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Key Points

  • Disease summary:

    • The diagnostic for calcium pyrophosphate dihydrate (CPPD) crystal deposition is given after the identification of CPPD crystals in synovial fluid (SF) or in tissue sections.

    • The terminology of CPPD-related diseases is still not uniform; the following definitions are the most commonly used:

      • Pseudogout—acute clinical syndrome of synovitis associated with CPPD deposition

      • Pyrophosphate arthropathy—structural abnormality of cartilage and bone associated with articular CPPD deposition

      • Chondrocalcinosis (CC)—radiographic observation of calcification of fibro and/or hyaline articular cartilage

    • CPPD deposition is the main cause of radiographic articular calcification of the knee, that is, CC. The deposition of other calcium salts, such as hydroxyapatite, can also be the cause of articular calcification.

  • Hereditary basis:

    • CPPD deposition disease (CPDD) can occur as a familial monogenic disorder showing an autosomal dominant pattern of inheritance.

  • Differential diagnosis:

    • Certain metabolic diseases predispose to CPDD deposition such as hemochromatosis, Gitelman syndrome, and hyperparathyroidism. It is possible to screen these disorders by laboratory hematologic testing and evaluate elevated serum calcium, decreased serum phosphate, elevated parathyroid hormone or parathormone, elevated alkaline phosphatase, and increased serum ferritin. However, it is important to refer that these disorders have strong genetic component themselves. In Table 143-1, it is possible to see how to perform a genetic differential diagnosis.

Table 143-1Genetic Differential Diagnosis

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