Startseite Complex Regional Pain Syndrome (CRPS): High risk of CRPS after trauma in another limb in patients who already have CRPS in one hand or foot: Lasting changes in neural pain modulating systems?
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Complex Regional Pain Syndrome (CRPS): High risk of CRPS after trauma in another limb in patients who already have CRPS in one hand or foot: Lasting changes in neural pain modulating systems?

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Veröffentlicht/Copyright: 1. Januar 2017
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In this issue of the Scandinavian Journal of Pain Ellen Satteson and her co-workers at Wake Forest School of Medicine, NC, USA, publish their estimates of risk of having a second CRPS developing in another extremity after trauma in patients who already have or have had CRPS in a hand or foot [1]. In almost 100 patients with a CRPS diagnosis they found that 20% developed CRPS after trauma in another foot or hand. This is very much higher than what is reported in the general adult population (about 0.023%). The risk of developing CRPS after a distal radius fracture is well known to be much higher than after all other kinds of injuries [2], from the literature it is estimated to be between 1% and 30% (mean about 6%) [2]. Thus it is fair to conclude that for some reason there is a clinically significant risk of developing a second case of CRPS if you already have, or have had, CRPS in one other extremity [1].

1 Why should there be risk of developing a second CRPS in patients who already have this diagnosis?

Fortunately, CRPS or CRPS-like pain conditions that occur in about 5–10% after trauma or surgery of a limb, in at least ¾ of them, the pain and other symptoms gradually diminish and disappear [2]. However, an unfortunate minority develop a chronic form of CRPS [2,3]. The cause and the pathogenesis of acute-to-chronic CRPS is still not well understood. What seems certain is that there is an ongoing abnormal inflammatory process that sensitizes peripheral and spinal cord nociceptor systems. Somehow the pain modulatory mechanisms in the spinal cord dorsal horn and higher up in the CNS malfunction. Neuropeptides released from nociceptors aggravate inflammation; vascular endothelial cells are affected, leading to vascular changes – dilatation and a warm, red, and hypersensitive limb with “warm CRPS”, later on vasoconstriction and a cold, pale, and sweaty limb with “cold CRPS”. Autoantibodies may develop in this neuroinflammatory process, binding to adrenoceptors and acetylcholine-receptors, leading to autonomic nervous system malfunctioning. Some chronic CRPS patients develop “neglect” phenomena and gross non-use of the affected limb [3].

With such widespread and profound changes in peripheral and central pain sensing and pain-modulating systems, it is not difficult to understand that patients who have or have had a typical chronic CRPS, will have sensitized and easily reawakened malfunctioning pain modulating systems. Therefore it is not surprising that Satteson and co-workers report that patients with a history of CRPS has a high risk of developing a second CRPS after trauma in another limb [1]. Their observation is related to what others have termed “spreading of CRPS”, possibly related to circulating autoantibodies to adrenergic receptors and muscarinic acetylcholine-receptors in autonomic structures [4].

Diagnosis of CRPS should be according to the IASP-Budapest criteria. Although well known by pain specialists, I believe it is useful to remind the readers of the fairly strict IASP-criteria which should differentiate a CRPS-pain condition from a chronic neuropathic pain condition [1,2,3]:

IASP-approved CRPS diagnostic criteria

  1. Continuing pain, which is disproportionate to any inciting event

  2. Must report at least 1 symptom in 3 of the 4 following categories:

    Sensory: reports of hyperesthesia and/or allodynia

    Vasomotor: reports of temperature asymmetry and/or skin colour changes and/or skin colour asymmetry

    Sudomotor/oedema: reports of oedema and/or sweating changes and/or sweating asymmetry

    Motor/trophic: reports of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)

  3. Must display at least 1 sign at the time of evaluation in 2 (for clinical practice) or 3 or more (for scientific research) of the following categories:

    Sensory: evidence of hyperalgesia (to pinprick) and/or allodynia (to light touch and/or deep somatic pressure and/or joint movement)

    Vasomotor: evidence of temperature asymmetry and/or skin colour changes and/or asymmetry

    Sudomotor/oedema: evidence of oedema and/or sweating changes and/or sweating asymmetry

    Motor/trophic: evidence of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)

  4. There is no other diagnosis that better explains the signs and symptoms

2 Recurrence or a second CRPS in children apparently cured of a CRPS-pain condition

Chronic CRPS in children appeared to have a better prognosis and more often seemed to be completely cured by conservative, psychosocial and functionally focused treatment than in adults with chronic CRPS [5]. Unfortunately, this is an overstated optimistic attitude [2]. The pathogenic mechanisms most likely are similar in childhood CRPS as in CRPS in adult patients [2,3], and it would be almost naïve to expect that there would not be any traces of such major neuropathological processes in the pain-modulating nervous systems in children apparently cured from CRPS. Thus, more than 50% of adults with a history of childhood-onset CRPS still experienced pain 12 years after childhood CRPS and more than a third had recurrences of CRPS later in life [6]. It now appears that there is a definite recurrence rate in successfully treated children with CRPS treated by a Norwegian multidisciplinary pain management team (Anne Gina Schie Berntsen – personal communication). Thus, there may still be a need for invasive techniques in a multidisciplinary approach to non-responders to conservative psychosocial care [7].


DOI of refers to article: http://dx.doi.org/10.1016/j.sjpain.2016.10.005.



Department of Pain Management and Research, Oslo University Hospital, Pox 4950 Nydalen, 0424 Oslo, Norway.

  1. Conflict of interest: None declared.

References

[1] Satteson ES, Jarbpur PW, Koman LA, Smith BP, Li Z. The risk of pain síndrome affecting a previously non-painful limb following trauma or surgery in patients with a history of complex regional pain síndrome. Scand J Pain 2017; 14:84–8.Suche in Google Scholar

[2] Bruehl S. Complex regional pain syndrome. BMJ 2015;350:h2730.Suche in Google Scholar

[3] Birklein F, Schlereth T. Complex regional pain syndrome–significant progress in understanding. Pain 2015;156:S–S94.Suche in Google Scholar

[4] Kohr D, Singh P, Tschernatsch M, Kaps M, Pouokam E, Diener M, Kummer W, Birklein F, Vincent A, Goebel A, Wallukat G, Blaes F. Autoimmunity against the β2 adrenergic receptor and muscarinic-2 receptor in complex regional pain syndrome. Pain 2011;152:2690–700.Suche in Google Scholar

[5] Stanton-Hicks M. Plasticity of complex regional pain syndrome (CRPS) in children. Pain Med 2010;11:1216–23.Suche in Google Scholar

[6] Tan EC, van de Sandt-Renkema N, Krabbe PF, Aronson DC, Severijnen RS. Quality of life in adults with childhood-onset of complex regional pain syndrome type I. Injury 2009;40:901–4.Suche in Google Scholar

[7] Rodriguez-Lopez MJ, Fernandez-Baena M, Barroso A, Yánez-Santos JA. Complex regional Pain síndrome in children: a multidisciplinary approach and invasive techniques for the management of nonresponders. Pain Pract 2015;15:E81–9.Suche in Google Scholar

Published Online: 2017-01-01
Published in Print: 2017-01-01

© 2016 Scandinavian Association for the Study of Pain

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