When is CRPS, CRPS?

Complex Regional Pain Syndrome is a tricky condition. It’s a challenging pain condition for clinicians and can be a crippling pain condition for people living with it.

The condition presents as a diverse set of signs and symptoms including inflammatory, sensory/motor, autonomic abnormalities and pain. Most commonly, CRPS tends to present following an injury directly to the hands and feet. It can present as a result of a fracture or crush injury and is understood to be one of the most common ways to develop the condition (>40% of cases) (Bruehl, 2015).

However, whilst CRPS is most common in hands and feet that does not disqualify that the precipitating event will involve the hands and feet (Borchers & Gershwin, 2014).

Something that should be stressed – CRPS can occur after ANY injury. Meaning signs and symptoms can occur in the hands following a neck injury (Winston, 2016) or in the feet following spinal surgery (Wolter, Knöller, & Rommel, 2012), following a stroke or even myocardial infarction (Borchers & Gershwin, 2014). Furthermore, CRPS albeit less common has been shown to develop in the knees (Harden et al., 2003), shoulder, elbow (Goh, Chidambaram, & Ma, 2017) and even the face, head and neck (Melis et al., 2002; Sakamoto et al., 2010)

In addition, where the current distinction between CRPS type I and type II includes direct involvement of a nerve injury in type II, evidence suggests ‘even in CRPS-I, some form of initial nerve trauma is an important trigger for the cascade of events leading to CRPS’ (Bruehl, 2010). This understandably leads to more confusion about the role of neuropathic mechanisms (Bruehl, 2015).

So, it is important when clinicians are assessing and feel there are indicators of CRPS the use of diagnostic criteria and testing can support a differential diagnosis. There are a number of diagnostic criteria to support diagnosis of the condition.  This includes the IASP Criteria, the Budapest Criteria and the Veldman criteria, unfortunately there does not appear to be 1) a consensus of agreement as to which of the criteria to use and 2) a standard diagnostic test and so this can cause arbitrary diagnosis (Borchers & Gershwin, 2014). Furthermore, It is important to consider that all of the features of CRPS can occur as a result of other diseases (see image).

Whilst the 3 named criteria above are the most common there are discrepancies between each, which unfortunately, depending on the criteria that the consulting physician uses, can potentially lead to confusion around diagnosis (Borchers & Gershwin, 2014). I tend to use the Budapest criteria as it provides more comprehensive criteria than the other two I mentioned previously. In any case, it is important that a thorough discussion is had with a consulting physician about a CRPS diagnosis.

It is also important that the consulting physician performs a neurological examination and use of the CRPS Severity score can be a helpful guide to support specific testing and diagnosis of CRPS (Harden et al., 2010).

Prednisone and CRPS

I wanted to share some additional experiences around the use of a steroid called prednisone. There is an increasing evidence base around its use for CRPS, which of course can lead to an increase in preference towards it use. If, following the diagnosis of the condition, prednisone is prescribed within the first 6 months it seems this helps to stop the inflammatory process in its tracks and reduces the further development of CRPS (Atalay, Ercidogan, Akkaya, & Sahin, 2014; Rowbotham, 2006; Winston, 2016).

However, with medication comes side-effects and assumptions cannot be made that every person with CRPS will benefit from prednisone or any other medication for that matter. What may have worked for one person may not work for the next. Due to the many mechanisms involved in CRPS people can be placed on a cocktail of medications and so I stress that it is important to have a regular review of medication with a GP or better still a pain specialist Doctor.

As previously mentioned features of CRPS can occur with other conditions and so it is important that use of the diagnostic criteria and a thorough assessment is performed so to eliminate other potential reasons why someone might present with features of CRPS.  Whilst prednisone appears to have such a profound effect on the inflammatory aspect of CRPS there are several other components to the condition that could also be perpetuating the conditions state.

Whilst medicine and health professionals know quite a lot more about the mechanisms of CRPS the treatment is always challenging particularly because of the individuality (or heterogeneity) of the condition. You can read about other treatment approaches for CRPS in other blogs I have published on the CRPS 101 page of the naked physio.

Thanks for having a read

TNP

References:

Atalay, N. S., Ercidogan, O., Akkaya, N., & Sahin, F. (2014). Prednisolone in complex regional pain syndrome. Pain Physician, 17(2), 179–185. Retrieved from http://europepmc.org/abstract/MED/24658479

Borchers, A. T., & Gershwin, M. E. (2014). Complex regional pain syndrome: A comprehensive and critical review. Autoimmunity Reviews, 13(3), 242–265. https://doi.org/10.1016/j.autrev.2013.10.006

Bruehl, S. (2010). An update on the pathophysiology of complex regional pain syndrome. Anesthesiology,113(3), 713–725. https://doi.org/10.1097/ALN.0b013e3181e3db38

Bruehl, S. (2015). Complex regional pain syndrome. British Medical Journal, 350. https://doi.org/10.1136/bmj.h2730

Goh, E. L., Chidambaram, S., & Ma, D. (2017). Complex regional pain syndrome: a recent update. Burns & Trauma, 5(2), 1–11. https://doi.org/10.1093/bjaceaccp/mkt043

Harden, R. N., Bruehl, S., Perez, R. S. G. M., Birklein, F., Marinus, J., Maihofner, C., … Vatine, J. J. (2010). Development of a severity score for CRPS. Pain, 151(3), 870–876. https://doi.org/10.1016/j.pain.2010.09.031

Harden, R. N., Bruehl, S., Stanos, S., Brander, V., Chung, O. Y., Saltz, S., … Stulberg, S. D. (2003). Prospective examination of pain-related and psychological predictors of CRPS-like phenomena following total knee arthroplasty: A preliminary study. Pain. https://doi.org/10.1016/j.pain.2003.08.009

Melis, M., Zawawi, K., Lobo Lobo, S., Resident Gelb Craniomandibular, D., Mehta, N., Professor, M., & Zawawi Gelb Craniomandibular, K. (2002). Complex Regional Pain Syndrome in the Head and Neck: A Review of the Literature. J Orofac Pain, 16, 93–104.

Rowbotham, M. C. M. D. (2006). Pharmacologic Management of Complex Regional Pain Syndrome. Clinical Journal of Pain, 22(5), 425–429. https://doi.org/10.1097/01.ajp.0000194281.74379.01

Sakamoto, E., Shiiba, S., Noma, N., Okada-Ogawa, A., Shinozaki, T., Kobayashi, A., … Imamura, Y. (2010). A possible case of complex regional pain syndrome in the orofacial region. Pain Medicine, 11(2), 274–280. https://doi.org/10.1111/j.1526-4637.2009.00777.x

Winston, P. (2016). Early treatment of acute complex regional pain syndrome after fracture or injury with prednisone: Why is there a failure to treat? A case series. Pain Research and Management, 2016, 1–6. https://doi.org/10.1155/2016/7019196

Wolter, T., Knöller, S. M., & Rommel, O. (2012). Complex regional pain syndrome following spine surgery: Clinical and prognostic implications. European Neurology, 68(1), 52–58. https://doi.org/10.1159/000337907