CRPS: what’s in a name? Taxonomy, epidemiology, neurologic, immune and autoimmune considerations

Abstract

This account of the condition now termed complex regional pain syndrome (CRPS) spans approximately 462 years since a description embodying similar clinical features was described by Ambroise Paré in 1557. While reviewing its historical origins, the text describes why it became necessary to change the taxonomies of two clinical syndromes with similar pathophysiologies to one which acknowledges this aspect but does not introduce any mechanistic overtones. Discussed at length is the role of the sympathetic component of the autonomic nervous system (ANS) and why its dysfunction has both directly and indirectly influenced our understanding of the inflammatory aspects of CRPS. As the following article will show, our knowledge has expanded in an exponential fashion to include musculoskeletal, immune, autoimmune, central and peripheral nervous system and ANS dysfunction, all of which increase the complexity of its clinical management. A burgeoning literature is beginning to shed light on the mechanistic aspects of these syndromes and the increasing evidence of a genetic influence on such factors as autoimmunity, and its importance is also discussed at length. An important aspect that has been missing from the diagnostic criteria is a measure of disease severity. The recent validation of a CRPS Severity Score is also included.

Complex regional pain syndrome and bone marrow oedema syndrome: family ties potentially closer than expected

“…This is perfectly illustrated here, the patient having the two entities developed at a few weeks’ interval only. Second, and contrary to the prevailing opinion, 6 this case report also suggests that imaging remains an important part of the diagnosis and management of CRPS. Deep-bone damage revealed by radiology, as already shown by Paul Sudeck a century ago 17 18 is evident here.…”

“…4 Over the years, the CRPS diagnostic criteria have evolved towards purely clinical criteria (the so-called Budapest criteria) 5 and any reference to deep bone involvement with osteopenia revealed by radiology, as already shown by Paul Sudeck a century ago, has been removed. 6 On the other hand, the diagnosis of BMES may be suspected clinically (pain and progressive functional impairment) but requires imaging confirmation (MRI). 7 Although generally considered to be separate entities, links are suspected between them, particularly because of similar bone involvements also often documented in the early phase of CRPS.…”

Utilization of Concurrent Dorsal Root Ganglion Stimulation and Dorsal Column Spinal Cord Stimulation in Complex Regional Pain Syndrome

“…Complex regional pain syndrome (CRPS) is a painful, debilitating, and heterogeneous condition that is often challenging to treat effectively (1, 2). The pathophysiology of CRPS involves a complex network of mechanisms including peripheral sensitization, central sensitization, cortical reorganization, and inflammation, making it difficult to treat the underlying etiology (1, 2).…”

“…The treatment algorithm for pharmacologic and interventional therapies is generally also considered in a step‐wise fashion. Neurostimulation is also included in the treatment algorithms of CRPS (2).…”

Changes in cardiovascular parameters in rats exposed to chronic widespread mechanical allodynia induced by hind limb cast immobilization

“…Chronic pain is often accompanied by autonomic disorders, as well as spontaneous pain, hyperalgesia, and allodynia [1][2][3][4][5]. Complex regional pain syndrome (CRPS) is characterized by changes in skin temperature and skin blood flow in the affected area along with chronic widespread pain.…”