Complex Regional Pain Syndrome in Adults

Andreas Goebel

Disclosures

Rheumatology. 2011;50(10):1739-1750. 

In This Article

Abstract and Introduction

Abstract

Complex regional pain syndrome (CRPS) is a highly painful, limb-confined condition, which arises usually after trauma. It is associated with a particularly poor quality of life, and large health-care and societal costs. The causes of CRPS remain unknown. The condition's distinct combination of abnormalities includes limb-confined inflammation and tissue hypoxia, sympathetic dysregulation, small fibre damage, serum autoantibodies, central sensitization and cortical reorganization. These features place CRPS at a crossroads of interests of several disciplines including rheumatology, pain medicine and neurology. Significant scientific and clinical advances over the past 10 years hold promise both for an improved understanding of the causes of CRPS, and for more effective treatments. This review summarizes current concepts of our understanding of CRPS in adults. Based on the results from systematic reviews, treatment approaches are discussed within the context of these concepts. The treatment of CRPS is multidisciplinary and aims to educate about the condition, sustain or restore limb function, reduce pain and provide psychological intervention. Results from recent randomized controlled trials suggest that it is possible that some patients whose condition was considered refractory in the past can now be effectively treated, but confirmatory trials are required. The review concludes with a discussion of the need for additional research.

Introduction

Complex regional pain syndrome (CRPS) is a painful condition that develops after trauma to a limb (Fig. 1). About 10% of patients report minor trauma or cannot remember any trauma. These patients are on average 9 years younger at disease onset, but there are no differences in signs or symptoms.[1–3] CRPS is characterized by limb-confined sensory, autonomic, motor, skin and bone changes, but the lead symptom is pain. Earlier names include reflex sympathetic dystrophy (RSD), algodystrophy, algoneurodystrophy, Sudeck's atrophy and causalgia. The diagnosis is clinical; the diagnostic Budapest Criteria (Fig. 2) have recently been updated and are widely accepted.[4] These criteria, in a prior version also termed Bruehl–Harden criteria replace earlier criteria that were not specific. Over the past 10 years, scientists and clinicians have jointly achieved remarkable progress in understanding and treating CRPS. In this review both scientific and therapeutic advances are summarized.

Figure 1.

Clinical presentation of CRPS. Early CRPS of the right hand; clearly visible signs include swelling, red colour and a shiny skin. As the disease progresses some of these visible signs can partially or completely disappear while pain may persist unabated.

Figure 2.

Budapest diagnostic criteria (A–D must apply). Note that it is possible to distinguish between CRPS-1 (without damage to major nerves) and CRPS-2 [associated with (yet not causing) damage to a major nerve, a very rare presentation], but there is currently no RCT-derived evidence that this distinction has any consequence for treatment. aThe reflected understanding of allodynia as painful sensation to a number of normally non-painful stimuli is under review by the IASP taxonomy group. Some experts suggest that the term allodynia should be reserved only for brush-stroke evoked pain (dynamic mechanical allodynia). bHyperalgesia is exaggerated pain to a painful stimulus such as a pinprick. cFor example, raised systemic inflammatory markers are not associated with CRPS, even in the initial inflammatory phase; such a finding of raised markers would lead to a search for an alternative or concomitant cause. Abnormal nerve conduction studies do not exclude CRPS, but the primary cause of the observed abnormality must be clarified: CRPS, by definition is always secondary, its presence cannot explain major nerve damage. Figure adapted from Ref. [4].

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