Complex Regional Pain Syndrome (CRPS; also called Reflex Sympathetic Dystrophy or causalgia) is a rare, disabling disease that is characterized by pain and a variety of other symptoms such as color or temperature changes of the affected part; swelling; neurological and muscle dysfunction; and disorders of skin, nails, hair, bone and the immune system. The number and collection of certain of these symptoms determines the diagnosis. It can be a serious illness that usually occurs in response to trauma such as a severe sprain, bone fracture, crush injury or surgery. It can be disabling and negatively impact many aspects of a person’s life, including daily activities, relationships, work or school. ​

 

CRPS is classified as a rare disorder by the United States Food and Drug Administration. However, up to 200,000 individuals may experience this condition in the United States in any given year.

 

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Diagnosis

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Recently, standard diagnostic criteria have been established, and must be determined by a doctor skilled and experienced in the disorder.  Any trained doctor can make the diagnosis if the criteria are followed, but often it takes a pain specialist to diagnose CRPS definitively. The diagnosis can be made in a doctor’s office without any special diagnostic tests. Early diagnosis is generally the key to better outcomes, however, diagnosing CRPS/RSD is not a simple matter and many patients may search months (or even years) for a definitive diagnosis of CRPS. Lists of doctors who are experienced in the diagnosis and treatment of CRPS can be found on the Reflex Sympathetic Dystrophy Syndrome Association of America’s website.

 

 

Diagnostic features of CRPS

 

• ​Pain that is described as deep, aching, cold, and/or burning

• Increased skin sensitivity

• An initiating injury or traumatic event, such as a sprain, fracture, surgery, etc., that should not cause prolonged severe pain and where the pain does not subside with healing

• Pain (moderate-to-severe) associated with allodynia, that is, pain from something that should not cause pain, such as the touch of clothing or a shower

• Continuing pain (moderate to severe) associated with hyperalgesia (that is, heightened sensitivity to painful stimulation)

• Abnormal swelling in the affected part

• Abnormal hair or nail growth

• Abnormal skin color changes

• Abnormal skin temperature (i.e., the affected part is warmer or colder than the unaffected part)

• Abnormal sweating of the affected area

• Limited range of motion, weakness, or other motor disorders such as paralysis or dystonia (twitching or involuntary repetitive movements)

• Symptoms and signs can wax and wane

• Can affect anyone, but is more common in women, with a recent increase in the number of children and adolescents who are diagnosed

 

 

The disease always starts in a limb but can sometimes spread toward the center of the body. Only rarely will the disease spread to distant parts of the body, but this is usually due to changes in the brain as a response to severe pain. CRPS pain can sometimes be persistent and intense. As a consequence, the extreme pain can disrupt many features of life, so sometimes patients may suffer from other disorders such as insomnia, depression and anxiety. 

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Treatment

 

The persistent pain and disability associated with CRPS/RSD requires coordinated, patient-centered care by teams of different types of experienced professionals to achieve pain reduction/cessation and better function. Usually an experienced specialist is needed to coordinate these multiple therapies and types of medical professionals. Doctors who have this special training often are physiatrists (rehabilitation doctors), pain specialists, anesthesiologists, and psychologists.

 

Treatment goals are not only to relive pain but to improve function and restore normal activities of daily living.

 

Important features of proper CRPS treatment are medications, physical therapy, occupational therapy, and behavioral psychotherapy. Certain very specialized treatments such as biofeedback and mirror box therapy can also help. If the simpler therapies are not effective, nerve blocks, corrective surgery, and in rare cases spinal cord stimulators, may be needed in specified cases.

 

 

Prepared by: 

Norman Harden MD

Professor, Northwestern University

Chairman of the International Research Consortium for CRPS