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Calcium Pyrophosphate Dihydrate Deposition (CPPD)


KEY FACTS

Terminology

  • Metabolic arthropathy

  • Crowned dens syndrome: Pain due to calcium pyrophosphate dihydrate deposition around dens

Imaging

  • Soft tissue calcifications

    • Usually linear, occasionally globular

    • Seen in ligaments, discs, facet joint capsules, hyaline cartilage

    • Horseshoe-shaped calcification around dens

  • Erosions of odontoid process, vertebral endplates

    • Usually sharply demarcated, often corticated

  • MR findings nonspecific

    • Calcium usually not visible, low signal intensity on all sequences

    • Soft tissue mass surrounding dens

    • Erosions of dens, vertebral endplates

Top Differential Diagnoses

  • Degenerative disc disease

  • Rheumatoid arthritis, adult

  • Osteomyelitis, pyogenic

  • Seronegative spondyloarthropathy

  • Hemodialysis arthropathy

  • Hyperparathyroidism

  • Gout

Clinical Issues

  • Acute attack of pain ± fever ± radiculopathy

    • Almost any joint may be involved

  • Other

    • Chronic back pain common

    • Myelopathy due to cord compression

Diagnostic Checklist

  • CT useful in distinguishing from rheumatoid arthritis, which does not calcify

Sagittal T1WI MR shows a large pseudopannus posterior to the odontoid process
with moderately severe effacement of the ventral thecal sac and mild mass effect upon the cervicomedullary junction. A large degenerative cyst is present in the odontoid process
.

Axial bone CT shows marked thickening of the retroodontoid soft tissues
with scattered calcifications
that are consistent with a diagnosis of calcium pyrophosphate dihydrate deposition (CPPD).

Sagittal bone CT shows severe CPPD at the craniocervical junction. There is a large calcified mass posterior to the dens
and severe odontoid erosion. Calcification is seen in the ligamentum flavum
and intervertebral discs
.

Axial CTA study shows multiple punctate foci of calcification
within a large soft tissue mass posterior to the odontoid process, which causes cord compression
.

TERMINOLOGY

Abbreviations

  • Calcium pyrophosphate dihydrate deposition (CPPD) disease

Definitions

  • Pseudogout: Acute painful episode due to CPPD

    • Terms with “pseudo” should generally be abandoned in favor of CPPD

  • Crowned dens syndrome: Pain due to calcium pyrophosphate crystal deposition around dens

IMAGING

General Features

  • Best diagnostic clue

    • Linear calcific deposits in disc, ligament, hyaline cartilage, or joint capsule

  • Location

    • Cervical, thoracic, or lumbar spine

      • –

        Calcification in ligaments, discs, facet joint capsules, hyaline cartilage

    • Sacroiliac joints, pubic symphysis

    • Peripheral skeleton: Most common in knee and triangular fibrocartilage of wrist

    • Spine involvement often seen in absence of peripheral skeleton involvement

Radiographic Findings

  • Radiography

    • Linear disc calcifications

    • Majority of cases also show calcification in pubic symphysis &/or triangular fibrocartilage of wrist

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