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Complex regional pain syndrome

CRPS; RSDS; Causalgia - RSD; Shoulder-hand syndrome; Reflex sympathetic dystrophy syndrome; Sudeck atrophy; Pain - CRPS

Complex regional pain syndrome (CRPS) is a long-term (chronic) pain condition that can affect any area of the body, but often affects an arm or a leg.

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Causes

Doctors are not sure what causes CRPS. In some cases, the sympathetic nervous system plays an important role in the 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.

CRPS has two forms:

CRPS 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, feeling (sensation), and temperature to the affected area. This leads to problems in the:

Possible causes of CRPS:

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.

This condition is more common in people ages 40 to 60, but younger people can develop it, too.

Symptoms

The key symptom is pain that:

In most cases, CRPS has three stages. But, CRPS does not always follow this pattern. Some people develop severe symptoms right away. Others stay in the first stage.

Stage 1 (lasts 1 to 3 months):

Stage 2 (lasts 3 to 6 months):

Stage 3 (irreversible changes can be seen)

If pain and other symptoms are severe or long-lasting, many people may experience depression or anxiety.

Exams and Tests

Diagnosing CRPS can be difficult, but early diagnosis is very important.

The health care provider will take a medical history and do a physical examination. Other tests may include:

Treatment

There is no cure for CRPS, but the disease can be slowed. The main focus is on relieving the symptoms and helping people with this syndrome live as normal a life as possible.

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

Medicines may be used, including pain medicines, corticosteroids, certain blood pressure medicines, bone loss drugs and antidepressants.

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

Surgical or invasive techniques that may be tried:

Outlook (Prognosis)

The outlook is better with an early diagnosis. If the doctor diagnoses the condition in the first stage, sometimes signs of the disease may disappear (remission) 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 other people, even with treatment the pain continues and the condition causes crippling, irreversible changes.

Possible Complications

Complications that may result include:

Complications can also occur with some of the nerve and surgical treatments.

When to Contact a Medical Professional

Contact your provider if you develop constant, burning pain in an arm, leg, hand, or foot.

Prevention

There is no known prevention at this time. Early treatment is the key to slowing the progression of the disease.

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, Tyburski MD, Harden RN. Chronic pain. In: Cifu DX, ed. Braddom's Physical Medicine and Rehabilitation. 5th ed. Philadelphia, PA: Elsevier; 2016:chap 37.

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Review Date: 2/4/2020  

Reviewed By: Amit M. Shelat, DO, FACP, FAAN, Attending Neurologist and Assistant Professor of Clinical Neurology, Stony Brook University School of Medicine, Stony Brook, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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