May be systemic (involving multiple organs in 80%) or localized (in single organ or tissue in 20%)
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
•
CT findings
Acute disease: Enlarged kidneys
Chronic disease: Small kidneys with cortical thinning
Bowel wall thickening, distension, intramural hemorrhage, and intussusceptions
–
Wall thickening can be focal or diffuse ± calcification
Hepatosplenomegaly
–
Patchy or diffuse hypoattenuation ± calcification
Retroperitoneal soft tissue infiltration ± calcification resembling retroperitoneal fibrosis
•
MR findings
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
TERMINOLOGY
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
Related
Diagnostic Imaging_ Gastrointes - Michael P Federle