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Information

 Bone and Soft-Tissue Disorders

= extracellular deposition of a chemically diverse group of protein polysaccharides in body tissues (abnormal folding + assembly of β2-microglobulin); tends to form around capillaries + endothelial cells of larger blood vessels causing ultimately vascular obliteration with infarction

β2-microglobulin = low-molecular-weight serum protein not filtered by standard dialysis membranes

Incidence: 6–10÷100,000 annually in USA + western Europe

Path: stains with Congo red

At risk: patients on long-term hemodialysis

Amyloid Arthropathy  !!navigator!!

= synovial-articular pattern of amyloidosis

Associated with: chronic hemodialysis, plasma cell dyscrasia, rheumatoid arthritis, familial Mediterranean fever, chronic osteomyelitis

Location: cervical spine, hip, shoulder, elbow, knee, wrist; bilateral

  • juxtaarticular soft-tissue masses (amyloid deposited in synovium, joint capsule, tendons, ligaments) ± extrinsic osseous erosion
  • mild periarticular osteopenia
  • subchondral cysts + well-defined sclerotic margin
  • joint effusion
  • joint space preserved until late in course of disease
  • subluxation of proximal humerus + femoral neck

MR:

  • extensive deposition of abnormal soft tissue of low to intermediate SI on T1WI + T2WI covering synovial membrane, filling subchondral defects, extending into periarticular tissue
  • low SI on T1WI + T2WI subjacent to vertebral endplates in lower cervical >thoracic / lumbar spine
  • joint effusion

DDx: inflammatory arthritis, PVNS

Diffuse Amyloid Marrow Deposition  !!navigator!!

  • generalized osteoporosis
  • coarse trabecular pattern (DDx: sarcoidosis)
  • pathologic collapse of vertebral body may occur

Amyloidoma  !!navigator!!

= localized destructive lesion of amyloidosis (rarest form)

Location: appendicular >axial skeleton

  • focal medullary lytic lesion with endosteal scalloping (± secondary invasion + erosion of articular bone)

Cx: pathologic fracture


 Outline