Reflex Sympathetic Dystrophy 

  • Author: Don R Revis Jr, MD; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Jan 19, 2012
 

Background

Reflex sympathetic dystrophy (RSD) is a clinical syndrome of variable course and unknown cause characterized by pain, swelling, and vasomotor dysfunction of an extremity. This condition is often the result of trauma or surgery. In 1864, Mitchell referred to this malady as causalgia, a Greek word meaning burning pain. Newer taxonomy refers to RSD as a type of complex regional pain syndrome (CRPS), which may develop after an initiating event such as trauma or surgery or may occur spontaneously.[1] Under this classification, causalgia is a type of CRPS that develops after nerve injury. In patients with either of these conditions, sympathetic mediation of the pain (ie, improvement with sympathetic blockade) may or may not be evident.

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Pathophysiology

The pathogenesis of RSD is unknown. Three conditions are deemed important in the development of RSD, including a persistent painful lesion, a predisposition or susceptibility to developing RSD, and an abnormal sympathetic reflex. Susceptibility factors are unknown and may include genetic predisposition (HLA typing)[2, 3, 4] and, in some patients, a tendency toward increased sympathetic activity. This includes cold hands, hyperhidrosis, or a history of fainting.

Healthy individuals undergo a sympathetic response to injury, with vasoconstriction designed to prevent blood loss and swelling. This initial response soon subsides and gives way to vasodilatation and increased capillary permeability, allowing tissue repair.

In patients with RSD, this sympathetic response continues unabated. The reasons for the perpetuation of the response are unknown but may be related to central dysregulation of nociceptive impulses. This dysregulation may be mediated by wide dynamic range neurons in the spinal cord. Prolonged ischemia caused by the vasoconstriction produces more pain, establishing a reflex arc that promotes further sympathetic discharge and vasospasm. This is compounded by the local response to trauma, with liberation of substantial amounts of proinflammatory mediators, such as histamine, serotonin, and bradykinin. The result is a swollen, painful, stiff, nonfunctioning extremity. At least partial sympathetic mediation of this phenomenon is likely because of the ability of sympathetic nerve blockade to relieve pain and other features of RSD in some patients.

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Epidemiology

Frequency

United States

An estimated 5% of patients who experience trauma to the upper extremity develop RSD, although this figure is not known with certainty because of confusion over the diagnosis. Extremity immobilization can trigger RSD. Without prophylactic measures (active physical therapy), RSD can develop in 12-20% of people who experience a hemiplegic stroke.

Mortality/Morbidity

RSD causes essentially no mortality.

Race

No racial predilection exists.

Sex

Sexual distribution is equal.

Age

  • The age of onset in most patients with RSD is 30-60 years, and the mean age is 49 years.[5]
  • RSD affects children and carries a much better prognosis than in adults.[6, 7]
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Contributor Information and Disclosures
Author

Don R Revis Jr, MD  Consulting Staff, Department of Surgery, Division of Plastic and Reconstructive Surgery, University of Florida College of Medicine

Don R Revis Jr, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, American Society for Aesthetic Plastic Surgery, and American Society of Plastic Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Elliot Goldberg, MD  Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Robert E Wolf, MD, PhD  Professor Emeritus, Department of Medicine, Louisiana State University School of Medicine in Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Affairs Medical Center

Robert E Wolf, MD, PhD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation, and Society for Leukocyte Biology

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

Lawrence H Brent, MD  Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting; Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

References

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  2. Kemler MA, van de Vusse AC, van den Berg-Loonen EM, et al. HLA-DQ1 associated with reflex sympathetic dystrophy. Neurology. Oct 12 1999;53(6):1350-1. [Medline].

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  5. Goebel A. Complex regional pain syndrome in adults. Rheumatology (Oxford). Oct 2011;50(10):1739-50. [Medline].

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  8. van Hilten BJ, van de Beek WJ, Hoff JI, et al. Intrathecal baclofen for the treatment of dystonia in patients with reflex sympathetic dystrophy. N Engl J Med. Aug 31 2000;343(9):625-30. [Medline].

  9. Gobelet C, Waldburger M, Meier JL. The effect of adding calcitonin to physical treatment on reflex sympathetic dystrophy. Pain. Feb 1992;48(2):171-5. [Medline].

  10. Kemler MA, Barendse GA, Van Kleef M, et al. Electrical spinal cord stimulation in reflex sympathetic dystrophy: retrospective analysis of 23 patients. J Neurosurg. Jan 1999;90(1 Suppl):79-83. [Medline].

  11. Kemler MA, Barendse GA, van Kleef M, et al. Spinal cord stimulation in patients with chronic reflex sympathetic dystrophy. N Engl J Med. Aug 31 2000;343(9):618-24. [Medline].

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  17. Pandita D, Danielson BD, Potti A, et al. Complex regional pain syndrome type-1: a rare complication of arteriovenous graft placement. J Rheumatol. Oct 1999;26(10):2254-6. [Medline].

  18. Poncelet C, Perdu M, Levy-Weil F, et al. Reflex sympathetic dystrophy in pregnancy: nine cases and a review of the literature. Eur J Obstet Gynecol Reprod Biol. Sep 1999;86(1):55-63. [Medline].

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  22. Severens JL, Oerlemans HM, Weegels AJ, et al. Cost-effectiveness analysis of adjuvant physical or occupational therapy for patients with reflex sympathetic dystrophy. Arch Phys Med Rehabil. Sep 1999;80(9):1038-43. [Medline].

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