Calcium pyrophosphate deposition disorder (CPPD) is a chronic (long-term) type of arthritis that causes joint problems due to an accumulation of calcium pyrophosphate crystals in and around the joints and tendons. It most commonly affects the knees, wrists, and hips.
CPPD can cause calcification of the tendons and ligaments. This typically affects the tendons around the shoulder joints (rotator cuff) and the achilles tendon (the heels) but any joint can be affected. Calcification can be severe and debilitating.
The disease is sometimes similar to gout because both diseases are caused by a build-up of crystals in the joints. In gout, the crystals are made of uric acid instead of calcium pyrophosphate. In both diseases, the immune system can the attack joints when crystal deposits suddenly appear. This causes severe pain, redness, and swelling. When this happens in CPPD, it may be referred to as pseudogout because is is similar to an attack of gout.
It is not known why calcium pyrophosphate crystals form in people who get CPPD. It is not believed that calcium supplements cause the disease or make it worse.
It can be difficult to diagnose CPPD because its symptoms are very similar to other arthritic diseases including gout, osteoarthritis, rheumatoid arthritis, and tendonitis.
CPPD typically affects men and women over the age of 50. It is often found in and around joints typically affected by osteoarthritis, especially the knees.
Calcium Pyrophosphate Deposition Disorder (CPPD) has a number of symptoms that are similar to many other arthritic diseases. The symptoms of CPPD depend on the arthritic disease it mimics.
The most common form of CPPD is calcification of tendons and other structures around joints. This can be severe and debilitating.
CPPD can cause calcification of the tendons, ligaments, and other structures around joints, causing them to be painful, red, and swollen in a similar way to tendonitis. CPPD typically affects the tendons around the shoulder joints (rotator cuff) and the Achilles tendon (the heels). The wrists, knees, pelvis, and other parts of the feet are also commonly affected.
Gout-like and Infected Joint-like Symptoms (Pseudogout)
CPPD can cause joints to become hot, intensely painful, red, and swollen in an “attack” that is similar to a disease called gout. This can be confused with an infection in the joint. In CPPD crystals of calcium pyrophosphate form inside a joint, an event that triggers the immune system to attack. Gout is also caused by a buildup of crystals inside a joint, but they are made of uric acid instead, so its causes and treatments are different. This type of CPPD is also called pseudogout.
Osteoarthritis (OA)-like Symptoms
CPPD can look like OA when it causes pain and swelling in joints that are commonly affected by OA, such as the small joints of the hands or the knees. CPPD can also impact joints that are not typically affected by OA such as the wrists, pelvis, elbows and shoulders. If a joint looks like it is affected by osteoarthritis but it isn’t in a typical location for OA, then it might be CPPD.
Rheumatoid Arthritis (RA)-like Symptoms
CPPD can affect the knuckles of the hand (metacarpophalangeal joints) causing the hands to become swollen, painful, and stiff in a very similar way to RA. An X-ray of the hand can help tell the difference because in CPPD it will not show changes that would typically be caused by RA and will look more like osteoarthritis. People with this type of CPPD rarely have high levels of iron in their blood (hemochromatosis).
Other Diseases Associated with CPPD
Other diseases are associated with CPPD and can appear alongside it in some people. This can introduce additional symptoms. These diseases include hypothyroidism (underactive thyroid gland), hyperparathyroidism (overactive parathyroid gland), hypomagnesemia (low magnesium) or hemochromatosis (too much iron in the body).
Calcium Pyrophosphate Deposition Disorder (CPPD) is best diagnosed by a rheumatologist, a type of doctor that specializes in arthritis and autoimmune disease.
To diagnose CPPD, they will take a careful and complete history and perform a thorough physical examination. Based on this information, the doctor will likely order tests like blood tests, scans, and other types of tests to confirm their diagnosis.
Common Tests to Diagnose CPPD
X-rays: X-rays of the affected joints can be very useful to see typical features of CPPD. The CPPD crystals can be seen on X-ray and the common spots they are found is in the wrists, knees, shoulders, and pelvis.
Ultrasound: An ultrasound can be used to show calcium buildup in the tendons. This is most often done for the shoulder joint.
Looking for crystals of calcium pyrophosphate: A sample of fluid from an affected joint can be examined to look for calcium pyrophosphate crystals. This is best done when the joint is acutely inflamed (i.e. an attack of pseudogout).
Blood tests to rule out other diseases: Blood tests are done to rule out other rheumatic diseases (i.e. rheumatoid arthritis).
Looking for other conditions associated with CPPD: Tests for levels of thyroid hormone (TSH) and parathyroid hormone (PTH) check for thyroid conditions, an iron (ferritin) test looks for hemochromatosis (too much iron), and a magnesium test looks for low-levels (hypomagnesemia).
Calcium Pyrophosphate Deposition Disorder (CPPD) is caused by a build-up of calcium pyrophosphate crystals in the joints and tendons throughout the body. It typically affects men and women over the age of 50.
Scientists do not yet know why these crystals form. They are pretty confident that taking calcium supplements does not cause CPPD or make it worse.
Calcium Pyrophosphate Deposition Disorder (CPPD) treatments can vary depending on what combination of symptoms a given patient is experiencing. A variety of medications are available that can help manage the disease and enable people with CPPD to lead active and productive lives
Cortisone (Steroid) Injections
Cortisone injections can be very effective in people with CPPD that experience pseudogout symptoms, RA-like CPPD, CPPD associated with osteoarthritis (OA), and CPPD that causes inflammation in tendons. This type of treatment can reduce the pain and swelling caused by CPPD.
It can take up to 24 or 48 hours to feel the effects of a cortisone injection. Once the effects set in, they can last for a few days to many months, depending on the individual person and the nature of their disease.
Usually, cortisone injections are limited to 2 or 3 in a single joint per year. This can also be effective treatment when symptoms are more like OA (soreness and swelling in wrists and knees).
Regular use of low-dose oral prednisone can be effective in managing attacks of pseudogout in some people.
Attacks of pseudogout can sometimes be prevented with a medicine called Colcrys (colchicine). This medicine is often taken by people who have gout. Once a person starts taking colchicine, they must remember to continue to take it on a regular basis. Stopping this medicine can cause a new attack of pseudogout.
NSAID Medications for Inflammation and Pain
Non-Steroidal Anti-Inflammatory Drugs (NSAIDs) can reduce the inflammation caused by CPPD and also help to reduce symptoms such as pain. NSAIDs can be used for people with pseudogout, tendonitis, RA-like CPPD, or CPPD that is associated with osteoarthritis.
NSAIDs are an important treatment option in cases where joints can’t be easily injected with cortisone. Some people may need to use NSAIDs on a more regular basis, depending on their level of symptoms and pain.
Medications for Pain
Non-prescription analgesic medications such as acetaminophen (Tylenol) can be used to control pain. It has been shown to be safe when used even for long periods of time. For more severe pain, stronger medicines called opioids may be used.
Living with CPPD
The pain and stiffness caused by CPPD can sometimes limit people’s normal activities, including work. There are many things people can do to lessen the impact of CPPD on their work and daily routine.
We recommend adjusting features of the workplace to help make working with CPPD easier. For example, people that sit for much of the day can adjust the position of chairs and desks for proper posture. Vehicle seats can be adjusted to make driving more comfortable and reduce stress on joints and affected tissues.
Those who find out they have CPPD while they’re still young can consider choosing work that isn’t physically demanding on their joints and back.
Exercise is important to overall health. It also helps keep the joints moving properly, and helps protect them by strengthening the muscles around them.
The level and amount of exercise people with CPPD can do depends on the activity of their disease.
A trained arthritis physiotherapist is the best person to help design an exercise program tailored to the needs of patients with CPPD.
The following are some useful articles on exercising with arthritis:
Exercise and Arthritis: An article by arthritis physiotherapist Marlene Thompson
Exercising in a Flare: Another excellent article by Marlene Thompson on how to cope with flares through your exercise routine.
Alcoholic beverages are not an effective treatment for CPPD, and they can interact with many medications.
People with CPPD that plan on drinking should ask their doctor about possible negative interactions with the medications they take.
Some examples of medication that mixes poorly with alcohol includes, and is not limited to: the DMARDs methotrexate, Arava (leflunomide), and Imuran (azathioprine).
Smoking is not healthy for people with CPPD. It can make symptoms worse and harder to treat.
Research has shown that some arthritis medications do not work as well in people who smoke.
We recommend that smokers quit immediately to reduce the impact of their symptoms, improve the effectiveness of their medications, and improve their overall health.
Smoking significantly increases the risk of cardiovascular disease. The chronic (long-term) inflammation caused by rheumatic diseases like CPPD already increases this risk without a big extra boost from smoking.
Travel is still possible when you have CPPD.
It is best for people with this disease to get organized well in advance of a trip to ensure a smooth, comfortable, and enjoyable time.
Check out our Travel Checklist page.
Though CPPD does not particularly cause a loss of sex drive, it can cause pain, fatigue and emotional hardships. These hardships risk creating barriers to sexual needs, ability, and satisfaction.
People with CPPD can take comfort knowing that sex and intimacy can be maintained. In many cases, it can help draw partners closer together, especially through improved communication.
For more information on intimacy and arthritis, a great book is: Rheumatoid Arthritis: Plan to Win by Cheryl Koehn, Taysha Palmer and John Esdaile.
Calcium Pyrophosphate Deposition Disorder (CPPD) has no effect on fertility. The chances of getting pregnant for people with CPPD are the same as with people that don’t have it. CPPD also has no known effects on the developing fetus or the newborn.
The two main issues doctors worry about with CPPD and pregnancy are:
Patients with CPPD might be treated with a number of medications that can affect pregancies including NSAIDs, anti-TNF biologics, and occasionally DMARDs. It is important for people with CPPD who want to become pregnant to discuss their medications with their doctor and create a pre-pregnancy plan. A plan is important for a number of reasons. For example, discontinuing a particular medication for safety of the newborn might cause CPPD to flare, so its wise to have a plan in place to handle these possibilities.
Any type of arthritis that affects the pelvis or the hips can make vaginal delivery difficult. It is important for those planning pregnancy to discuss delivery options with an obstetrician.
Following the basics of healthy eating can help improve health and well-being in everyone, including those with CPPD. Keeping a healthy weight helps reduce the load on weight-bearing joints including the spine, hips, and knees, making it easier to live with the disease.
Unfortuantely, no special diet has ever been proven to significantly alter the course of CPPD or any other types of arthritis.
In addition, no known natural remedies or complementary therapies have been proven to help CPPD in any significant way.
People with CPPD should consult with their doctor to make sure that any supplements or alternative therapies they consume will not negatively interact with their medications.
Its very important for people with CPPD to attend regularly scheduled appointments with their rheumatologist, and promptly get any tests that are ordered by their doctors.
It is often very important that medications are taken exactly as prescribed. Patients should always talk to their doctor about any issues they experience, or if they would like to change anything.
Calcium Pyrophosphate Deposition Disorder is a chronic disease and a rheumatologist is the best partner in helping patients achieve the best quality of life possible.