Malabsorption and/or Pseudo-Obstruction in Intestinal Amyloidosis

Pathology:

(1) amyloid protein in muscularis propria and other layers of the intestinal wall

(2) involvement of the myenteric plexus

Clinical features:

(1) intermittent obstructive symptoms

(2) diarrhea and steatorrhea with deterioration of nutritional status

A biopsy of small bowel would have a high rate of false negative.

The diagnosis requires clinical acumen and the presence of some other clue:

(1) presence of a disease associated with amyloidosis such as multiple myeloma

(2) presence of myopathy, neuropathy or carpal tunnel syndrome

(3) cardiac and/or renal dysfunction

(4) skin deposits

(5) macroglossia