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For further information, see CMDT Part 20-03: Crystal Deposition Arthritis

Key Features

  • CPPD in fibrocartilage and hyaline cartilage (chondrocalcinosis) can cause an acute crystal-induced arthritis ("pseudogout"), a degenerative arthropathy, and a chronic inflammatory polyarthritis ("pseudorheumatoid arthritis")

  • Hyperparathyroidism, familial hypocalcuric hypercalcemia, hemochromatosis, and hypomagnesemia confer risk of CPPD, but most cases have no associated condition

  • Usually seen in individuals age 60 yr and older

  • Pseudogout, like gout, frequently develops 1–2 days after major surgery

  • Familial CPPD is uncommon, but identification of the affected gene as a regulator of inorganic pyrophosphate transport underscores the importance of pyrophosphate homeostasis in the development of CPPD

Clinical Findings

  • CPPD can be asymptomatic

  • Pseudogout

    • Characterized by acute, recurrent and rarely chronic arthritis involving large joints (most commonly the knees and the wrists)

    • Almost always accompanied by radiographic chondrocalcinosis of the affected joints

    • The crowned dens syndrome

      • Caused by pseudogout of the atlantoaxial junction associated with "crown-like" calcifications around the dens

      • Manifests with severe neck pain, rigidity, and high fever that can mimic meningitis or polymyalgia rheumatica

    • The degenerative arthropathy associated with CPPD can involve joints not usually affected by osteoarthritis (eg, glenohumeral joint, wrist, patellofemoral compartment of the knee)

    • The "pseudorheumatoid arthritis" of CPPD affects the metacarpophalangeal joints and wrists

Diagnosis

  • CPPD may be detected as incidental chondrocalcinosis on radiographs

  • Identification of calcium pyrophosphate crystals in joint aspirates is diagnostic

  • Radiographs demonstrate chondrocalcinosis and degenerative changes (such as asymmetric joint space narrowing and osteophyte formation) in both degenerative arthropathy and "pseudorheumatoid arthritis"

  • With light microscopy, the rhomboid-shaped crystals differ from the needle-shaped gout crystals

  • A red compensator is used for positive identification, since pseudogout crystals are blue when parallel and yellow when perpendicular to the axis of the compensator

  • Urate crystals give the opposite pattern

Treatment

  • NSAIDs are used for acute episodes

  • Colchicine, 0.6 mg orally once or twice daily, is more effective for prophylaxis than for acute episodes

  • Aspiration of the inflamed joint and intra-articular injection of triamcinolone, 10–40 mg, depending on size of the joint, is valuable in resistant cases

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