Pathogenesis and etiology of calcium pyrophosphate crystal deposition (CPPD) disease
- Michael A Becker, MD
Michael A Becker, MD
- Section Editor — Crystal Diseases
- Professor Emeritus of Medicine
- University of Chicago Pritzker School of Medicine
Precipitation of crystals of calcium pyrophosphate dihydrate (CPP) in connective tissues may be asymptomatic or may be associated with several clinical syndromes associated with acute and chronic arthritis. These disorders comprise the spectrum of calcium pyrophosphate crystal deposition (CPPD) disease .
The pathogenesis and etiology of CPPD disease will be reviewed here. The clinical manifestations, diagnosis, and treatment of this disorder are discussed separately. (See "Clinical manifestations and diagnosis of calcium pyrophosphate crystal deposition (CPPD) disease" and "Treatment of calcium pyrophosphate crystal deposition (CPPD) disease".)
Calcium pyrophosphate dihydrate (CPP) was formerly abbreviated and commonly referred to as “CPPD” because the dihydrate is necessary for crystallization; but the abbreviation “CPPD” is now reserved for “CPP deposition.” Alternative names representing specific clinical or radiographic features of CPPD disease, including pseudogout, chondrocalcinosis, and pyrophosphate arthropathy, have retained some popularity, but each has limitations:
●Pseudogout/acute CPP crystal arthritis – Pseudogout is a term that has historically been used in two contexts: first, to differentiate the overall concept of CPPD disease from that of monosodium urate crystal deposition disease (gout or urate gout); and second, to describe acute attacks of CPP crystal-induced arthritis clinically resembling those commonly encountered in urate gout. The term “acute CPP crystal arthritis” is now preferred in place of “pseudogout” to describe the latter instance .
●Chondrocalcinosis – Chondrocalcinosis refers to radiographic calcification in hyaline and/or fibrocartilage. It is commonly present in patients with CPPD, but is neither absolutely specific for CPPD nor universal among affected patients.
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