Calcium pyrophosphate dihydrate deposition disease

Calcium pyrophosphate dihydrate disease (CPPD disease), also referred as pyrophosphate arthropathy and perhaps confusingly as pseudogout, is common, especially in the elderly, and is characterised by the deposition of calcium pyrophosphate in soft tissues and cartilage.

CPPD is one of many causes of soft tissue calcification (chondrocalcinosis). It is not synonymous with chondrocalcinosis and not the only cause of soft tissue calcification.

Where crystal deposition causes acute clinical manifestation, the term pseudogout should be used. Pyrophosphate arthropathy is a term that describes arthropathy secondary to CPPD deposition. However, it is often used indiscriminately to refer to chondrocalcinosis too.

CPPD is commonest in patients over the age of 50. Men and women are equally affected. 

The crystals are weakly positively birefringent on polarised microscopy and have a rhomboid or rod shape. Causes of CPPD can be divided into:

CPPD has many features of osteoarthritis with unusual distribution, for example, they tend to be symmetric in distribution and involve non-weight bearing joints or, in the hands, mainly involve intercarpal and MCP joints. 

Features of degenerative joint disease in joints that are not commonly affected by it (i.e. non-weight bearing joints):

Chondrocalcinosis can occur in many locations. Notable sites include:

  • knee: medial meniscus and patellofemoral joint
  • wrist: triangular fibrocartilage complex and lunotriquetral ligaments 
  • spine: periodontoid tissue

Large subchondral cysts may be present.

It is controversial whether gout leads to calcification of articular fibrocartilage or hyaline cartilage 6. CPPD disease can be differentiated from gout on ultrasound given that echogenic monosodium urate crystals line the surface of articular cartilage, whereas echogenic CPPD calcifications are located within the cartilage itself 7.

Possible imaging differential considerations include


Arthritides
Share Article

Article Information

rID: 1037
Section: Pathology
Synonyms or Alternate Spellings:
  • Pseudogout
  • Calcium pyrophosphate dihydrate deposition arthropathy
  • CPPD
  • Calcium pyrophosphate deposition disease (CPPD)
  • CPPD arthropathy
  • Calcium pyrophosphate dihydrate deposition disease (CPPD)

Support Radiopaedia and see fewer ads

  • Drag
    Case 1: T2 : CPPD of dens
    Drag here to reorder.
  • Drag
     Case 2: knee
    Drag here to reorder.
  • Drag
    Case 3: shoulder
    Drag here to reorder.
  • Drag
    Case 4: knee
    Drag here to reorder.
  • Drag
    CT cervical spine...
    Case 5
    Drag here to reorder.
  • Drag
    Case 6
    Drag here to reorder.
  • Drag
    Case 7
    Drag here to reorder.
  • Updating… Please wait.
    Loadinganimation

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.