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Speaker: Complex regional pain syndrome can be difficult to diagnose
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WASHINGTON, D.C. — Complex regional pain syndrome can be challenging to diagnose properly, but its main features are sensory, motor, sudomotor and motor/trophic disturbances, according to Jennifer A. Sweet, MD, who presented at the American Association of Neurological Surgeons.
The Budapest criteria, she explained, is followed to identify complex regional pain syndrome (CRPS). The diagnosis can be one of exclusion, according to Sweet.
“It can be a really tricky disorder to work with,” she said. “The underlying pathology of this disease is a little unclear.”
A large number of patients undergo surgery or experience an injury to the limb prior to experiencing symptoms, according to Sweet. The disease is classified as either CRPS-1, in which no underlying nerve damage is present, or as CRPS-2, in which nerve injury is associated with symptoms.
Dermatological findings can be useful in making a diagnosis, Sweet said. Erythema, edema, atrophy, hyperhidrosis, hypohidrosis, changes in the nails, changes in the skin and changes in bones can be present, and symptoms can be mild, transient or very severe.
“What is important about this disorder is that there can be a lot of stages of progression of the disease,” Sweet said. “We know that if we intervene early enough, we can have an important impact on ... how these patients do.”
Sweet said between 75% and 80% of patients show improvement after the first year of diagnosis, especially if they are treated early; however, 15% to 20% of patients become refractory and difficult to treat. In stage one, the primary complaint is usually neuropathic limb pain and no obvious changes on imaging, but intervention at this stage can be important, according to Sweet.
“If we wait too long, patients can progress to stage two,” she said. “This can happen as early as 3 to 6 months after diagnosis.”
During this stage, worsening of all of the symptoms, including radiographic symptoms, occurs, Sweet said.
Stage three involves significant clinical symptoms, including motor and trophic changes, radiographic findings and diffuse osteopenia, particularly in joints.
Risk factors include female gender, post-menopause status, fractures or dislocations at the distal radius or the ankle, or immobilization of a limb. Depression and psychological factors, Sweet said, are not risk factors.
Another challenge of diagnosis is that CRPS can be confused with cellulitis or osteomyelitis, according to Sweet.
“We think there might be a proinflammatory state that puts patients more at risk, or that occurs in the process,” Sweet said. “There might be an association with autoimmunity.” Sweet added patients with CRPS often have other autoimmune disease, such as multiple sclerosis, but the presence of an autoimmune disease is not diagnostic of CRPS.
Treatment requires a multidisciplinary approach, according to Sweet. Pain medication; physical rehabilitation; and psychological intervention, such as cognitive behavioral therapy and patient education, are important treatments, she said. Pharmacological treatments include anti-inflammatory drugs, analgesics, vasodilators, calcium channel blockers and calcitonin bisphosphonate, which can improve outcomes.
Mobilizing the limb is also important to avoid progression of the disease, Sweet said, adding that patients may need new or additional orthopedic surgery if CRPS follows an injury; however, symptoms should be resolved before undergoing the procedure.
“We prefer to wait, if possible, until many of their CRPS symptoms are less pronounced,” she said. - by Shirley Pulawski
Reference:
Sweet JA. Complex regional pain syndrome: diagnosis and management. Presented at: American Association of Neurological Surgeons. May 2-6, 2015; Washington, D.C.
Disclosure : Sweet reports no relevant financial disclosures.