Complex Regional Pain Syndrome in Emergency Medicine 

  • Author: Steven J Parrillo, DO, FACOEP, FACEP; Chief Editor: Robert E O'Connor, MD, MPH   more...
 
Updated: Apr 15, 2011
 

Background

Reflex sympathetic dystrophy syndrome (RSDS) has been recognized since the Civil War when it was called causalgia, a name chosen to describe intense, burning extremity pain after an injury. Since then, RSDS has had a number of name changes. Bonica coined the term reflex sympathetic dystrophy in 1953. The American Association of Hand Surgery proposed changing the name to sympathetic maintained pain syndrome. A consensus expert panel recommended a change to complex regional pain syndrome (CRPS). However, although many clinicians still use the term RSDS, the terms currently in favor are complex regional pain syndrome I (the equivalent of RSD) and complex regional pain syndrome II, also known as causalgia.

CRPS/RSDS has readily identifiable signs and symptoms and is treatable if recognized early; however, the syndrome may become disabling if unrecognized. Emergency physicians are frequently in a position to identify the problem and may play a significant role in minimizing impact of this common entity.

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Pathophysiology

No single hypothesis explains all features of reflex sympathetic dystrophy syndrome (RSDS). Schwartzman stated that a common mechanism may be injury to central or peripheral neural tissue.[1] Roberts proposed that sympathetic pain results from tonic activity in myelinated mechanoreceptor afferents. Input causes tonic firing in neurons that are part of a nociceptive pathway. Campbell et al propose a hypothesis that places the primary abnormality in the peripheral nervous system.[2] More recent articles agree that the cause is still unknown.[3] Recent interest has focused on an immune-mediated mechanism.[4]

Most agree that complex regional pain syndrome (CRPS) is a neurologic disorder affecting central and peripheral nervous systems.[5]

Other etiologic possibilities have been suggested. German research has noted the association between elevated levels of soluble tumor necrosis factor receptor 1 (sTNF-R1) and enhanced tumor necrosis factor-alpha activity in patients with polyneuropathy with allodynia.[6] Other German researchers have described autoantibodies in patients with CRPS, especially CRPS type 2.[7]

Harvard researchers Oaklander and Fields have proposed that distal degeneration of small-diameter peripheral axons may be responsible for the pain, vasomotor instability, edema, osteopenia, and skin hypersensitivity of CRPS-1.[8]

The recent association between the use of ACE inhibitors and CRPS has caused some to consider a neuroinflammatory pathogenesis.[9]

Recent research has demonstrated cortical changes, suggesting a possible role in pathophysiology.[10]

All agree that, regardless of the mechanism, the patient experiences intense, burning pain in one or more extremities.

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Epidemiology

Frequency

United States

CRPS (formerly called reflex sympathetic dystrophy syndrome) occurs in approximately 1-15% of peripheral nerve injury cases. Schwartzman states that CRPS usually occurs secondary to fractures, sprains, and trivial soft tissue injury.[1] The incidence after fractures and contusions ranges from 10-30%. While some cases are associated with an identifiable nerve injury, many are not. Even "microtrauma" as might occur with an immunization may be responsible. The upper extremities are more likely to be involved than the lower. Entities that have led to RSDS include the following:

Mortality/Morbidity

In and of itself, the disease is not fatal. Morbidity of RSDS is associated with disease progress through a series of stages (see Physical).

Schwartzman et al recently reviewed questionnaires from 656 patients with CRPS. Once patients had experienced symptoms for more than one year, the majority of signs and symptoms were developed. No one reported spontaneous remission of symptoms.[11]

Race

The 2009 questionnaire review by Schwartzman et al showed that, in the population he studied, the overwhelming majority (96%) were white and 80% were female.[11]

Sex

Stanton-Hicks and others note that women predominate in a range of 60-80% of cases.[12]

Age

Persons of all ages are affected; however, complex regional pain syndrome (CRPS) is treated most effectively in pediatric patients.[13]

An Israeli group suggested that CRPS in children and adolescents is underdiagnosed. They emphasize the importance of early recognition and multidisciplinary treatment.[14]

Several have looked into the possibility that there may be an increased risk of CRPS development in siblings. de Rooij et al showed that, although the overallrisk is not increased, the risk in siblings younger than 50 years is significantly increased.[15, 16]

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Contributor Information and Disclosures
Author

Steven J Parrillo, DO, FACOEP, FACEP  Associate Professor, Emergency Medicine, Jefferson Medical College and Philadelphia College of Osteopathic Medicine; Medical Director, Department of Emergency Medicine, Einstein Elkins Park; Chair, Emergency Management Committee, Albert Einstein Healthcare Network; Adjunct Professor, School of Health and Science, Philadelphia University; Medical Director and Faculty, Disaster Medicine and Management Masters Program, Philadelphia University

Steven J Parrillo, DO, FACOEP, FACEP is a member of the following medical societies: American College of Emergency Physicians, American College of Osteopathic Emergency Physicians, American Osteopathic Association, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Joseph A Salomone III, MD  Associate Professor and Attending Staff, Truman Medical Centers, University of Missouri-Kansas City School of Medicine; EMS Medical Director, Kansas City, Missouri

Joseph A Salomone III, MD is a member of the following medical societies: American Academy of Emergency Medicine, National Association of EMS Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

J Stephen Huff, MD  Associate Professor of Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia School of Medicine

J Stephen Huff, MD is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Neurology, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

John D Halamka, MD, MS  Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center

John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Robert E O'Connor, MD, MPH  Professor and Chair, Department of Emergency Medicine, University of Virginia Health System

Robert E O'Connor, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American College of Physician Executives, American Heart Association, American Medical Association, Medical Society of Delaware, National Association of EMS Physicians, Society for Academic Emergency Medicine, and Wilderness Medical Society

Disclosure: Nothing to disclose.

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