Diseases

Calcium Pyrophosphate Deposition Disorder

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.

Calcium pyrophosphate deposition can cause calcification of the tendons and ligaments which can be severe and debilitating. This typically affects the tendons around the shoulder joints (rotator cuff) and the Achilles tendon (the heels) but any joint can be affected.

The disease is sometimes presents similar to gout because both diseases are caused by a build-up of crystals in the joints that trigger a response by the immune system.

Calcium pyrophosphate deposition 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.

Understanding Calcium Pyrophosphate Deposition Disorder

Symptoms

Calcium Pyrophosphate Deposition Disorder has a number of symptoms that are similar to many other arthritic diseases. The symptoms of calcium pyrophosphate deposition disorder depend on the arthritic disease it mimics.

The most common form of Calcium pyrophosphate deposition disorder is calcification of tendons and other structures around joints. This can be severe and debilitating.

Tendonitis-like Symptoms

Calcium pyrophosphate deposition 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. Calcium pyrophosphate deposition 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)

Calcium pyrophosphate deposition can cause joints to become hot, intensely painful, red, and swollen in an “attack” that is similar to a disease called gout. This can also be confused with an infection in the joint.

In calcium pyrophosphate deposition disorder 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 calcium pyrophosphate deposition disorder is also called pseudogout.

Osteoarthritis-like Symptoms

Calcium pyrophosphate deposition disorder can look like osteoarthritis when it causes pain and swelling in joints that are commonly affected by osteoarthritis, such as the small joints of the hands or the knees. Calcium pyrophosphate deposition can also impact joints that are not typically affected by osteoarthritis 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 osteoarthritis, then it might be calcium pyrophosphate deposition disorder.

Rheumatoid Arthritis-like Symptoms

Calcium pyrophosphate deposition disorder can affect the knuckles of the hand (metacarpophalangeal joints) causing the hands to become swollen, painful, and stiff in a very similar way to Rheumatoid Arthritis. An X-ray of the hand can help tell the difference because in calcium pyrophosphate deposition disorder it will not show changes that would typically be caused by Rheumatoid Arthritis and will look more like osteoarthritis. People with this type of calcium pyrophosphate deposition can rarely have high levels of iron in their blood (hemochromatosis).

Other Diseases Associated with Calcium Pyrophosphate Deposition Disorder

Other diseases are associated with calcium pyrophosphate deposition 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).

Diagnosis

It can be difficult to diagnose calcium pyrophosphate deposition disorder because its symptoms are very similar to other arthritic diseases including gout, osteoarthritis, rheumatoid arthritis, and tendonitis.

Calcium pyrophosphate deposition disorder is best diagnosed by a rheumatologist, a type of doctor that specializes in arthritis and autoimmune disease.

To diagnose calcium pyrophosphate deposition disorder , a rheumatologist 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 Calcium Pyrophosphate Deposition Disorder

Scans

X-rays: X-rays of the affected joints can be very useful to see typical features of calcium pyrophosphate deposition disorder . The calcium pyrophosphate 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.

Synovial (Joint) Fluid Test

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

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 Calcium Pyrophosphate Deposition Disorder: Tests for levels of thyroid hormone and parathyroid hormone check for thyroid and parathyroid conditions, an iron (ferritin) test looks for hemochromatosis (too much iron), and a magnesium test looks for low-levels (hypomagnesemia).

Causes

Calcium pyrophosphate deposition disorder 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 calcium pyrophosphate deposition or make it worse.

Treatments

Calcium pyrophosphate deposition disorder 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 calcium pyrophosphate deposition to lead active and productive lives

Cortisone (Steroid) Injections

Cortisone injections can be very effective in people with calcium pyrophosphate deposition that experience pseudogout symptoms, Rheumatoid Arthritis-like symptoms, symptoms associated with osteoarthritis, and calcium pyrophosphate deposition that causes inflammation in tendons. This type of treatment can reduce the pain and swelling caused by calcium pyrophosphate deposition disorder .

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 3 or 4 in a single joint per year. This can also be effective treatment when symptoms are more like osteoarthritis (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 calcium pyrophosphate deposition disorder and also help to reduce symptoms such as pain. They can be used for people with pseudogout, tendonitis, Rheumatoid Arthritis-like symptoms, or calcium pyrophosphate deposition that is associated with osteoarthritis.

Non-Steroidal Anti-Inflammatory Drugs are an important treatment option in cases where joints can’t be easily injected with cortisone. Some people may need to use them 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.