Hereditary non-neuropathic systemic amyloidosis is a rare autosomal dominant disease in which amyloid deposition in the gastrointestinal tract is usually fatal by the fifth decade. The disease can be caused by several proteins that undergo pathologic aggregation. In some families, the disease is caused by mutations in the apolipoprotein AI gene, whereas in others the cause is mutations in apolipoprotein AII, lysozyme, or the α-chain of the protein fibrinogen A. This disease also sometimes is called Ostertag, Ostertag Type A, or familial visceral amyloidosis.