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Complex Regional Pain Syndrome

(CRPS; Reflex Sympathetic Dystrophy [RSD]; Causalgia; Sympathetically Maintained Pain)

Definition

Complex regional pain syndrome (CRPS) refers to a chronic condition that affects the nerves and blood vessels of one or more limbs. Its features include unpleasant burning sensations, swelling, sweating, color changes, and other symptoms.
There are two types of CRPS:
  • CRPS 1 (also called reflex sympathetic dystrophy or RSD)— No nerve damage is observable with this type.
  • CRPS 2 (also called causalgia)—This produces similar symptoms after a verified nerve injury has occurred.
Complex Regional Pain Syndrome
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Causes

The cause of CRPS is not known. The condition likely results from several factors. It may involve overactivity of the sympathetic nervous system. This directs automatic body functions that a person cannot control. Inflammation may also play a role in the disorder.

Risk Factors

Factors that may increase your risk of CRPS include:

Symptoms

If you have any of these symptoms do not assume it is due to CRPS. These symptoms may be caused by other conditions.
The upper limbs, particularly the hands, are most commonly affected. Symptoms progress and may vary during the course of the condition. Pain may spread from one side of the body to another. Many doctors describe symptoms in terms of stages.

Within Hours or Days After Injury

Symptoms may include:
  • Deep aching or burning pain, typically more severe than expected from the injury
  • Sensitivity to touch or even a light breeze
  • Swelling in the arm or leg
  • Unusual sweating
  • Excessively warm or cool skin
  • Hair and nails changes
Symptoms may increase with stress and worsen over time.

3-6 Months After Injury

Symptoms may include :
  • Burning pain above and below the initial injury
  • Swelling in the arm or leg that hardens
  • Muscle spasms or weakness
  • Extremity that become cold
  • Hair growth that is slow
  • Nails that become brittle or crack

Six Months or Later After Injury

Symptoms may include :
Symptoms may include :
  • A pale, blue, and/or shiny look to the skin
  • Limited joint movement
  • Muscle loss

Diagnosis

Your doctor will ask about your symptoms and medical history. A physical exam will be done. To diagnose CRPS, the doctor uses four criteria:
  • An initial traumatic or painful event to a limb, or keeping the limb still for a period of time
  • Continuing pain in the limb that is out of proportion to any stimulus, such as pain with even light touch
  • History of swelling, changes in skin blood flow, or temperature only in the affected limb
  • No other cause for the symptoms
Tests may be ordered to rule out other conditions. Your doctor may refer you to a pain specialist for further evaluation and management. Tests may include the following:
  • Pictures may need to be taken of your bodily structures. This can be done with:
  • The electrical activity in your nerves and muscles may need to be tested. This can be done with:
  • You may need to have your body's heat measured. This can be done with a thermogram.
  • You may need to have your autonomic nervous system evaluated. This can be done with quantitative sudomotor axon reflex test (QSART).

Treatment

Treatment aims to relieve pain and improve function. Visit the doctor as soon as possible. Early therapy may lead to better outcomes. In some cases, the condition goes away on its own; this is more common in children.
Treatment options include:
  • Biofeedback
  • Acupuncture
  • Physical therapy—Active and passive exercises help maintain function. Exercising in a warm pool may feel better than exercising on land.
  • Mirror box therapy—With this therapy, you place your affected hand or foot in a box, which has a mirror on one side. While moving your limb inside of the box, you move your unaffected limb in front of the mirror. To your brain, it appears that you are moving your painful limb easily and without discomfort. Mirror therapy may help to reduce pain and improve motor function in people who have CRPS due to stroke.
  • Nerve stimulation devices, such as:
    • Transcutaneous nerve stimulation (TENS)—A device worn on the skin surface that creates a tingling sensation and may relieve pain in some cases
    • Implantable spinal cord stimulation—may be helpful for some patients
  • Medicines, such as:
    • Anti-anxiety medicines, low-dose antidepressants, and drugs used to treat epilepsy
    • Pain medicines like narcotics
    • Bisphosphonates—drugs used to treat osteoporosis
    • Steroids
    • Medicines applied to the skin, such as Capsaicin and dimethylsulfoxide
  • Sympathetic nerve block—The injection of drugs that prevent signals from being sent along sympathetic nerves may temporarily relieve pain in some cases.
  • Sympathectomy—If the nerve block is successful, a surgeon can permanently destroy sympathetic nerves. In some cases, surgery can worsen symptoms.
  • Psychological support— Long-term pain often leads to depression or anxiety . Counseling is often required to help patients deal with chronic pain and loss of function.

Prevention

Quick mobilization after surgery or injury can help minimize the risk of CRPS in the affected limb. Other steps that might be helpful include:
  • Early use of pain relievers after trauma
  • Use of sympathetic nerve block after trauma
  • Desensitization techniques, if needed
  • Use of vitamin C may reduce the risk of CRPS after a wrist fracture.

RESOURCES

International Research Foundation for RSD/CRPS http://www.rsdfoundation.org

Reflex Sympathetic Dystrophy Syndrome Association http://www.rsds.org

CANADIAN RESOURCES

Health Canada http://www.hc-sc.gc.ca

Promoting Awareness of RSD and CRPS in Canada http://www.rsdcanada.org

References

Cecil RL, Goldman L, Bennett J. Cecil Textbook of Medicine. 21st ed. Philadelphia, PA: WB Saunders Company; 2000.

Complex regional pain syndrome. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated February 5, 2013. Accessed February 20, 2013.

Ferri FF. Ferri's Clinical Advisor: Instant Diagnosis and Treatment. St. Louis, MO: Mosby Inc; 2005.

Harden RN, Bruehl SP. Diagnosis of complex regional pain syndrome: signs, symptoms, and new empirically derived diagnostic criteria. Clinical Journal of Pain . 2006;22:415-419.

Complex Regional Pain Syndrome Fact Sheet. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/reflex%5Fsympathetic%5Fdystrophy/detail%5Freflex%5Fsympathetic%5Fdystrophy.htm . Updated September 19, 2012. Accessed February 20, 2013.

8/10/2007 DynaMed's Systematic Literature Surveillance DynaMed's Systematic Literature Surveillance : Zollinger PE, Tuinebreijer WE, Breederveld RS, Kreis RW. Can vitamin C prevent complex regional pain syndrome in patients with wrist fractures? A randomized, controlled, multicenter dose-response study. J Bone Joint Surg Am. 2007;89:1424-1431.

11/9/2009 DynaMed's Systematic Literature Surveillance DynaMed's Systematic Literature Surveillance : Cacchio A, DeBlasis, E, Necozione S, di Orio F, Santilla V. Mirror therapy for chronic complex regional pain syndrome type 1 and stroke. N Engl J Med. 2009;361(6):634-636.

Revision Information

  • Reviewer: Rimas Lukas, MD
  • Review Date: 03/2013 -
  • Update Date: 00/31/2013 -

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

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