Calcium Pyrophosphate Deposition (CPPD) Disease

Background

• CPPD disease is due to calcium pyrophosphate dihydrate crystal deposition in articular tissues and has clinical manifestations ranging from asymptomatic to acute inflammatory arthritis ("pseudogout").
• CPPD typically affects patients over age 50 years.
• CPPD may be idiopathic, genetic, or secondary to metabolic disorders, such as those that cause hypomagnesemia, hemochromatosis, or hyperparathyroidism.

Evaluation

• Consider CPPD disease in patients ≥ 50 years old presenting with:
  • acute inflammatory arthritis (acute calcium pyrophosphate crystal arthritis) in 1 or more joints, especially the knee or wrist, with symptoms reaching a maximum intensity within 6-24 hours
  • osteoarthritis (OA) with chondrocalcinosis (pseudo-OA type) in the affected joint (CPPD is often characterized by subchondral cysts, large osteophytes, and/or presence of OA in the glenohumeral joints, wrists, and metacarpophalangeal joints [especially second and third metacarpophalangeal joints])
  • chronic inflammatory arthritis (pseudo-rheumatoid arthritis type), which is a less common manifestation of CPPD
• Patients can experience multiple manifestations of CPPD over a lifetime or simultaneously.
• Appearance of chondrocalcinosis on x-ray or ultrasound supports diagnosis, but absence does not exclude diagnosis.
• Definitive diagnosis requires identification of CPPD crystals in synovial fluid or biopsied tissue.

Management

• No treatment is necessary for asymptomatic patients.
• Treatment of acute attack should be individualized based on patient characteristics, risk factors, and comorbidities.
  • Nonpharmacologic treatment options may include rest and ice packs.
  • Joint aspiration and intra-articular injection of a long-acting steroid may be sufficient.
  • Systemic medication options for acute attacks include:
    • oral nonsteroidal anti-inflammatory drugs (NSAIDs)
    • colchicine 0.5 or 0.6 mg up to 3-4 times daily
    • short course of oral or parenteral corticosteroids
    • corticotropin (ACTH) if there are comorbidities or a lack of response to NSAIDs or colchicine
    • anakinra (interleukin-1 antagonist) 100 mg/day subcutaneously for 3 days
• For chronic calcium pyrophosphate crystal inflammatory arthritis, options include:
  • NSAID orally with gastroprotective treatment if needed
  • colchicine 0.5 or 0.6 mg up to 3-4 times daily
  • low-dose corticosteroid, methotrexate, or hydroxychloroquine for patients who are unable to tolerate or are refractory to NSAID or colchicine
• For prevention of recurrences in patients with frequent episodes of acute calcium pyrophosphate crystal inflammatory arthritis (pseudogout):
  • NSAID orally with gastroprotective treatment if needed
  • colchicine 0.5 or 0.6 mg up to 3-4 times daily
• No treatment is known to modify or dissolve calcium pyrophosphate crystals.