Calcium Pyrophosphate Crystal Deposition Disease (CPPD)

(Pseudogout)

(Chondrocalcinosis)

 

Epidemiology:

    • >50y, more common with increasing age

 

Common sites:

    • menisci / knees
    • wrists
    • elbows
    • shoulders
    • ankles
    • intervertebral discs

 

Gross features:

    • Deposits first develop in articular matrix, menisci, and intervetrebral discs
    • Chalky white friable deposits
    • Mass-like aggregates like tophi rarely

 

Histologic features:

    • Neutrophil-rich inflammatory infiltrate
    • Deposits are oval, blue-purple aggregates
      • Foreign-body giant cell reaction surrounding them
    • Crystals are weakly birefringent and form geometric shapes
      • Best seen on deparafinized, unstained slides

 

Immunophenotype:

Marker:

Sensitivity:

Specificity:

 

 

 

 

Molecular features:

    • ANKH gene mutation in AD familial form
      • Transmembrane inorganic pyrophosphate transport channel

 

Other features:

    • Secondary:
      • Previous joint damage
      • Hyperparathyroidism
      • Hemochromatosis
      • Hypomagnesemia
      • Hypothyroidism
      • Ochronosis
      • diabetes

 

References:

    • Robbins 2005