Calcium Pyrophosphate Deposition (CPPD) Disease Clinical Presentation

Updated: Apr 03, 2021
  • Author: Constantine K Saadeh, MD; Chief Editor: Herbert S Diamond, MD  more...
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Presentation

History

Presentations of calcium pyrophosphate deposition (CPPD) disease include the following [2] :

  • Asymptomatic CPPD, as an incidental radiographic finding
  • Acute pseudogout
  • Pseudo-osteoarthritis
  • Pseudo–rheumatoid arthritis
  • Pseudo–neuropathic joints

Asymptomatic (lanthanic) CPPD

This is usually associated with radiographic findings of chondrocalcinosis in the absence of clinical manifestations and may be the most common form of CPPD.

The classic radiologic findings include chondrocalcinosis of the hyaline cartilage and fibrocartilage of the knees, the fibrocartilage of the triangular ligament of the wrist, the fibrocartilage of the symphysis pubis, and the acetabulum labrum of the hips.

Acute pseudogout

Acute pseudogout is characterized by acute monoarticular or oligoarticular arthritis. Pseudogout usually involves the knee or the wrist, although almost any joint can be involved, including the first metatarsophalangeal (MTP) joint, as occurs in patients with gout. This form of CPPD accounts for 25% of cases. Glucose levels are usually normal.

Clinical manifestations are similar to those of acute gouty arthritis—typically an acute monoarthritis with pain and swelling—although generally not as intense. Polyarticular attacks may occur on occasion. Pseudogout may be precipitated by medical illness such as myocardial infarction, congestive heart failure, or stroke or may occur after surgery. Trauma may also be a precipitating factor. Events that affect serum calcium levels also may precipitate attacks of pseudogout.

Occasionally, pseudogout may present as a pseudoseptic syndrome with acute arthritis, fever, and leukocytosis with a left shift.

Pseudo-osteoarthritis

Pseudo-osteoarthritis often involves the metacarpophalangeal (MCP) joints, wrists, elbows, and shoulders—joints unlikely to be involved in primary osteoarthritis. Most commonly, however, it affects the knees, and it can involve the proximal interphalangeal (PIP) joints and spine, as occurs in patients with primary osteoarthritis. Pseudo-osteoarthritis accounts for 50% of all CPPD cases. Approximately half of these patients also have associated pseudogout.

Pseudorheumatoid arthritis

Pseudorheumatoid arthritis in patients with CPPD involves symmetrical inflammation of the PIP and MCP joints. Clinically, these patients complain of morning stiffness and joint swelling. In a study from Finland, the prevalence of CPPD in 435 patients with early seronegative rheumatoid arthritis was 3.9% overall, but the percentage was 7.0% in patients age 60 years or older at baseline. [13]

Pseudoneuropathic joints

Neuropathic-like arthropathy, which is observed in fewer than 5% of patients with CPPD, most commonly involves the knee. This is a severe, destructive arthropathy. Unlike true neuropathic arthropathy, no clear underlying neurologic disorder is present. The presence of chondrocalcinosis can aid in making the diagnosis.

CPPD can present as spondylodiscitis. This was shown in 2 case reports, one in a 75-year-old woman who presented with neck pain and in whom magnetic resonance imaging showed spondylodiscitis based on contrast enhancement of C5-C6 vertebrae corpus, and the other in a 61-year-old woman with spondylodiscitis at the C5-C6 intervertebral disc. [14]

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Physical Examination

The physical examination findings vary depending on the form of CPPD in a given patient, who may present with an acute arthritis or different patterns of chronic arthritis.

Acute pseudogout

Physical examination findings show an acutely inflamed joint with swelling, effusion, warmth, tenderness, and pain on range of motion similar to acute gouty arthritis. This typically occurs in the knee but may be present in the wrists, shoulders, ankles, hands, and feet.

Pseudo-osteoarthritis

Physical examination findings show a picture similar to osteoarthritis, sometimes with an unusual joint predilection. If a patient has osteoarthritis involving the MCP joints and wrists, consider CPPD associated with an underlying metabolic disease.

Pseudorheumatoid arthritis

Physical examination findings show a picture similar to rheumatoid arthritis, with synovitis in a symmetrical, polyarticular pattern, especially involving the wrists and MCP joints.

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Complications

Common complications from CPPD include acute synovitis and chronic degenerative arthritis, which is expected from the various phenotypes (ie, pseudogout, pseudo-osteoarthritis, pseudorheumatoid arthritis). Joint destruction from a neuropathic-like arthropathy is very rare. In addition, case reports of invasive (tumoral or tophaceous) CPPD have been reported. Kudoh et al reported a case of tophaceous pseudogout of the temporomandibular joint extending to the base of the skull in a 38-year-old man. [15] Tumoral pseudogout of a PIP (proximal interphalangeal joint) with an enlarging calcified mass and secondary bony erosion was reported by Park et al. [16]  

Coexistent infection with CPPD is a possible complication but the occurrence rate for this is not established. However, evaluation for coexistent infection should be performed when clinically indicated. Tuberculosis of the wrist accompanied by calcium pyrophosphate deposition was reported by Watanabe et al. [17]  

Crowned dens syndrome (calcific deposits in the cruciform and alar ligaments surrounding the odontoid process of the second cervical vertebra [the dens], which appear as a 'crown' surrounding the top of the dens on imaging studies) may complicate CPPD. Patients with crowned dens syndrome typically present with localized pain at the base of skull/back of the neck, neck stiffness, and systemic evidence of inflammation (eg, fever and elevated levels of inflammatory markers). [18] Haikal et al reported that cervical computed tomography (CT) scans revealed crowned dens syndrome in 34 of 57 patients with CPPD,  and recommended considering cervical CT in elderly patients with neck pain in the setting of CPPD. [19]

Odontoid fracture with nonunion rates are significantly higher in CPPD patients.  This illustrates the importance of making the appropriate diagnosis and deciding on treatment. [20]

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