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Complex Regional Pain Syndrome

Complex Regional Pain Syndrome (CRPS) may not have a cure, but at UF Health, our dedicated team of pain medicine specialists is committed to slowing the progression of the disease and alleviating your symptoms. A pain specialist in our chronic pain clinic will use a holistic approach that focuses on pain management and improving your quality of life, empowering you to live as normally as possible.

What is complex regional pain syndrome?

Complex regional pain syndrome (CRPS) is a debilitating chronic pain condition that typically affects one limb following an injury but can also occur without any apparent cause. It is characterized by intense, persistent nerve pain, also known as neuropathic pain, and is accompanied by symptoms such as swelling and localized changes in skin color and temperature. The severity and duration of symptoms can vary, with some individuals experiencing long-term disability while others may see symptoms improve over time​.

Complex regional pain syndrome has two types, though treatment for both is similar.

  • Patients with symptoms of complex regional pain syndrome but without evidence of a specific nerve injury are classified as having CRPS-1, previously called reflex sympathetic dystrophy disorder.
  • Patients with symptoms of complex regional pain syndrome, and who have confirmed associated nerve damage, are classified as having CRPS-2. CRPS-2 was previously called causalgia.

What causes complex regional pain syndrome?

In most patients, complex regional pain syndrome is triggered by an injury and is thought to result from damage to the nervous system. This includes the nerves that control the blood vessels and sweat glands.

The damaged nerves are no longer able to properly control blood flow, sensation, and temperature in the affected area. This leads to problems in blood vessels, bones, muscles, nerves, and skin.

Most cases of CRPS are in patients who have suffered from a fracture in a limb, which was then immobilized in a cast.

But while CRPS is usually associated with trauma or injury, doctors are not sure why it is triggered in some patients but not others. In some cases, the sympathetic nervous system plays an important role in the chronic pain. Another theory is that CRPS is caused by a triggering of the immune response, which leads to the inflammatory symptoms of redness, warmth, and swelling in the affected area.

Possible causes of CRPS include direct injury to a nerve and injury or infection in an arm or leg.

In rare cases, sudden illnesses such as a heart attack or stroke can cause CRPS. The condition can sometimes appear without obvious injury to the affected limb.

What are the risk factors for complex regional pain syndrome?

CRPS can occur in anybody but is more common in women and peaks around 40 years of age. It is rare in the elderly and uncommon in young children.

Those with a family history of CRPS may be more likely to develop it early in life. Patients who smoke or have poor circulation, diabetes, autoimmune disorders, or previous injuries involving nerve damage may also be at higher risk.

What are CRPS symptoms?

The key symptom is severe pain that is prolonged and:

  • Is intense and burning and is much stronger than would be expected for the type of injury that occurred
  • Gets worse over time
  • Begins at the point of injury, but may spread to the whole limb, or to the arm or leg on the opposite side of the body

In most cases, CRPS has three stages. However, progression through the stages is not the same for everybody. Some people develop severe symptoms right away and others stay in the first stage.

Stage 1 (lasts 1 to 3 months):

  • Changes in skin temperature, switching between warm or cold
  • Faster growth of nails and hair
  • Muscle spasms and joint pain
  • Severe burning, aching pain that worsens with the slightest touch or breeze
  • Changes in the texture of the skin; it may become blotchy, or it may appear shiny and thin

Stage 2 (lasts 3 to 6 months):

  • Continued changes in the skin
  • Nails that are cracked and break more easily
  • Pain that continues to grow worse over time
  • Slower hair growth
  • Stiff joints and weak muscles

Stage 3 (irreversible changes can be seen)

  • Limited movement in limb because of tightened muscles and tendons, also known as contracture
  • Muscle wasting
  • Pain in the entire limb

What are some possible complications of complex regional pain syndrome?

Complications that may result include:

  • Problems with thinking and judgment
  • Depression or anxiety
  • Loss of muscle size or strength in the affected limb
  • Spread of the disease to another part of the body

How is complex regional pain syndrome diagnosed?

There is no single test which can confirm CRPS, and so diagnosing it can be difficult. Additionally, many patients experience gradually improving symptoms over time, sometimes making it more difficult to diagnose as time goes on.

A health care provider will make a careful review of a patient’s medical history and perform a physical examination. Other tests may include:

  • A test to show temperature changes and lack of blood supply in the affected limb, which is called thermography
  • Bone scans
  • Nerve conduction studies and electromyography (usually done together)
  • X-rays or magnetic resonance imaging (MRI)
  • Autonomic nerve testing, which measures sweating and blood pressure

Comprehensive testing will also help rule out whether a patient has a different condition with similar symptoms, such as arthritis or a muscle disease.

What are the treatment options for complex regional pain syndrome?

The main focus of treatment and pain management is on relieving the symptoms and helping people with this syndrome live as normal a life as possible.

Physical therapy and occupational therapy should be started early. Starting an exercise program and learning to keep joints and muscles moving may prevent the disease from getting worse. It can also help patients with everyday activities.

There are currently no medicines which have been specifically approved by the FDA for use in treating CRPS. Medications such as pain medicines, corticosteroids, certain blood pressure medicines, bone loss drugs, and antidepressants may provide pain relief in some patients.

Some type of talk therapy, such as cognitive behavioral therapy or psychotherapy, can help teach the skills needed to live with chronic pain.

Surgical or invasive techniques that may be tried:

  • Nerve blocks which is injected medicine that numbs the affected nerves or pain fibers around the spinal column.
  • An intrathecal drug pump which is an internal pain pump that directly delivers medicines to the spinal cord.
  • A spinal cord stimulator, which involves placing electrodes (electrical leads) next to the spinal cord. A low-level electrical current, used to create a pleasant or tingling sensation in the painful area, is able to reduce pain in some people.
  • A dorsal root ganglion stimulator, which is similar to a spinal cord stimulator but specifically targets the dorsal root ganglia, which are clusters of sensory nerve cell bodies, to deliver electrical pulses that modulate pain signals at a more targeted level.
  • Surgery that cuts the nerves to destroy the pain, known as surgical sympathectomy, although some experts oppose this treatment as it may make symptoms worse in some patients.

Addressing factors which may impact the body’s ability to heal such as diabetes, tobacco use, or malnutrition may also improve a patient’s odds of recovering quickly.

No treatment or combination of treatments is guaranteed to be effective in every patient, and many treatments carry their own risks of complications. Each patient should work with their health care provider to develop a safe, personalized treatment plan.

What to expect with CRPS

Just as CRPS has different causes in different patients, outcomes are also highly variable from one patient to the next. Children and adolescents have a generally better prognosis than adults or the elderly.

Early diagnosis is always preferable. An early diagnosis allows for early and aggressive treatment of symptoms. If CRPS is diagnosed in the first stage, sometimes signs of the disease may disappear and normal movement is possible.

If the condition is not diagnosed quickly, changes to the bone and muscle may get worse and may not be reversible.

In some people, symptoms go away on their own. In others, CRPS pain continues even with treatment and the condition causes crippling, irreversible changes.

When to contact your doctor

Contact your provider if you develop constant pain in an arm, leg, hand, or foot. Some patients describe the pain as burning, pins and needles, or as a squeezing sensation.

References

Aburahma AF. Complex regional pain syndrome. In: Sidawy AN, Perler BA, eds. Rutherford's Vascular Surgery and Endovascular Therapy. 9th ed. Philadelphia, PA: Elsevier; 2019:chap 192.

Gorodkin R. Complex regional pain syndrome (reflex sympathetic dystrophy). In: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. 7th ed. Philadelphia, PA: Elsevier; 2019:chap 90.

Stanos SP, Mark D T, Harden RN. Chronic pain. In: Cifu DX, ed. Braddom's Physical Medicine and Rehabilitation. 6th ed. Philadelphia, PA: Elsevier; 2021:chap 37.

https://permanent.fdlp.gov/gpo175228/CRPSFactSheetE508C.pdf

https://www.ninds.nih.gov/health-information/disorders/complex-regional-pain-syndrome

Deer TR, Levy RM, Kramer J, et al. Dorsal root ganglion stimulation yielded higher treatment success rate for complex regional pain syndrome and causalgia at 3 and 12 months: a randomized comparative trial. Pain. 2017;158(4):669-681. doi:10.1097/j.pain.0000000000000814

Oaklander A. Complex Regional Pain Syndrome (Reflex Sympathetic Dystrophy) & Posttraumatic Neuralgia. In: Stone JH. eds. Current Diagnosis & Treatment: Rheumatology, 4e. McGraw Hill; 2021. Accessed May 22, 2024. https://accessmedicine.mhmedical.com/content.aspx?bookid=3017§ionid=253715012

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