Familial protein-losing enteropathy and protein-losing nephropathy in SoftCoated Wheaten Terriers: 222 cases (1983-1997)

Records and pedigrees of Sofi Coated Wheaten Terriers (SCWT) with protein-l osing enteropathy (PLE) or protein-losing nephropathy (PLN) were studied re trospectively. Criteria for inclusion were defined based on analysis of blo od (panhypoproteinemia for PLE, hypoalbuminemia for PLN) and urine (protein uria for PLN) and histopathologic examination of tissue. Two hundred twenty -two affected dogs (female:male ratio = 1.6, P < .001) were clinically iden tified. Dogs were diagnosed with PLE earlier (P < .005: mean +/- SD age: 4. 7 +/- 2.6 years, n = 76) than with PLN (6.3 +/- 2.0 years, n = 84) or with both diseases (5.9 +/- 2.2 years, n = 62). Clinical signs included vomiting , diarrhea, weight loss, pleural and peritoneal effusions. and less commonl y thromboembolic disease. Dogs with PLE generally had panhypoproteinemia an d hypocholesterolemia: intestinal lesions included inflammatory bowel disea se, dilated lymphatics, and lipogranulomatous lymphangitis. Dogs with PLN g enerally had hypoalbuminemia, proteinuria. hypercholesterolemia, and azotem ia: renal lesions typically showed chronic glomerulonephritis/ glomeruloscl erosis, and less commonly endstage renal disease. Dogs with combined PLE/PL N had intermediate mean values (P < .001) for serum total protein, albumin, globulin, and cholesterol but had a higher mean urine protein:creatinine r atio than did PLN dogs (P < .05); intestinal and renal lesions in these dog s were similar to those in the other groups. Two dogs had incidental mild r enal dysplasia. Pedigree analysis from 188 dogs demonstrated a common male ancestor, although the mode of inheritance is unknown. Both PLE and PLN are common diseases in this small breed population. The prognosis is poor. Com pared with previously reported intestinal and renal diseases in dogs, a new , distinctive familial predisposition for both PLE and PLN has been recogni zed in the SCWT breed.