Objective-To estimate the prevalence of perinuclear antineutrophilic cytoplasmic autoantibodies (pANCA) in the serum of healthy Soft Coated Wheaten Terriers (SCWTs) in the United Kingdom and to identify potential risk factors and heritability patterns associated with a positive result for pANCA. Animals-188 SCWTs (age range, 18 months to 14.3 years). Procedures-Blood samples were obtained from SCWTs in various locations in England. Serum was tested for pANCA by use of an immunofluorescence assay, and total protein and albumin concentrations were determined. Pedigrees were evaluated to identify close relatives that had protein-losing enteropathy (PLE) or protein-losing nephropathy (PLN). Results-39 of 188 (20.7%) dogs, including young dogs, had positive results for pANCA. Dogs had significantly higher odds of having positive results for pANCA if they had at least 1 littermate that had PLE or PLN (odds ratio, 12.1) or if they had at least 1 full sibling from another litter known to be affected with PLE or PLN (odds ratio, 4.0). Conclusions and Clinical Relevance-This study revealed a high prevalence of pANCA in the serum of a representative sample of healthy SCWTs in the United Kingdom and a significant association between positive results for pANCA and a diagnosis of PLE or PLN in a sibling.