Focal amyloid involvement may be low signal on T2WI
• Fluoroscopic findings
Stomach: Thick folds (which may appear nodular or mass-like) ± calcification
Small intestine: Symmetrical thickening of folds, impaired motility, and slow transit
Colon: Luminal narrowing, loss of haustrations, and thickened transverse folds
CLINICAL ISSUES
• Symptoms depend on site of protein deposition
• Most common sites are kidneys, heart, liver, and GI tract
• Renal failure due to amyloid deposition in kidneys is most common cause of death (50%)
• GI tract involvement: GI bleeding, intestinal dysmotility, malabsorption, and protein-losing gastroenteropathy
(Left) Axial NECT shows an abnormally hypodense and mildly enlarged liver and spleen in this patient with biopsy-proven primary amyloid of the liver.
(Right) Spot film from a small bowel follow-through shows diffuse fold thickening throughout the jejunum. This is a very nonspecific finding, one that required biopsy for the diagnosis of amyloidosis.
(Left) Axial CECT in a patient with amyloidosis demonstrates rind-like soft tissue thickening surrounding the left kidney with internal calcification .
(Right) Coronal CECT in the same patient demonstrates similar thickening and calcification surrounding the left kidney, as well as a more focal soft tissue mass (amyloidoma) in the adjacent retroperitoneum.
TERMINOLOGY
Synonyms
• Systemic amyloidosis
Definitions
• Group of diseases characterized by deposition of abnormal protein (amyloid) in various tissues
May be systemic (involving multiple organs in 80%) or localized (in single organ or tissue in 20%)
Classified based on type of protein being deposited
– 30 different amyloidogenic proteins in humans
• Primary: Due to monoclonal plasma cell dyscrasia (associated with no other underlying disease)
• Secondary: Develops in setting of a number of different chronic inflammatory diseases
IMAGING
General Features
• Best diagnostic clue
Small bowel wall thickening and hypomotility in patient with underlying chronic illness
• Location
Small intestine, stomach, colorectum, esophagus, liver, kidney, heart
• Morphology
Nonspecific small bowel fold thickening and hypomotility
Buy Membership for Radiology Category to continue reading. Learn more here