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Calcium Pyrophosphate Deposition Disease

  • Terminology

    • Chondrocalcinosis – calcification of hyaline (articular) cartilage or fibrocartilage (menisci) or ligaments
      • Usually but not always due to calcium pyrophosphate
        • May also be seen with oxalosis
    • Pseudogout is an older clinical term referring to acute pain (similar to gout) but without response to the usual treatment for gout
    • CPPD – Deposition of crystals in the joint with or without chondrocalcinosis
  • Most common crystalline arthropathy
  • Prevalence
    • Widespread in older population
    • M:F = 3:2

  • Clinical findings

    • Intermittent attacks
      • May be mono-articular or polyarticular (more often)
        • Pain
        • Redness
        • Swelling

  • Types

    • Frequently occurs in association with osteoarthritis
    • Aging process with no known etiology
    • In association with metabolic diseases
      • Hyperparathyroidism
      • Hemochromatosis
      • Hypothyroidism
      • Hypomagnesemia
      • Hypophosphatasia
      • Ochronosis
  • Calcium pyrophosphate crystals may be recovered from synovial fluid (most often) or within leukocytes
    • Characteristic weakly positive birefringent diffraction pattern

  • Location

    • Knee
      • Especially meniscus
      • Cartilage of patellofemoral joint
    • Wrist
      • Triangular fibrocartilage in distal radioulnar joint bilaterally
    • Pelvis
      • Sacroiliac joint
      • Symphysis
    • Spine
      • Annulus fibrosis of lumbar intervertebral disk
        • Never in nucleus pulposus as in ochronosis
    • Shoulder
      • Glenoid
      • Hip
        • Labrum
      • Elbow
      • Ankle
      • Acromioclavicular joint

  • Imaging Findings

    • Pyrophosphate arthropathy resembles osteoarthritis
      • Joint space narrowing
      • Extensive subchondral sclerosis
    • Polyarticular chondrocalcinosis (in fibro- and hyaline cartilage)
    • In knee, disproportionate narrowing of patellofemoral joint
      • Large subchondral cysts are a hallmark
      • Numerous intra-articular bodies
        • Fragmentation of subchondral bone
    • In hand, beaklike projections from 2nd, 3rd metacarpal heads
      • Subchondral cysts (esp. carpal bones)
      • Unusual distribution of disease (radiocarpal/ulnar joint, patellofemoral joint)
      • SLAC - scapholunate advanced collapse
        • Caused by laxness of the ligaments and malpositioning of the scaphoid and lunate
        • May develop in 25% with CPPD but also occurs for other reasons
        • Radio-scaphoid, but not radio-lunate, joint is narrowed
        • Usually have a deep concave scaphoid fossa in distal radius in CPPD as opposed to SLAC from trauma
  • Treatment

    • Oral anti-inflammatory drugs (NSAIDs) and corticosteroid joint injections successful in shortening the length of pain and dysfunction of acute attacks of pseudogout
    • Treatments to prevent attacks, such as colchicine, may be effective
    • No treatment is available to dissolve the crystal deposits
    • Controlling inflammation helps to halt the progression of joint degeneration

Calcium Pyrophosphate Deposition Arthropathy

CPPD Arthropathy. There is chondrocalcinosis in the triangular fibrocartilage of the ulna (white arrow). There is narrowing of the radio-carpal joint and proximal migration of the capitate into the widened space between the scaphoid and the lunate (yellow arrow).
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CPPD Arthropathy

CPPD of hand and wrist - upper photo shows hook-like projections arising from radial aspect of metacarpal heads; lower photo shows SLAC-scapholunate advanced collapse with characteristic indentation in distal radius by scaphoid bone. There is also chondrocalcinosis of the triangular fibrocartilage of the distal ulna.

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Imaging of the Wrist and Hand: Gilula and Yin, W.B. Saunders, 1996.