Modern principles of diagnosis and treatment in complex regional pain syndrome

Background: Complex regional pain syndrome (CRPS) is a relatively common complication, occurring in 5% of cases after injury or surgery, particularly in the limbs. The incidence of CPRS is around 5-26/100 000. The latest revision of the International Statistical Classification of Diseases and Related Health Problems (ICD-11) now categorizes CRPS as a primary pain condition of multifactorial origin, rather than a disease of the skeletal system or the autonomic nervous system.Method: Based on a selective search of the literature, we summarize current principles for the diagnosis and treatment of CRPS.Results: Regional findings in CRPS are accompanied by systemic symptoms, especially by neurocognitive disorders of body perception and of symptom processing. The therapeutic focus is shifting from predominantly passive peripheral measures to early active treatments acting both centrally and peripherally. The treatment is centered on physiotherapy and occupational therapy to improve sensory perception, strength, (fine) motor skills, and sensorimotor integration/ body perception. This is supported by stepped psychological interventions to reduce anxiety and avoidance behavior, medication to decrease inflammation and pain, passive physical measures for reduction of edema and of pain, and medical aids to improve functioning in daily life. Interventional procedures should be limited to exceptional cases and only be performed in specialized centers. Spinal cord and dorsal root ganglion stimulation, respectively, are the interventions with the best evidence.Conclusion: The modern principles for the diagnosis and treatment of CRPS consider both, physiological and psychological mechanisms, with the primary goal of restoring function and participation. More research is needed to strengthen the evidence base in this field.