Etiology

• Not known but genetic predisposition thought to be important together with a stress factor, enteric infection or dietary component being required to initiate clinical signs.
• Appears to be associated with an abnormal immune response by the alimentary tract.
• Response to oral antibiotics suggest that a loss of tolerance to GI microflora may be involved.

Specific

• Stress is frequently reported to initiate episodes of clinical disease.

• Genetic predisposition?

Pathophysiology

• Undefined immune response within the alimentary tract that results in a diffuse hypertrophic gastropathy, although 50% of cases may show evidence of atrophic gastropathy, and intestinal inflammation.
• Severe protein losing enteropathy characterized by marked abnormalities in mucosal architecture and increased cellular infiltration involving involving the small intestine, not the colon.
• Marked hyperglobulinemia and increased serum IgA.
• Diarrhea associated with small intestinal lymphocytic plamacytic enteritis which can be severe enough to result in hypoalbuminemia and therefore can be classified as a protein losing enteropathy.
• Diarrhea may in some part be due to hypersecretion of gastric acid inactivating pancreatic enzymes.
• Some patients also have mild glomerular nephritis Glomerulonephritis associated with immune complex deposition, ulcerative dermatitis of pinnae, all suggesting an immune mediated disease.

Timecourse

• Once clinical signs develop the disease is generally progressive with most dogs being euthanazed within two years of diagnosis, although reports of dogs surviving for five or more years have been documented.

Epidemiology

• Reported to occur in 3% of the Basenji population.
• Almost always seen within the first three years of life.
• Asymptomatic carriers appear more common.