Lunden Lars Kristian, Jorum Ellen
Section of Clinical Neurophysiology, Department of Neurology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway.
Scand J Pain. 2021 Apr 12;21(3):548-559. doi: 10.1515/sjpain-2021-0036. Print 2021 Jul 27.
Complex regional pain syndrome (CRPS) is a disabling usually post-traumatic pain condition. International guidelines emphasize early diagnosis for treatment and improved outcome. Early intense and persistent pain along with features of autonomic dysfunction in the first week's post-injury are early warning signs for development of CRPS. We have previously reported a delayed diagnosis of CRPS. The main purpose of the present study was to investigate possible causes of a delayed diagnosis, with a special focus of recognition of risk factors.
A total of 52 CRPS 1 (without detectable nerve damage) and CRPS 2 (with evidence of nerve lesion) patients were included in the study. When examined at OUS-Rikshospitalet, we retrospectively asked the patients on the development of pain and autonomic abnormalities from the time of the eliciting injury, performed a thorough clinical investigation with an emphasis on signs of autonomic failure and compared symptoms and clinical findings with such information in previous medical records. We also evaluated symptoms and signs according to the type of injury they had suffered.
Of a total of 52 patients (30 women and 22 men, mean age 39.0 years at the time of injury), 34 patients had CRPS type 1 (65.4%) and 18 CRPS type 2 (34.6%), 25 patients with pain in the upper and 27 in the lower extremity. A total of 35 patients (67.3%) were diagnosed with CRPS (following mean 2.1 years) prior to the investigation at OUS-Rikshospitalet (mean 4.86 years following injury). Mean time from injury to diagnosis was 33.5 months (SD 30.6) (2.8 years) for all patients. In retrospect, all 17 patients first diagnosed at OUS met the CRPS diagnosis at an earlier stage. All patients retrospectively reported intense pain (numeric rating scale > 7) from the time of injury with a large discrepancy to previous medical records which only stated intense pain in 29.4% of patients with CRPS type 1 and 44.4% of patients with CRPS type 2 within the first four months. While the patients reported an early onset of autonomic dysfunction, present in 67.3 and 94.2% of the patients within one week and one month, respectively, reports of autonomic abnormalities within the first four months was far less (maximum in 51.7% of patients with CRPS type 1 and in 60% in CRPS 2). In 10 patients with CRPS type 1, no symptom nor sign of autonomic abnormalities was reported.
We still find a significant delay in the diagnosis of CRPS. There is a large discrepancy between both self-reporting of intense, disproportionate pain, as well as symptoms of autonomic abnormalities from the time of injury, and documentation in previous medical records. Our findings suggest a lack of awareness of risk factors for the development of CRPS, such as early intense pain and autonomic abnormalities without recovery, contributing to delayed diagnosis. The present results suggest causes of delayed CRPS-diagnosis. An increased attention to early warning signs/risk factors may improve diagnosis of CRPS.
复杂性区域疼痛综合征（CRPS）是一种通常由创伤引起的致残性疼痛病症。国际指南强调早期诊断以进行治疗并改善预后。受伤后第一周出现的早期强烈且持续的疼痛以及自主神经功能障碍的特征是CRPS发展的早期预警信号。我们之前曾报告过CRPS的延迟诊断情况。本研究的主要目的是调查延迟诊断的可能原因，特别关注危险因素的识别。
本研究共纳入52例CRPS 1型（无可检测到的神经损伤）和CRPS 2型（有神经损伤证据）患者。在奥斯陆大学医院进行检查时，我们回顾性地询问患者从引发损伤之时起疼痛和自主神经异常的发展情况，进行了全面的临床检查，重点关注自主神经功能衰竭的体征，并将症状和临床发现与之前病历中的此类信息进行比较。我们还根据患者所遭受的损伤类型评估症状和体征。
在总共52例患者（30名女性和22名男性，受伤时平均年龄39.0岁）中，34例为CRPS 1型（65.4%），18例为CRPS 2型（34.6%），25例上肢疼痛，27例下肢疼痛。在奥斯陆大学医院进行调查之前（受伤后平均4.86年），共有35例患者（67.3%）被诊断为CRPS（平均2.1年之后）。所有患者从受伤到诊断的平均时间为33.5个月（标准差30.6）（2.8年）。回顾来看，所有17例首次在奥斯陆大学医院被诊断出的患者在更早阶段就符合CRPS诊断标准。所有患者回顾性报告从受伤之时起就有强烈疼痛（数字评分量表>7），这与之前病历记录有很大差异，之前病历记录中仅显示CRPS 1型患者中有29.4%以及CRPS 2型患者中有44.4%在最初四个月内有强烈疼痛。虽然患者报告自主神经功能障碍出现较早，分别在一周内和一个月内出现在67.3%和94.2%的患者中，但在前四个月内关于自主神经异常的报告要少得多（CRPS 1型患者中最多为51.7%，CRPS 2型患者中为60%）。在10例CRPS 1型患者中，未报告自主神经异常的症状和体征。
我们仍然发现CRPS的诊断存在显著延迟。从受伤之时起，强烈、不成比例的疼痛以及自主神经异常症状的自我报告与之前病历记录之间存在很大差异。我们的研究结果表明，对CRPS发展的危险因素缺乏认识，如早期强烈疼痛和自主神经异常且未恢复，导致了延迟诊断。目前的结果提示了CRPS延迟诊断的原因。对早期预警信号/危险因素的更多关注可能会改善CRPS的诊断。
