can aid in diagnosis. Immunosuppressive drug therapies have been used to treat the disease and dogs appear to respond well [3] This has furthered support for the hypothesis of an immune-mediated aetiology for myositis in this particular breed
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