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Calcium pyrophosphate deposition disease (CPPD) is a medical condition in which crystals of Calcium pyrophosphate dihydrate accumulate in the hyaline articular cartilage or fibrocartilage, which can result in painful inflammation of the joint. It is a form of crystal arthritis, and — as its name indicates — often misdiagnosed as gout (a similar condition in which crystals of monosodium urate form in joints). CPPD is more common in women than in men while gout occurs much more often in men than in women.

Definition

Calcium pyrophosphate deposition disease (CPPD) is sometimes incorrectly referred to as pseudogout or chondrocalcinosis, though these entities are not synonymous with CPPD. Pseudogout refers to the clinical syndrome associated with CPPD, which is characterized by red, tender, and swollen joints and mimics Diagnostic_Radiology/Musculoskeletal_Imaging/Joint_Disorders/Gout. Chondrocalcinosis, on the other hand, is the radiologic or pathologic presence of calcifications in articular or fibrocartilage. This is often caused by calcium pyrophosphate dihydrate crystals, but may also be caused by other forms of calcification. The calcification is initially only visible microscopically, but can eventually accumulate in amounts sufficient to be seen radiographically as well. Shedding of crystals into the joint produces an acute inflammatory synovitis resembling a classic gouty attack, which latter is caused by urate crystals.

Clinical Aspects

CPPD crystal deposition disease is a polyarticular arthritis, although it can initially present as monoarticular.

CPPD crystals tend to form within articular tissues. Diagnosis is by microscopy of fluid from a joint aspiration. Rhomboidal crystals should be seen under the microscope which exhibit weakly positive birefringence under polarized light. X-rays of the joint may show signs of chondrocalcinosis. Asymptomatic deposits can form in cartilage, joint capsules, intervertebral discs, tendons, and ligaments. Crystal deposition within cartilage (both hyaline and fibrocartilage) is known as chondrocalcinosis. Chondrocalcinosis initially is only visible microscopically, but if there is enough calcification, we can seen it radiographically as well. Common locations of chondrocalcinosis include the knees, wrists, elbows, and hips.

Crystal-induced synovitis is the result of shedding of crystals in the joint space after rupture of a CPPD deposit. Clinically this presents as an acute inflammatory synovitis very similar to that of an attack of gout, and has been referred to as "pseudogout."

Radiologic Findings

Three main manifestations of CPPD deposition:

  1. chondrocalcinosis
  2. crystal induced synovitis (pseudogout syndrome)
  3. pyrophosphate arthropathy

Treatment

Treatment of CPPD is mostly aimed at preventing further crystal formation and reducing symptoms from crystal deposition. CPPD crystal deposition cannot be reversed. If an underlying disease (such as hemochromatosis or Diagnostic_Radiology/Musculoskeletal_Imaging/Metabolic_Basic/Primary hyperparathyroidism) is present, treatment should be aimed specifically at controlling the disease. For symptomatic joints, treatment is similar to treatment of gout. When a single joint is involved, joint aspiration and intra-articular corticosteroid injection is often used, in addition to NSAIDs and/or colchicine. When multiple joints are involved, joint injection is often impractical, or limited only to the most severely involved joint, and oral systemic treatment is chosen instead.

References

External links

Arthritis

Pyrofosfatsynovit

 

This article is licensed under the GNU Free Documentation License. It uses material from the "Calcium pyrophosphate deposition disease".

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