Complex Regional Pain Syndrome (CRPS)

What is complex regional pain syndrome (CRPS)?

Complex regional pain syndrome (CRPS) is a condition that causes pain, changes in skin color and other symptoms in a certain part of your body — usually in your extremities. Your extremities include your arm, leg, hand or foot.

The symptoms of CRPS can greatly impact the function of your affected limb, sleep, daily activities and your mental health.

Experts believe that CRPS occurs as a result of dysfunction in your central or peripheral nervous systems. Your central nervous system consists of your brain and spinal cord. Your peripheral nervous system relays information from your brain and spinal cord to your organs, arms, legs, fingers and toes. The abnormal functioning results in an overreaction to pain signals that your nervous system can’t shut off.

There are two subtypes of CRPS:

• Type I: This type occurs without nerve damage. It happens after an illness or injury that didn’t directly damage a nerve. Type I was formerly known as reflex sympathetic dystrophy.
• Type II: This type occurs after known nerve damage. It was formerly known as causalgia.

CRPS can also either be acute (short-term) or chronic (lasting longer than six months). It’s usually treatable.

Who does CRPS affect?

CRPS more commonly affects adults than children. The peak onset is around 40 years of age. CRPS affects people assigned female at birth more often than people assigned male at birth.

About 66% to 80% of cases occur in people of European ancestry.

How common is CRPS?

CRPS is relatively rare. It affects about 200,000 people every year in the United States.

What are the symptoms of CRPS?

How severe and how long symptoms of complex regional pain syndrome (CRPS) last varies from person to person.

Symptoms of CRPS typically start within four to six weeks after an injury, fracture or surgery, but they can develop without a known cause.

The most common and prominent symptom of CRPS is pain. The pain is constant or intermittent and is a burning, stinging or tearing sensation. It’s often deep inside your affected limb.

Sensory changes are also common in the affected area and may include:

• Increased sensitivity to painful stimuli (a pinch may feel more painful than usual).
• Feeling pain from stimuli that are usually not painful (such as just touching your skin).
• Numbness.

Other symptoms of CRPS in the affected area include:

• Skin swelling: Swelling may come and go or remain constant.
• Decreased function in your affected limb: You may experience a decreased ability to move your affected limb and/or increased stiffness. You may also have difficulty placing pressure on your affected limb or joint.
• Changes in skin temperature: The skin on your extremity may feel warmer or cooler compared to the opposite one.
• Changes in skin color: Your skin may appear blotchy, pale, purple/bruised or red.
• Changes in skin texture: Your skin may become shiny and thin or excessively sweaty.
• Changes in nail and hair growth: You may have rapid hair or nail growth or no growth.

Since the symptoms of CRPS usually improve over time, it’s easiest for healthcare providers to diagnose it in the early stages. It’s important to see a provider soon after you experience symptoms of CRPS.

What causes CRPS?

Researchers aren’t sure why some people develop CRPS while others with similar injuries don’t. They think it’s due to an inflammatory or immune reaction in both your peripheral and central nervous systems.

In more than 90% of cases, CRPS results from nerve trauma or injury to the affected limb that damages the thinnest sensory and autonomic nerve fibers. These small fibers transmit pain, itch and temperature sensations. They also control the small blood vessels and the overall health of surrounding cells.

The most common injury associated with developing CRPS is a bone fracture, especially a wrist fracture. A displaced or splintered bone or pressure from a tight cast can damage nerves.

Other common injuries that can lead to CRPS include:

• Surgery: A surgical incision, stitches or scarring can cause nerve injury.
• Sprains or strains: When connective tissues are damaged, it can result in excessive movement of a joint, which over-stretches nearby nerves.
• Burns, bruises or cuts: These are all noticeable signs of injuries that may also have damaged underlying nerves.

CRPS can also develop without an obvious injury or due to periods of prolonged immobilization.

Certain factors that may increase your risk of developing CRPS include:

• Poor nerve health: Conditions such as diabetes can leave your nerves less resilient and able to repair themselves. It may be difficult for people with peripheral neuropathy to regrow their nerve cells from an injury that wouldn’t otherwise cause problems. Smoking and previous chemotherapy can also make it difficult for your nerves to regenerate.
• Immune system issues: Your immune system plays a large role in inflammation. Some people with CRPS have elevated levels of inflammatory chemicals called cytokines that contribute to certain symptoms of CRPS. CRPS is also more common in people with other inflammatory and autoimmune diseases, such as asthma.
• Genetics: Your genes can affect your ability to recover from an injury. Researchers have discovered family clusters of CRPS. Although this is rare, it suggests that there may be a genetic link to CRPS.

How is CRPS diagnosed?

Since CRPS is rare and healthcare providers don’t totally understand it, it’s often misdiagnosed.

There’s no specific test to diagnose CRPS. Healthcare providers mainly diagnose it through a careful medical history, physical examination and review of your symptoms. Your provider will ask you if you’ve had a recent injury or surgery.

They will look for:

• A change in the appearance, temperature and texture of your skin in the affected area.
• A higher-than-expected amount of pain from an injury.
• Any other disease or conditions that could cause your pain, changes in your skin or other symptoms.

They may order imaging tests, such as an ultrasound or magnetic resonance imaging (MRI), to look for underlying nerve damage. But it’s not always possible to find the nerve damage.

Your provider may also order other tests to rule out conditions that cause similar symptoms. For example, electromyography (EMG) may reveal other causes of neuropathy, which may result in some similar pain scenarios.

What is the treatment for CRPS?

The goal of treatment is to decrease your pain and other symptoms, restore function to the affected limb and maintain the quality of your life.

It’s important to start treatment early in the course of CRPS. This is because CRPS can cause the affected limb to stiffen over time. In addition, the pain usually worsens without treatment and movement becomes more and more difficult.

It’s best to see healthcare providers who have experience in treating CRPS, if possible. Treatment requires a combination of carefully managed approaches, including:

• Physical therapy and occupational therapy.
• Lifestyle changes.
• Psychosocial and behavioral therapy.
• Medications.
• Alternative therapies for pain management.

For young children with CRPS, psychosocial and rehabilitation therapies are the mainstay treatment. Healthcare providers typically only suggest pain-reducing medical procedures for older adolescents who have symptoms that aren’t responding to other therapies.

Physical therapy and occupational therapy

Physical therapy is one of the most important methods of treatment for CRPS.

A physical therapist can help improve blood flow to your affected limb as well as increase your flexibility, strength, muscle tone and function with certain exercises. An occupational therapist can teach you new ways to accomplish everyday tasks.

Other aspects of physical therapy for CRPS include:

• Graded motor imagery: This is a set of treatments that help reduce pain and improve the ability to move the affected part of your body. When one of your limbs is painful, it can become difficult to touch, move or use. Your brain also has a memory or “map” of different body parts and how these feel, move and function in daily activities. If you don’t touch, move or use the affected limb, this map starts to change and can begin to disappear. However, you can change this map or re-established it with graded motor imagery.
• Mirror therapy: This treatment technique is one of the exercises of graded motor imagery. It involves looking at the reverse image of your non-painful limb in a mirror. The reflection in the mirror then appears as the affected limb. This can “trick” your brain into thinking that the painful limb now feels and moves as normal.
• Desensitization: This technique involves touching the affected area with materials of different textures and weights and placing the affected limb into water of warmer and cooler temperatures. By exposing the affected area/limb to different sensations slowly over time, your brain adjusts to the sensations and pain begins to lessen.

Lifestyle changes

Removing certain barriers that can prevent your nerves from healing can help increase the chance of recovery and the speed of recovery, including:

• Quitting smoking: Smoking greatly interferes with nerve regeneration.
• Managing existing health conditions: Poor management of diabetes and other conditions that can affect circulation and nerves can make it difficult for your nerves to heal.

Other simple steps you can take at home to help your symptoms include:

• Keeping your affected limb elevated when resting or sleeping can help excess fluid return to your heart.
• Exercising every day is critical to improving circulation to the damaged nerves. Talk to your provider or a physical therapist about an exercise plan that’s appropriate for you.
• Wearing compression stockings or sleeves may help limit swelling, particularly when standing. Ask your provider if this is an option for you.

Psychosocial and behavioral therapy

Having CRPS is associated with worsening anxiety, depression and stress, which can increase pain. Psychotherapy can help. Psychotherapy, also called talk therapy, is a term for a variety of treatment techniques that aim to help a person identify and change unhealthy emotions, thoughts and behaviors.

If possible, it’s best to see a pain management clinical psychologist with training in treating CRPS. During psychotherapy, you talk to the psychologist to learn ways to better cope with factors that contribute to your pain and other symptoms.

Medications

No medications are specifically approved for CRPS in the U.S. However, your healthcare provider may recommend certain medications to manage your symptoms.

Medications that have pain-reducing effects include:

• Nonsteroidal anti-inflammatory drugs (NSAIDs), such as acetaminophen, ibuprofen and naproxen.
• Topical analgesic creams and patches, such as lidocaine.
• Certain antidepressants, such as amitriptyline and duloxetine.
• Anti-seizure drugs, such as gabapentin, pregabalin and topiramate.
• Bisphosphonates, such as alendronate.
• Botulinum toxin (Botox) injections.

The choice of medication(s) to begin with varies from person to person. Your healthcare provider will consider certain factors, including:

• Your age.
• Other existing health conditions.
• Any current medications you’re taking.
• The potential for medication side effects or interactions with the current medications you’re taking.

Alternative therapies for pain management

Alternative therapies for pain management may include:

• Biofeedback.
• Acupuncture.
• Hypnosis.
• Reiki.
• Chiropractic.

If your CRPS hasn’t responded well to the therapies mentioned above or you have severe pain or ongoing CRPS, your provider may recommend the following more invasive treatments:

• Trigger point/tender point injections: You may have trigger/tender points in the muscles in your shoulder girdle when CRPS is limited to your upper limb. Injection of each trigger/tender point with local anesthetics with or without glucocorticoids can help treat this type of CRPS in the early stage.
• Sympathetic nerve blocks: These blocks can provide significant pain relief for some people. The lumbar sympathetic block involves injecting an anesthetic next to your spine in your lower back. This directly blocks the sympathetic chain that transmits the pain signal from your lower limbs to your spinal cord and brain. For upper limb CRPS pain, the block is called stellate ganglion block, which involves injecting an anesthetic on the side of your neck.
• Spinal cord stimulation: This treatment involves surgically implanting a pulse generator device under your skin in your abdomen or buttock and wires (electrodes) near your spinal cord. The device, which is similar to a pacemaker, sends low-level electrical currents to your spinal cord. Electrical pulses interfere with and reduce the pain signal being sent to your brain.
• Dorsal root ganglia stimulation: This treatment is similar to spinal cord stimulation except that the smaller wires are implanted right on the dorsal root ganglion, which is a cluster of sensory neurons at the junction where each segmental nerve exits your spinal cord. The dorsal root ganglion is a relay station of the pain signal being sent from the peripheral nervous system (your limbs) to the central nervous system (your spinal cord and brain). The stimulation could be a more targeted and efficient (requiring less stimulation) therapy, as it concentrates on these small relay stations.
• Peripheral nerve stimulation: This treatment places a stimulating wire near a major nerve in your limb that covers the painful area. It works by interfering with the pain signal conducted through the targeted nerve.
• IV ketamine infusion: This treatment uses an IV infusion of a low dose of ketamine for 3 to 5 days. Ketamine works by blocking a particular molecule (NMDA receptor) in your nervous system that can wind up and worsen your pain.
• Intrathecal drug pumps: This treatment involves using an implanted catheter to send pain-relieving drugs, such as ziconotide (Prialt®), right into your spinal fluid.

Can I prevent CRPS?

Since researchers don’t know the exact cause of CRPS, there’s no conclusive way to prevent it. Some studies have revealed that taking vitamin C before a future surgery might prevent CRPS.

What can I expect if I have CRPS?

Each person’s experience with CRPS is different. CRPS usually improves over time and eventually goes away (goes into remission) in most people.

Severe or prolonged cases, which are rare, can greatly affect your life. In some people, CRPS gets worse and even spreads to other areas of their body. Experiencing increased psychological distress during the injury that led to CRPS may affect its severity and prognosis.

CRPS recurs (happens again) in about 10% to 30% of people. Most recurrences are due to unknown reasons.

The most important goals are to relieve pain and restore movement and strength in the affected limb. By achieving pain relief, you increase the odds of improving its function and your quality of life. Carefully selected treatment plans allow some people with CRPS to successfully manage their pain and lead active lives.

When should I see my healthcare provider?

Because of the complexity of this condition and the fact that it’s often misdiagnosed, seek out a pain management specialist or a specialty pain center with knowledge of CRPS if:

• You think you have symptoms of CRPS.
• Your symptoms are getting worse.
• Your condition hasn’t responded to other treatment methods.

Is CRPS considered a disability?

In the United States, the Social Security Administration recognizes CRPS as a potential cause of disability depending on its severity and impact on your life. Consult government officials for more information on the Social Security Administration’s eligibility requirements.

Is CRPS a mental illness?

No, CRPS isn’t a mental health condition. It’s a neurological condition. CRPS can, however, cause or worsen anxiety, depression and stress. It can sometimes lead to post-traumatic stress disorder (PTSD).

A note from Cleveland Clinic

Complex regional pain syndrome (CRPS) can greatly affect your quality of life. The good news is that there are many treatment options and many combinations of options that you can try. The earlier you receive a CRPS diagnosis and start treatment, the better the chance that your symptoms will respond to treatment. If you think you may have CRPS, talk to your healthcare provider. They’re available to help you.