Chang Christopher, McDonnell Patrick, Gershwin M Eric
Division of Rheumatology, Allergy and Clinical Immunology, University of California, Davis, 451 Health Sciences Drive, Suite 6510, Davis, CA, 95616, USA.
Division of Immunology, Allergy and Rheumatology, Joe DiMaggio Children's Hospital and Memorial Healthcare System, 1131 N 35th Avenue, Suite 220, Hollywood, FL, 33021, USA.
J Transl Autoimmun. 2020 Dec 24;4:100080. doi: 10.1016/j.jtauto.2020.100080. eCollection 2021.
Complex regional pain syndrome (CRPS) purports to explain extremity pain accompanied by a variety of subjective complaints, including sensitivity to touch, fatigue, burning sensations, allodynia and signs consistent with voluntary immobilization, including skin changes, edema and trophic changes. By its own definition, CRPS pain is disproportionate to any inciting event or underlying pathology, which means that the syndrome describes non-anatomic and exaggerated symptoms. Although CRPS was coined in the early 1990s, physicians have described unexplained exaggerated pain for centuries. Before a small group of researchers assigned this historical phenomenon with the name CRPS, other physicians in various subspecialties investigated the existence of a common pathophysiologic mechanism but found none. The literature was searched for evidence of a reproducible pathologic mechanism for CRPS. Although some have suggested that CRPS is an autoimmune disease, there is a paucity of evidence to support this. While cytokines such as IL-1β, IL-6 and TNF-α have been detected during the early phases of CRPS, this cannot lead to the conclusion that CRPS is an autoimmune disease, nor that it is an autoinflammatory disorder. Moreover, intravenous immunoglobulin has showed inconsistent results in the treatment of CRPS. On the other hand, CRPS has been found to meet at least three out of four criteria of malingering, which was previously a DSM-IV diagnosis; and its diagnostic criteria are virtually identical to current DSM-5 Functional Neurological Disorder ("FND"), and proposed ICD-11 classification, which includes FND as a distinct neurological diagnosis apart from any psychiatric condition. Unfortunately, the creation of CPRS is not merely misguided brand marketing. It has serious social and health issues. At least in part, the existence of CRPS has led to the labeling of many patients with a diagnosis that allows the inappropriate use of invasive surgery, addictive opioids, and ketamine. The CRPS hypothesis also ignores the nature and purpose of pain, as a symptom of some organic or psychological process. Physicians have long encountered patients who voice symptoms that cannot be biologically explained. Terminology historically used to describe this phenomenon have been medically unexplained symptoms ("MUS"), hysterical, somatic, non-organic, psychogenic, conversion disorder, or dissociative symptoms. The more recent trend describes disorders where there is a functional, rather than structural cause of the symptoms, as "functional disorders." Physicians report high success treating functional neurological symptoms with reassurance, physiotherapy, and cognitive behavior therapy measured in terms of functional improvement. The CRPS label, however, neither leads to functional improvement in these patients nor resolution of symptoms. Under principles of evidence-based medicine, the CRPS label should be abandoned and the syndrome should simply be considered a subset of FNDs, specifically Functional Pain Disorder; and treated appropriately.
复杂性区域疼痛综合征(CRPS)旨在解释伴有多种主观症状的肢体疼痛,这些症状包括对触摸敏感、疲劳、烧灼感、痛觉过敏以及与自主制动相符的体征,如皮肤变化、水肿和营养改变。根据其自身定义,CRPS疼痛与任何诱发事件或潜在病理状况不成比例,这意味着该综合征描述的是非解剖学的且夸张的症状。尽管CRPS这一术语在20世纪90年代初才被创造出来,但数世纪以来医生们一直在描述无法解释的夸张疼痛。在一小群研究人员给这一历史现象命名为CRPS之前,各个亚专业的其他医生曾探究是否存在共同的病理生理机制,但未找到。检索文献以寻找CRPS可重复病理机制的证据。尽管有人认为CRPS是一种自身免疫性疾病,但支持这一观点的证据很少。虽然在CRPS早期阶段检测到了诸如IL-1β、IL-6和TNF-α等细胞因子,但这既不能得出CRPS是自身免疫性疾病的结论,也不能得出它是一种自身炎症性疾病的结论。此外,静脉注射免疫球蛋白在CRPS治疗中的效果并不一致。另一方面,已发现CRPS至少符合伪装的四项标准中的三项,伪装曾是《精神疾病诊断与统计手册》第四版(DSM-IV)的诊断;其诊断标准实际上与当前的《精神疾病诊断与统计手册》第五版(DSM-5)功能性神经障碍(“FND”)以及提议的国际疾病分类第11版(ICD-11)分类相同,后者将FND作为一种独立于任何精神疾病状况的神经学诊断。不幸的是,CRPS的创立不仅仅是误导性的品牌营销。它存在严重的社会和健康问题。至少在一定程度上,CRPS的存在导致许多患者被贴上该诊断标签,从而允许不恰当地使用侵入性手术、成瘾性阿片类药物和氯胺酮。CRPS假说也忽视了疼痛作为某些有机或心理过程症状的本质和目的。医生们长期以来遇到过诉说无法从生物学角度解释症状的患者。历史上用于描述这一现象的术语有医学上无法解释的症状(“MUS”)、癔症性、躯体性、非器质性、心因性、转换障碍或分离性症状。最近的趋势是将症状由功能性而非结构性原因导致的疾病描述为“功能性障碍”。医生报告称,通过安慰、物理治疗和认知行为疗法治疗功能性神经症状,在功能改善方面取得了很高的成功率。然而,CRPS这一标签既不能使这些患者功能得到改善,也不能使症状得到缓解。根据循证医学原则,应摒弃CRPS这一标签,该综合征应简单地被视为FND的一个子集,特别是功能性疼痛障碍,并进行适当治疗。