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Clinical manifestations and diagnosis of calcium pyrophosphate crystal deposition disease

INTRODUCTION

Precipitation of crystals of calcium pyrophosphate dihydrate (CPPD) in connective tissues may be asymptomatic or associated with several clinical syndromes. These disorders comprise the spectrum of calcium pyrophosphate crystal deposition disease [1-3]. Alternative names representing specific clinical or radiographic features have retained some popularity, but are of limited applicability. These include pseudogout, chondrocalcinosis, and pyrophosphate arthropathy:

  • Pseudogout accurately describes acute attacks of CPPD crystal-induced synovitis, which clinically resemble urate gout. However, the majority of individuals with CPPD crystal deposition never experience such episodes.
  • Chondrocalcinosis refers to radiographic calcification in hyaline and/or fibrocartilage. It is commonly present in patients with CPPD crystal deposition disease but is neither absolutely specific for CPPD nor universal among affected patients.
  • Pyrophosphate arthropathy is sometimes applied to the joint disease or radiographic abnormalities accompanying CPPD crystal deposition. Although this name has little justification in terms of the pathologic anatomy of the disorder, evidence of aberrant inorganic pyrophosphate metabolism in the pathogenesis of CPPD crystal deposition disease may legitimize use of this term.

The clinical manifestations and diagnosis of CPPD crystal deposition disease are discussed here. The pathogenesis and etiology of this disorder and treatment of CPPD deposition diseases, including pseudogout, are discussed separately. (See "Pathogenesis and etiology of calcium pyrophosphate crystal deposition disease" and "Treatment of calcium pyrophosphate crystal deposition disease".)

CLINICAL MANIFESTATIONS

A clinical classification of CPPD crystal deposition disease has emerged from the studies of McCarty [1] and colleagues [2,3]. This classification draws particular attention to the capacity of this disorder to mimic virtually any type of arthritis. The clinical spectrum of CPPD crystal deposition disease includes:

  • Asymptomatic disease
  • Pseudogout
  • Pseudo-rheumatoid arthritis
  • Pseudo-osteoarthritis, with or without superimposed acute attacks
  • Pseudo-neuropathic joint disease

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Last literature review version 18.2: May 2010
This topic last updated: August 20, 2009
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References
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  1. McCarty, DJ. Calcium pyrophosphate dihydrate crystal deposition disease--1975. Arthritis Rheum 1976; 19 Suppl 3:275.
  2. Rosenthal, AK, Ryan, LM, McCarty, DJ. Calcium pyrophosphate crystal deposition disease, pseudogout, and articular chondrocalcinosis. In: Arthritis and Allied Conditions, 15th ed, Koopman, WJ, Moreland, LW (Eds), Lippincott Williams & Wilkins, Philadelphia, 2005, p. 2373.
  3. Rosenthal, AK. Pseudogout: presentation, natural history, and associated conditions. In: Crystal-induced Arthropathies. Gout, Pseudogout and Apatite-associated Syndromes, Wortmann, RL, Schumacher, HR Jr, Becker, MA, Ryan, LM (Eds), Taylor & Francis Group, New York, 2006. p. 99.
  4. Ellman, MH, Levin, B. Chondrocalcinosis in elderly persons. Arthritis Rheum 1975; 18:43.
  5. Dieppe, PA, Aleander, GJM, Jones, HE, et al. Pyrophosphate arthropathy: A clinical and radiological study of 105 cases. Ann Rheum Dis 1982; 41:371.
  6. Bilezikian, J, Auerbach, G, Connor, T, et al. Pseudogout after parathyroidectomy. Lancet 1973; 1:445.
  7. Luzar, MJ, Altawil, B. Pseudogout following intra-articular injection of sodium hyaluronate. Arthritis Rheum 1998; 41:939.
  8. Malnick, SDH, Ariel-Ronen, S, Evron, E, Sthoeger, ZM. Acute pseudogout as a complication of pamidronate. Ann Pharmacother 1997; 31:499.
  9. Sandor, V, Hassan, R, Kohn, E. Exacerbation of pseudogout by granulocyte colony-stimulating factor. Ann Intern Med 1996; 125:781.
  10. Bong, D, Bennett, R. Pseudogout mimicking systemic disease. JAMA 1981; 246:1438.
  11. Bennett, RM, Mall, JC, McCarty, DJ. Pseudogout in acute neuropathic arthropathy: A clue to pathogenesis. Ann Rheum Dis 1974; 33:563.
  12. Gerster, JC, Vischer, TL, Fallet, GH. Destructive arthropathy in generalized osteoarthritis with articular chondrocalcinosis. J Rheumatol 1975; 2:265.
  13. Richards, AJ, Hamilton, EB. Destructive arthropathy in chondrocalcinosis articularis. Ann Rheum Dis 1974; 33:196.
  14. Cabre, P, Pascal-Moussellard, H, Kaidomar, S, et al. Six cases of cervical ligamentum flavum calcification in Blacks in the French West Indies. Joint Bone Spine 2001; 68:158.
  15. Muthukumar, N, Karuppaswamy, U. Tumoral calcium pyrophosphate dihydrate deposition disease of the ligamentum flavum. Neurosurgery 2003; 53:103.
  16. Gerster, JC, Lagier, R, Boivin, G, Schneider, C. Carpal tunnel syndrome in chondrocalcinosis of the wrist. Clinical and histologic study. Arthritis Rheum 1980; 23:926.
  17. Bjelle, A, Crocker, P, Willoughby D. Ultra-microcrystals in pyrophosphate arthropathy. Acta Med Scand 1980; 207:89.
  18. Gordon, C, Swan, A, Dieppe, P. Detection of crystals in synovial fluids by light microscopy: sensitivity and reliability. Ann Rheum Dis 1989; 48:737.
  19. Schumacher, HR, Sieck, MS, Rothfuss, S, et al. Reproducibility of synovial fluid analysis. A study among four laboratories. Arthritis Rheum 1986; 29: 770.
  20. Hasselbacher, P. Variation in synovial fluid analysis by hospital laboratories. Arthritis Rheum 1987; 30:637.
  21. Yamakawa, K, Iwasaki, H, Masuda, I, et al. The utility of alizarin red s staining in calcium pyrophosphate dihydrate crystal deposition disease. J Rheumatol 2003; 30:1032.
  22. Adamson, TC, Resnik, CS, Guerra, J Jr, et al. Hand and wrist arthropathies of hemochromatosis and calcium pyrophosphate deposition disease: Distinct radiographic features. Radiology 1983; 147:377.
  23. Martel, W, Champion, CK, Thompson, GR, Carter, TL. A roentgenologically distinctive arthropathy in some patients with the pseudogout syndrome. Am J Roentgenol Radium Ther Nucl Med 1970; 109:587.
  24. Resnick, D, Niwiyama, G. Carpal instability in rheumatoid arthritis and calcium pyrophosphate deposition disease. Ann Rheum Dis 1977; 36:311.
  25. Lagier, R. Rare femoral erosions and osteoarthrosis of the knee associated with chondrocalcinosis. A histological study of this cortical remodelling. Virchows Arch A Pathol Anat Histol 1974; 364:215.
  26. Martel, W, McCarter, DK, Solsky, MA, et al. Further observations on the arthropathy of calcium pyrophosphate crystal deposition disease. Radiology 1982; 141:1.
  27. Frediani, B, Filippou, G, Falsetti, P, et al. Diagnosis of calcium pyrophosphate dihydrate crystal deposition disease: ultrasonographic criteria proposed. Ann Rheum Dis 2005; 64:638.
  28. Jones, AC, Chuck, AJ, Arie, EA, et al. Diseases associated with calcium pyrophosphate deposition disease. Semin Arthritis Rheum 1992; 22:188.
  29. Ishida, T, Dorfman, HD, Bullough, PG. Tophaceous pseudogout (tumoral calcium pyrophosphate dihydrate crystal deposition disease). Hum Pathol 1995; 26:587.
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