Feb 13, 2023

Complex Regional Pain Syndrome

Updated: May 22

What is it?

The word syndrome refers to a condition that has a group of signs and symptoms that co-occur. In CRPS these may include(1):

Pain
Changes in skin color
Swelling in the area
Changes in temperature in the area
Changes in hair growth, nail and skin texture
Decreased function (strength, mobility, others)

Which mainly effects the hand and feet but can occur or spread to different area(2). It is divided into two subtypes; CRPS type-1 where no nerve lesion is identified vs CRPS type-2 where a nerve lesion is identifiable(2,3). However, knowing the exact type does not seem to be important in outcomes, treatment options, duration and can be difficult/timely/expensive to test for.

What can cause CRPS?

The cause for the development of CRPS is not completely understood(4,5,9). It can occur following some form of trauma (fracture, sprain, strain, surgery) in 93% of cases(5). 7% of cases have no clear cause(5). There is some evidence to suggest that immobilization (e.g., use of cast/splint) can increase the risk of CRPS development (10).

CRPS seems to be have involvement from several phenomenon including;

Inflammation(2,5,6,9). Several inflammatory markers have been found to be high in CRPS, this could assist in explaining sensitisation, hair growth, sweating and skin temperature(5,6). Inflammation seems to be most predominant in the first 12 months(6).
Central changes(2,6,9). These tend to occur in persistent CRPS lasting >3 months. Changes in the structure and function of the brain and spinal cord start to happen(6). This phenomenon is named ‘plasticity’, it is important to note that these are not permanent changes and these can be reversed.
Sympathetic nervous system(2,6,9). It is thought the SNS plays a role in CRPS however, the exact mechanism/contribution is not well understood(5).

There seems to be a relationship with psychological factors/conditions and the development of CRPS(3,5,6,9). Anxiety, pain-related fear/disability are related to poor outcomes at 12 months(6). Post-traumatic stress symptoms are reported in 38% of individuals with CRPS(6).

CRPS is 2-4 times more common in females then males(5). It also occurs more frequently in people with Fibromyalgia(5).

Genetics is also believed to play role in CRPS(5,9).

Signs/symptoms which may indicate CRPS?

There have been two subtypes described; warm and cold. The ‘primarily warm’ subtype seems to precede the ‘primarily cold’ subtype(6).

The warm subtype includes reddish skin discoloration, swelling, dryness, pain and warmth and is thought to involve inflammation(3,5). While the cold subtype includes blueish skin discoloration, pain, sweating(3.6). The hallmark feature of CRPS is pain which occurs in all cases(6). Swelling and a loss of strength are very common(6). While some cases (not all) will experience the following signs and symptoms:

Changes to nails
Development of ulcers
Increase or decrease in hair growth
Changes in sweating

How do I know I have it?

Complex Regional Pain Syndrome is diagnosed by a health professional using the Budapest criteria(5,6,9). It is important to note that this criteria is not perfect and continues to be an area of debate(3). In the future these criteria may be updated or changed.

From Birklein & Dimova (2017)(6)

What are the available treatments?

As CRPS and any other pain condition involve multiple different factors, it ideally requires multidisciplinary treatment to address different factors(6). The combination of these treatments (+/- medication) seems to be most effective(3). Physical treatments aim to improve function, pain and other symptoms experienced in CRPS. There is no standardized treatment for the condition and it should be individual based. Movement is essential throughout the treatment process. A lack of movement during the inflammatory phase can result in contractures (fixed tightening of tendon/muscle)(6). Interventions may include the following:

Graded motor imagery (including mirror therapy)(1,2,3,5,6,7)
Hydrotherapy(4)
Aerobic exercise(1,3,4)
Resistance and mobility exercises(3,4)
Desensitisation(3,4)
Graded exposure or pain exposure therapy(1,3,4,6)
Biofeedback(4)
Relaxation/breathing training(4)
Virtual reality
Edema (swelling) control(3)

Psychological interventions aim to assist in the ability to cope, manage and live with pain(3,4). If a person is fearful of movements as they may believe they have negative consequences, Cognitive-Behaviour Therapy can be helpful(8). CRPS can often co-occur with decreased mood, anxiety and/or sleep disturbances, psychological interventions can again assist(3). Medical procedures may also assist, especially if the above have not been effective or if it is too painful to participate in physical interventions(3). These may include nerve blocks, medication or spinal cord stimulation(2,4,5,6). Sympathetic nerve blocks are commonly used and seem to be effective for 71% of CRPS cases for the first 4 weeks(5). After this period only 14% report ongoing positive effects(5). It is important to remember that the effectiveness (and side-effects) for medications varies person to person. Medications that may be explored include;

Glucocorticoid (Prednisolone) in the inflammatory stage(6).
Bisphosphonates(2,3,5,6) - in the early phases (9)
Calcitonin (Miacalcin)(2,3)
Pregabalin
Gabapentin (Neurontin)(2,3,5,6)
Amitriptyline(3) - especially if sleep and/or psychological factors are an issue.
Duloxetine or other SNRIs (9)

Other interventions that have been studied but have shown either unclear or no effect on pain or disability are:

Acupuncture(1)
Ultrasound therapy(1)
Manual lymphatic drainage(1)
Laser therapy(1)
TENS(1)
Medical Cannabis(5)
Ketamine infusions (9,11,12)

From Ferraro et al., (2024)(9)

Additional tips

It is important to return to normal activities as soon as possible this includes social activities, work and other meaningful activities(3). A rehabilitation/vocational consultant are able to assist in this program creating a return to work programme(3).

Avoid long periods of bed rest or inactivity, even when pain is intense, this can be detrimental to your recovery(3).

Will it get better?

It certainly can, although recovery times can differ significantly between individuals. For the ‘warm’ CRPS subtype the average time is 4.5 months while for the ‘cold’ subtype it is 20 months.

How can an exercise physiologist or physiotherapist help?

Both an exercise physiologist and physiotherapist can assist in providing physical interventions (described above). Their main goal is to improve physical function, manage or improve pain, normalize other symptoms (e.g., swelling, sensitisation) and work towards physical goals relevant to the individual.

Why Adapt Movement?

At Adapt Movement we emphasize on personalized care and rehabilitation based on the most current treatments available. We look at the person as a whole and don’t just treat the diagnosis🧍. Human beings are not like machines, and we cannot “fix” parts, instead we assist in creating the optimal environment for letting the body heal itself. Our treatment is not done “on you” but with you. This means that we acknowledge that we don’t have magic hands/tools to fix you and that real healing/recovery needs to come from a collaboration between the patient and practitioner.

We provided in person services to Geraldton and the Mid-West region including home-visits. We provide telehealth services for anyone unable to visit our clinic or outside the Mid-West region.

References

Shafiee, E., MacDermid, J., Packham, T., Walton, D., Grewal, R., & Farzad, M. (2023). The Effectiveness of Rehabilitation Interventions on Pain and Disability for Complex Regional Pain Syndrome. The Clinical Journal of Pain, 39(2), 91–105. https://doi.org/10.1097/ajp.0000000000001089
O’Connell, N. E., Wand, B. M., McAuley, J., Marston, L., & Moseley, G. L. (2013). Interventions for treating pain and disability in adults with complex regional pain syndrome. The Cochrane Database of Systematic Reviews, 2013(4), CD009416. https://doi.org/10.1002/14651858.CD009416.pub2
Harden, R. N., McCabe, C. S., Goebel, A., Massey, M., Suvar, T., Grieve, S., & Bruehl, S. (2022). Complex Regional Pain Syndrome: Practical Diagnostic and Treatment Guidelines, 5th Edition. Pain Medicine, 23(Supplement_1), S1–S53. https://doi.org/10.1093/pm/pnac046
Vescio, A., Testa, G., Culmone, A., Sapienza, M., Valenti, F., Di Maria, F., & Pavone, V. (2020). Treatment of Complex Regional Pain Syndrome in Children and Adolescents: A Structured Literature Scoping Review. Children, 7(11), 245. https://doi.org/10.3390/children7110245
Taylor, S.-S., Noor, N., Urits, I., Paladini, A., Sadhu, M. S., Gibb, C., Carlson, T., Myrcik, D., Varrassi, G., & Viswanath, O. (2021). Complex Regional Pain Syndrome: A Comprehensive Review. Pain and Therapy, 10(2), 875–892. https://doi.org/10.1007/s40122-021-00279-4
Birklein, F., & Dimova, V. (2017). Complex regional pain syndrome–up-to-date. PAIN Reports, 2(6), e624. https://doi.org/10.1097/pr9.0000000000000624
Shafiee, E., MacDermid, J., Packham, T., Grewal, R., Farzad, M., Bobos, P., & Walton, D. (2023). Rehabilitation Interventions for Complex Regional Pain Syndrome: An Overview of Systematic Reviews. The Clinical journal of pain, 39(9), 473–483. https://doi.org/10.1097/AJP.0000000000001133
Norman Harden, R., Swan, M., King, A., Costa, B., & Barthel, J. (2006). Treatment of Complex Regional Pain Syndrome. The Clinical Journal of Pain, 22(5), 420–424. https://doi.org/10.1097/01.ajp.0000194280.74379.48
Ferraro, M. C., O'Connell, N. E., Sommer, C., Goebel, A., Bultitude, J. H., Cashin, A. G., Moseley, G. L., & McAuley, J. H. (2024). Complex regional pain syndrome: advances in epidemiology, pathophysiology, diagnosis, and treatment. The Lancet. Neurology, 23(5), 522–533. https://doi.org/10.1016/S1474-4422(24)00076-0
Pons, T., Shipton, E. A., Williman, J., & Mulder, R. T. (2015). Potential risk factors for the onset of complex regional pain syndrome type 1: a systematic literature review. Anesthesiology research and practice, 2015, 956539. https://doi.org/10.1155/2015/956539
Xu, J., Herndon, C., Anderson, S., Getson, P., Foorsov, V., Harbut, R. E., Moskovitz, P., & Harden, R. N. (2019). Intravenous Ketamine Infusion for Complex Regional Pain Syndrome: Survey, Consensus, and a Reference Protocol. Pain medicine (Malden, Mass.), 20(2), 323–334. https://doi.org/10.1093/pm/pny024
Orhurhu, V., Orhurhu, M. S., Bhatia, A., & Cohen, S. P. (2019). Ketamine Infusions for Chronic Pain: A Systematic Review and Meta-analysis of Randomized Controlled Trials. Anesthesia and analgesia, 129(1), 241–254. https://doi.org/10.1213/ANE.0000000000004185

This blog was written by Samuel Bulten