Calcium Pyrophosphate Deposition Disease Scott Gabbard

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Calcium Pyrophosphate Deposition DiseaseScott
Gabbard
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Prevalence

• Pathologic surveys indicate about 4 of the adult
  population have articular CPPD deposits at the
  time of death
• Recent studies demonstrate radiographic evidence
  of chondrocalcinosis in up to 50 of those older
  than 85

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Pathogenesis

• CPPD crystal formation is initiated in cartilage
  located near the surface of chondrocytes
• Data suggests that the disorder is associated
  with excessive cartilage production of
  pyrophosphate which leads to calcium
  pyrophosphate supersaturation and CPPD crystal
  deposition

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Pathogenesis

• CPPD crystals are phagocytosed by neutrophils
• This results in release of lysosomal enzymes and
  chemotactic factors

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Etiology

• Most attacks are idiopathic
• Disease associations include
• Joint trauma
• Familial chondrocalcinosis
• Hemochromatosis
• Gitelmans syndrome
• Hypomagnesemia, hypophosphatemia

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Manifestations

• CPPD has the capacity to mimic any type of
  arthiritis
• Asymptomatic disease
• Most joints with radiographic evidence of CPPD
  deposition have mild to no symptoms of disease

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Manifestations

• Pseudogout
• Subacute attacks of arthritis involving one or
  several extremity joints
• These attacks closely resemble those of urate
  gout both in the typical accompanying signs of
  severe acute inflammation and in the occasional
  occurrence of cluster attacks

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Manifestations

• Pseudo-Rheumatoid Arthritis
• Multiple joints are involved with attacks of
  articular inflammation lasting up to several
  months
• Symptoms include significant morning stiffness,
  fatigue, synovial thickening, localized edema,
  and restricted joint motion

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Manifestations

• Pseudo-Osteoarthritis
• 50 percent of patients with symptomatic CPPD
  crystal deposition disease show progressive joint
  degeneration
• The joints most commonly affected include the
  knees, followed by the wrists, MCPs, hips,
  shoulders, elbows, and spine

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Differential diagnosis

• Gout
• Septic arthritis
• Rheumatoid arthritis
• Polymyalgia rheumatica
• Malignancy
• Seronegative spondyloarthropathies
• Hemochromatosis
• Lyme arthritis

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Diagnosis

• Synovial fluid
• Positively birefringent rhomboid crystals
• During acute pseudogout attacks, will find
  phagocytosed crystals within PMNs

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Diagnosis

• Intracellular phagocytosis of CPPD crystal

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Diagnosis
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Diagnosis

• Chondrocalcinosis
• Punctate and linear radiodensities in
  fibrocartilage and articular cartilage

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Diagnosis

• Chondrocalcinosis of knee joint

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Diagnostic criteria

• Definitive diagnosis of CPPD deposition requires
• Demonstration of CPPD crystals in synovial fluid
  by x-ray diffraction, or
• Positively birefringent crystals seen by
  compensated polarized light and typical cartilage
  calcification on x-ray

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Treatment

• Acute Pseudogout
• Removal of crystals by joint aspiration
• Administration of NSAIDs or colchicine
• Intraarticular injection of steroids such as
  triamcinolone
• Joint immobilization

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Treatment

• Colchicine
• Blocks release of chemotactic factors for
  neutrophils and mononuclear cells and inhibits
  neutrophil-endothelial cell binding

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Treatment

• Probenecid
• Inhibits transmembrane anion transport, thus
  decreasing extracellular pyrophosphate production
  

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References

• Becker, Michael A. Clinical manifestations and
  diagnosis of calcium pyrophosphate crystal
  deposition disease. UpToDate. 2007.
• Becker, Michael A. Pathogenesis and etiology of
  calcium pyrophosphate crystal deposition disease.
  UpToDate. 2007.
• Becker, Michael A. Treatment of calcium
  pyrophosphate crystal deposition disease.
  UpToDate. 2007.
• Klippel, John. Primer on the Rheumatic Diseases.
  The Arthritis Foundation, 2001
• http//www.clevelandclinic.org/health/health-info/
  Pictures/Pseudogout.gif, Cleveland Clinic
  Foundation
• http//www.wheelessonline.com/image9/cppd2.jpg
• Lahita, Robert. Educational Review Manual of
  Internal Medicine Volume 1, Rheumatologic
  Diseases, CCGMP

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Im outta here!