Calcium Pyrophosphate Deposition Disease Clinical Presentation

Updated: Jan 23, 2018
  • Author: Constantine K Saadeh, MD; Chief Editor: Herbert S Diamond, MD  more...
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Presentation

History

Presentations of calcium pyrophosphate deposition disease (CPDD) include the following:

  • Asymptomatic CPDD, as an incidental radiographic finding
  • Acute pseudogout
  • Pseudo-osteoarthritis
  • Pseudorheumatoid arthritis
  • Pseudoneuropathic joints

Asymptomatic (lanthanic) CPDD

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

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 CPDD 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. It affects the knees most commonly and can involve the proximal interphalangeal (PIP) joints and spine, as occurs in patients with primary osteoarthritis. This form of CPDD accounts for 50% of all cases. Approximately half of these patients also have associated pseudogout.

Pseudorheumatoid arthritis

This pattern is found in approximately 5% of patients with CPDD and is associated with symmetrical inflammation of the PIP and MCP joints. Clinically, these patients complain of morning stiffness and joint swelling.

Pseudoneuropathic joints

Neuropathic-like arthropathy, which is observed in fewer than 5% of patients with CPDD, 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.

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Physical Examination

The physical examination findings vary depending on the form of CPDD 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 CPDD 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 CPDD 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) CPDD 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. [12] Tumoral pseudogout of a PIP (proximal interphalangeal joint) with an enlarging calcified mass and secondary bony erosion was reported by Park et al. [13]  

Coexistent infection with CPDD is a complication but the occurence 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. [14]  

Crowned dens syndrome (calcific deposits around the dens in the transverse ligament of the atlas and alar ligaments) is a rare complication of CPDD. Tedeschi et al reported a case of crowned dens syndrome with myelodysplastic syndrome (MDS) in a 75-year-old man; the authors hypothesize that this was paraneoplastic pseudogout secondary to MDS. [15]

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