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Review
. 2016 Jun 30;374(26):2575-84.
doi: 10.1056/NEJMra1511117.

Calcium Pyrophosphate Deposition Disease

Ann K Rosenthal  1 , Lawrence M Ryan  1
Affiliations
Review

Calcium Pyrophosphate Deposition Disease

Ann K Rosenthal et al. N Engl J Med. .
No abstract available

PubMed Disclaimer

Conflict of interest statement

No potential conflict of interest relevant to this article was reported.

Figures

Figure 1.
Figure 1.. Calcium Pyrophosphate Deposition (CPPD).
Rhomboid, birefringent calcium pyrophosphate (CPP) crystals are seen under polarizing light microscopy in this sample of synovial fluid that was obtained from a patient with acute CPP crystal arthritis of the wrist (Panel A). The hands of an elderly patient with CPPD disease show swelling in the left wrist and the third proximal interphalangeal joint of the left hand (Panel B).
Figure 2.
Figure 2.. Imaging of Chondrocalcinosis in Patients with CPPD Disease.
Panel A shows a radiograph of a knee with meniscal chondrocalcinosis (arrow). Panel B shows a radiograph of a wrist with chondrocalcinosis of the triangular cartilage (arrow). Panel C shows a radiograph of a hand with hooklike osteophytes (arrows). Panel D shows an ultrasonographic image of a right knee, which was obtained with the transducer in the anatomical axial plane, with the knee flexed 90 degrees. The probe was pointed at the femoral cartilage on the “V” of the patellar groove. Chondrocalcinosis is seen in the substance of the cartilage; the arrow indicates the direction of the probe.
Figure 3.
Figure 3.. Pathophysiological Features of CPPD Disease.
The formation of CPP crystals occurs in the articular cartilage pericellular matrix and is facilitated by extracellular vesicles known as articular cartilage vesicles. Pyrophosphate (PPi) is generated from extracellular ATP and forms complexes with calcium to create CPP crystals. Panel A of the box, upper right, lists the factors that are known to modulate levels of extracellular ATP and PPi, and Panel B the extracellular matrix factors that regulate the formation of CPP crystals. P2X indicates one class of purinergic receptors. CPP crystals induce inflammation in the synovial space but also have adverse biomechanical consequences and direct catabolic effects on joint tissues owing to the production of prostaglandin E2 and matrix metalloproteinases. These factors ultimately produce cartilage degeneration, as shown by the CPP crystal deposit in cartilage in situ (inset). ANKH denotes human homologue of the protein product of the progressive ankylosis gene, ENPP1 ectonucleotide pyrophosphatase 1, ePi extracellular phosphate ion, ePPi extracellular PPi, IGF-1 insulin-like growth factor 1, iPPi intracellular PPi, and TGF-β transforming growth factor β.
Figure 4.
Figure 4.. Management Strategy for Acute CPP Crystal Arthritis.
A treatment is considered to be feasible if it is not associated with unacceptable side effects. NSAID denotes nonsteroidal antiinflammatory drug.
Figure 5.
Figure 5.. Management Strategy for Chronic CPP Crystal Arthritis.
Combination therapy may include various combinations of colchicine, prednisone, methotrexate, and hydroxychloroquine.
See this image and copyright information in PMC

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