Version May 2024
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CPPD DISEASE DEFINITION — Pyrophosphate is a normal chemical that everyone has in the body. It is important for the healthy function of connective tissues such as bones, cartilage, and joints. However, in some cases, excess pyrophosphate complexes with calcium to form microscopic calcium pyrophosphate (CPP) crystals, which preferentially deposit in joint tissues.
Crystals sometimes do not cause symptoms; this is called "asymptomatic" calcium pyrophosphate crystal deposition (CPPD) disease. At other times, the crystals can cause acute or more chronic (long-term) inflammation or degeneration of involved tissues. Each of these outcomes is a part of the spectrum of CPPD disease.
TYPES OF CPPD DISEASE — Calcium pyrophosphate crystal deposition (CPPD) disease can present in a number of different ways. The range of presentations includes:
●Asymptomatic CPPD disease (in which the person has no symptoms, but crystals can be seen on imaging tests, such as X-rays)
●Acute calcium pyrophosphate (CPP) crystal arthritis (formerly called "pseudogout")
●Chronic CPP crystal inflammatory arthritis
●Osteoarthritis with CPPD
●Severe joint degeneration
●Spinal involvement
Acute CPP crystal arthritis (pseudogout) — Acute CPP crystal arthritis was the first type of CPPD disease to be widely recognized. It refers to sudden-onset attacks of joint pain, swelling, warmth, and difficulty using the affected joint. An attack can last for days or even weeks. The knee is affected in more than half of people with acute CPP crystal arthritis; however, the disorder can also affect the ankles, feet, shoulders, elbows, wrists, or hands.
This condition has also been called "pseudogout" because the symptoms of acute CPP crystal arthritis are very similar to those of gout, an arthritis caused by urate (uric acid) crystals rather than CPP crystals (see "Patient education: Gout (Beyond the Basics)"). Although the two disorders can cause similar symptoms, patients with acute CPP crystal arthritis are treated somewhat differently than patients with gout. (See 'CPPD disease treatment' below.)
Chronic CPP crystal inflammatory arthritis — Some people with CPPD disease have more than a single joint involved at once. They have stiffness, pain, and swelling in multiple joints, which can last weeks to months. The pattern of involved joints is similar to those involved in acute CPP crystal arthritis. (See 'Acute CPP crystal arthritis (pseudogout)' above.)
Osteoarthritis with CPPD — CPPD can also occur in a joint with osteoarthritis in which the cartilage has worn down. This is particularly common in the hip and knee joints. (See "Patient education: Osteoarthritis symptoms and diagnosis (Beyond the Basics)".)
Severe joint degeneration and spinal involvement — CPPD disease can lead over time to serious problems such as degeneration (breakdown) of the cartilage. When crystals form in and around the spine, it can also lead to back pain and stiffness, and sometimes compression of the spinal cord or surrounding nerves, but this type of involvement is unusual.
CPPD DISEASE RISK FACTORS — Calcium pyrophosphate crystal deposition (CPPD) disease symptoms develop in some (but not all) people in response to the deposition of calcium pyrophosphate (CPP) crystals in the joints. The crystals first develop in the joint cartilage and eventually move to the lining of the joint (also called the synovium) or into the joint fluid, where they can cause inflammation with associated pain, swelling, and disability in the affected joint. In most people with CPPD disease, it is not known exactly why CPP crystals form and deposit in the joints. (See "Pathogenesis and etiology of calcium pyrophosphate crystal deposition (CPPD) disease".)
Some people, particularly older adults, have CPP crystals in their joints (called "chondrocalcinosis") but never experience symptoms of acute arthritis. Chondrocalcinosis is present in up to 50 percent of people by age 90, often without or with only modest symptoms.
In addition to older age, there are several other factors that increase the risk of accumulating CPP crystals in the joints, including:
●Joint trauma – People who have previously experienced a significant injury to or surgery on a joint have an increased risk of developing CPPD.
●Genetics – People can inherit a predisposition to CPPD (called "familial chondrocalcinosis"); these people are more likely to develop acute CPP crystal arthritis or other features of CPPD disease earlier in life than patients with sporadic CPPD disease.
●Excess iron – People with a genetic disorder called hemochromatosis, which causes the body to store excess iron, are at an increased risk of developing CPPD. (See "Patient education: Hereditary hemochromatosis (Beyond the Basics)".)
●Other related disorders – Several other diseases of metabolism or endocrine glands are associated with CPPD disease. These include hyperparathyroidism (overactive parathyroid glands), hypophosphatasia (an inherited metabolic bone disorder), hypomagnesemia (low levels of magnesium in the blood), Gitelman syndrome (an inherited kidney disorder), and possibly others.
CPPD DISEASE DIAGNOSIS — A health care clinician can confirm or rule out a diagnosis of calcium pyrophosphate crystal deposition (CPPD) disease by performing an examination and tests. In many patients, a sample of joint fluid is obtained in order to determine whether calcium pyrophosphate (CPP) dihydrate crystals are present in the fluid on microscopic examination and to exclude arthritis due to other causes, such as gout or joint infection.
Synovial fluid analysis — Synovial (joint) fluid is obtained under sterile conditions through a needle inserted into the affected joint. The fluid is then analyzed to determine if crystals or infection are present. The presence of CPP crystals in a patient with joint pain and inflammation strongly supports a diagnosis of acute CPP crystal arthritis.
Imaging — A clinician may examine the painful joint(s) or other frequently involved joints by taking X-rays or doing other imaging tests. X-ray images can reveal calcium-containing crystal deposits in the cartilage, a condition known as chondrocalcinosis. Ultrasonography (ultrasound examination) of affected joints is another imaging method that is increasingly being used to establish or confirm the diagnosis of CPPD disease or to guide synovial fluid aspiration (a "joint tap") to obtain material for synovial fluid analysis. Sometimes a special kind of computed tomography (CT) scan called dual-energy CT can also be used to detect the presence of CPPD.
CPPD DISEASE TREATMENT — There is no treatment that can completely remove or prevent the formation of calcium pyrophosphate (CPP) dihydrate crystals. However, the joint pain and swelling generally resolve with treatment. Treatments vary depending on the type of calcium pyrophosphate crystal deposition (CPPD) disease but may including the following:
●Joint immobilization – Patients with a flare of acute CPP crystal arthritis may be advised to avoid weightbearing (walking or running if the legs or feet are involved), avoid excessive movement, and limit activity for a period of time to minimize pain and swelling. In some cases, a temporary splint will be recommended to limit joint movement.
●Joint aspiration and/or injection – Patients with acute CPP crystal arthritis affecting one or two joints may need a joint aspiration and/or injection. A clinician may insert a needle into the affected joint to remove the fluid and crystals that have accumulated. This can help to relieve pressure and pain. An injection of glucocorticoids (steroids) into the joint may relieve the associated joint inflammation. (See "Patient education: Steroid injection (The Basics)".)
●Oral antiinflammatory medications – Oral antiinflammatory medications, such as a nonsteroidal antiinflammatory drug (NSAID), an oral glucocorticoid, or colchicine, may be preferred over joint injections for treating attacks of acute CPP crystal arthritis that affect more than two joints. They are usually continued until the attack resolves. In addition, oral antiinflammatory medications are sometimes used to try to prevent attacks of acute CPP crystal arthritis and for treating chronic CPP crystal inflammatory arthritis.
•(See "Patient education: Nonsteroidal antiinflammatory drugs (NSAIDs) (Beyond the Basics)".)
•(See "Patient education: Oral steroid medicines (The Basics)".)
●Immunosuppressive medications – Sometimes patients with acute or chronic CPP crystal arthritis may need medications that help calm down the immune system and reduce inflammation. As an example, providers may prescribe medications like anakinra or canakinumab for a patient with a bad flare of acute CPP crystal arthritis if the patient cannot take or does not respond to other options.
●Treatment of related conditions – If CPPD is caused by a separate disorder (see 'CPPD disease risk factors' above), that condition may require treatment, although this may not affect the course of CPP crystal-related joint disease.
The cost of colchicine has been an issue for many patients and their clinicians. Programs are available to assist with the cost of colchicine for eligible patients; visit the NeedyMeds website for more information. (See "Patient education: Gout (Beyond the Basics)" and "Patient education: Coping with high prescription drug prices in the United States (Beyond the Basics)".)
WHERE TO GET MORE INFORMATION — Your healthcare provider is the best source of information for questions and concerns related to your medical problem.
This article will be updated as needed on our web site (www.uptodate.com/patients). Related topics for patients, as well as selected articles written for healthcare professionals, are also available. Some of the most relevant are listed below.
Patient level information — UpToDate offers two types of patient education materials.
The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.
Patient education: Gout (The Basics)
Patient education: Calcium pyrophosphate deposition disease (The Basics)
Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.
Patient education: Gout (Beyond the Basics)
Patient education: Hereditary hemochromatosis (Beyond the Basics)
Patient education: Osteoarthritis treatment (Beyond the Basics)
Patient education: Nonsteroidal antiinflammatory drugs (NSAIDs) (Beyond the Basics)
Professional level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.
Clinical manifestations and diagnosis of calcium pyrophosphate crystal deposition (CPPD) disease
Pathogenesis and etiology of calcium pyrophosphate crystal deposition (CPPD) disease
Treatment of calcium pyrophosphate crystal deposition (CPPD) disease
The following organizations also provide reliable health information.
●National Library of Medicine
(www.nlm.nih.gov/medlineplus/healthtopics.html)
●National Institute of Arthritis and Musculoskeletal and Skin Diseases
(301) 496-8188
●American College of Rheumatology/Association of Rheumatology Health Professionals
(404) 633-3777
●The Arthritis Foundation
(800) 283-7800
ACKNOWLEDGMENT — The editorial staff at UpToDate would like to acknowledge Michael A Becker, MD, who contributed to an earlier version of this topic review.
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