CPPD Deposition Disease

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Basics of cppd deposition disease

CPPD (Calcium Pyrophosphate Dihydrate) crystal deposition disease causes pain, swelling and sometimes redness and/or heat in one or many joints. It is defined by the presence of a distinctive type of crystal: calcium pyrophosphate dihydrate (CPPD).

Sometimes CPPD crystals can cause symptoms that resemble rheumatoid arthritis and/or osteoarthritis. Unlike these other diseases however the symptoms of CPPD crystal deposition disease are caused by tiny CPPD crystals.

Some people use other terms to describe this condition. In the past many doctors referred to CPPD crystal deposition disease as pseudogout because the crystals in both gout and CPPD crystal deposition disease can cause similar symptoms.

Prognosis

CPPD crystal deposition disease is a chronic (long-lasting) disorder with recurrent flare-ups. This means it may occur periodically throughout your lifetime. Some people have only one or at the most a few attacks in a lifetime. While there is not yet a cure for it it can be controlled fairly well with proper treatment in most cases.

CPPD crystal deposition disease should not prevent you from continuing most aspects of your normal lifestyle. Because there is no cure for it you may need to continue your treatment program even on those days when you feel better. Unless there has been serious damage to your joints you probably can go about your usual activities with only minor restrictions during flare-ups.

Incidence

Calcium Pyrophosphate Dihydrate (CPPD) crystal deposition disease usually occurs in people over 60. Apparently some change in the joint cartilage of older people allows or favors the formation of crystal deposits. It affects men and women equally.

CPPD crystal deposition disease can occur in younger people if:

  • there has been an injury to or an operation performed on a joint or
  • there is a family history of the disease or
  • the person has had other diseases that increase susceptibility to CPPD crystal deposition disease

Causes

The symptoms of CPPD crystal deposition disease are caused by two processes:

  • the presence of tiny CPPD crystals in the joints and
  • the body's reaction to these crystals.

In a healthy joint a firm rubbery material called cartilage covers the end of each bone. Cartilage acts as a cushion or shock absorber between the bones. Joint tissue is enclosed by a capsule. The capsule is lined by a thin material called the synovial membrane. The membrane releases a slippery or sticky fluid called the synovial fluids into the enclosed joint space. This liquid helps the joint tissues move smoothly and easily (see figure 1).

The CPPD crystals although first found in the joint cartilage also can occur later in the synovial membrane and synovial fluid (see figure 2). This probably is a result of their release from the cartilage. When crystals are present only in the cartilage they may cause no symptoms. The body is more likely to react to crystals released into the joint fluid.

Scientists don't know why the body forms CPPD crystals. In some instances these crystals occur because of an abnormality in the cartilage cells. In other instances another disease such as osteoarthritis occurs first and causes cartilage cells or connective tissue to become abnormal.

Other conditions sometimes occur in people with CPPD crystal deposition disease and may cause or aggravate the symptoms. If you have CPPD crystal deposition disease you should also be checked for:

  • Hemochromatosis: An inherited condition in which there is too much iron in the body. This can lead to diabetes or liver and heart problems. These problems are preventable if hemochromatosis is recognized in time.
  • Hyperparathyroidism: A condition caused by an overactive or hyperactive parathyroid gland in which there is too much calcium in the blood; this can lead to CPPD crystal deposition disease as well as bone and kidney problems.
  • Hypothyroidism: A condition caused by an underactive thyroid gland in which there are low levels of thyroid hormone; this may lead to extreme tiredness weight gain skin dryness and sensitivity to cold.
  • Hypomagnesemia: A condition of low levels of magnesium in the blood. Since magnesium is needed by the body to use pyrophosphate low levels can result in CPPD crystal deposition disease.

Your doctor may check for these and other lesser-known disorders. The possibility of them occurring needs to be considered as many are easily treated. CPPD crystal deposition disease may sometimes be an important clue in detecting other treatable and preventable conditions.

There is one other possible cause of CPPD crystal deposition disease--heredity/genetic makeup. In some cases CPPD crystal deposition disease may run in families and may be more common in some ethnic groups. Although CPPD crystal deposition disease is caused by calcium-containing crystals it is not caused by drinking milk or by calcium in your diet. It is not contagious so you can't give it to others or catch it from someone else. In some cases surgery a medical illness or a joint injury may trigger episodes of pain and swelling in the disease.

Click to enlarge

Figure 1 - A healthy joint

Click to enlarge

Figure 2 - Enlarged view of deposit sites of CPPD crystals in knee joint

Symptoms

Whatever the initial cause CPPD crystals can cause a variety of different problems.

The most dramatic symptoms occur when a number of crystals break loose from their location in the cartilage meniscus or synovial membrane and move into the joint space. This movement of the crystals into the joint can cause sudden and severe pain in the joint along with redness warmth and swelling (inflammation). Inflammation is one way the body can react to CPPD crystals. It sees them as "invaders" and sends disease-fighting white blood cells to attack them.

This joint pain may last for days or weeks. It resembles a gout attack (hence the old term "pseudogout"). It can occur in any joint but commonly occurs in the knee or wrist. A low-grade fever may accompany this form of the disease. In many people the symptoms go away even without treatment only to recur at some later time.

More often however CPPD crystals create less severe but longer lasting problems. These include stiffness swelling heat and pain that affect many joints. This often happens in the knees but can also happen in the wrists knuckles hips shoulders elbows ankles or toes. The pain is not sudden; instead it develops over a long period of time and can last many weeks or months. The pain may be worse the more you move your joints.

Over time either form of the disease may damage the cartilage and/or the meniscus of the knee. When this happens the bones rub together and cause a grating sensation and pain when the joint is used.

You may have only one of these patterns or you may have both. With either pattern you may have periods when your symptoms are worse than at other times. You also may have completely symptom-free periods.

Because CPPD crystal deposition disease often takes a long time to develop some people have no symptoms at all even when joint damage is already slowly occurring. Evidence of the CPPD crystals often can be seen on X-rays before symptoms are present.

Conditions with similar symptoms

The symptoms of CPPD crystal deposition disease are similar to symptoms of other diseases such as:

  • Gout causes sudden attacks of joint pain due to another type of crystal. This is most common at the base of the big toe but can occur anywhere.
  • Rheumatoid arthritis causes chronic swelling heat morning stiffness and pain in the joints and can also involve other tissues. The hands and fingers are involved in most cases.
  • Osteoarthritis causes joint pain and swelling due to damage to the cartilage in the joint.

Treatment

If you have CPPD deposition disease your treatment program will depend on how severe your disease is which type of symptoms you have and whether you also have another disease or form of arthritis along with CPPD crystal deposition disease.

The goals of treatment are to relieve pain maintain full joint movement and muscle strength and keep inflammation under control.

A treatment program may include a combination of joint aspiration occasional corticosteroid injections into affected joints medication rest exercise and joint protection. A few people with CPPD deposition disease may need surgery.

Exercise and therapy

Exercise can help strengthen the muscles around your joints help maintain full movement and use of your joints and keep your body physically fit.

Range of motion exercises are particularly helpful during times when CPPD crystal deposition disease is active. These are gentle exercises done without any weights and designed to preserve the mobility of the joint. The sore joint should be gently moved through its full available range of motion each day paying special attention to the end of the motion where mobility often is first lost. Using heat or cold applications before or after exercising can increase your comfort and the effectiveness of these exercises. Do range of motion exercises on a daily basis to maintain or restore motion after checking with your doctor.

During times when CPPD crystal deposition disease is not active strengthening exercises can be done to ensure maximum function and stability of your joints. These exercises should be taught by a physical or occupational therapist or physician preferably one who is familiar with CPPD crystal deposition disease.

Walking bicycling and aquatic exercises are often recommended for people with CPPD crystal deposition disease.

During aquatic exercise the buoyancy of the water usually protects the joints from rapid or stressful movement. Your doctor or physical therapist can help you plan an exercise program and can explain specific kinds of exercise to do during flares and remissions of the illness.

During a sudden attack of inflammation due to CPPD crystal deposition disease you may need to rest your sore joint(s). Sometimes splints are recommended for resting and protecting the joint(s). Splints canes and other devices for protecting joints can be fitted by physical or occupational therapists.

Medications

Some medicines are used for short term relief from pain. Others are used to keep the disease under long-term control. Nonsteroidal anti-inflammatory drugs (NSAIDs) are the medicines used most often. NSAIDs reduce joint pain and swelling and usually relieve symptoms within three to 10 days. NSAIDs may cause mild stomach up set in many people. For this reason doctors often recommend taking these drugs with fluids (such as water or milk) and with meals. If you are taking an NSAID check with your doctor about the best way to take the medication and how he or she wants to supervise its use.

Corticosteroids are a synthetic form of a natural body hormone cortisone. They may be injected directly into the sore joint or taken in pill form for a few days to relieve pain and swelling of an attack. These drugs can be very helpful but can cause serious side effects. Colchicine has been shown to be mildly effective in preventing joint pain and swelling as well as in treating sudden attacks of pain. Keep colchicine and other medications away from children.

As your symptoms improve your doctor may lower the dose of medicine. Some people will be able to stop taking all medications while others will have to continue taking some medicine to keep the disease under control. Never stop taking your medicine (even when you are feeling better) without checking with your doctor.

At the present time there are no medications available to eliminate the crystals. Those medications that currently are available only relieve symptoms.

A note about unproven remedies: Beware of anything called a "quick cure such as drugs, vitamins and minerals, or devices. These products do not cure CPPD crystal deposition disease; they only cost you money. Some may actually be harmful or keep you from getting proper care. Follow your doctor's advice closely and discuss any other forms of treatment you are considering.

Surgery

In a few patients surgery may be used to reduce pain and improve movement in a joint that is badly damaged or unstable. Some joints can be partially repaired by removing CPPD crystals or cartilage fragments through an arthroscope (a small scope that looks into a joint). Some joints especially the hip or knee can be replaced with an artificial joint made of plastic and metal.

Joint aspiration

Joint aspiration is the process of removing some fluid from the sore joint(s). This not only helps your doctor determine if you have the disease but it also removes many of the CPPD crystals that cause the pain and swelling. Removing the crystals also can help medications work better.

Credits

Some of this material may also be available in an Arthritis Foundation brochure. 

Adapted from the pamphlet originally prepared for the Arthritis Foundation by H. Ralph Schumacher M.D. Peter Hasselbacher M.D. This material is protected by copyright.

Condition research

Researchers continue to learn more about this illness and how it affects the body. They have identified other types of crystals in the joints that may cause some illnesses similar to CPPD crystal deposition disease. They also are studying the relationship between CPPD crystal deposition disease and other illnesses. They are trying to discover how to prevent crystal formation and how to speed crystal removal. This work may lead to new tests or treatments for this condition.