Adler Brittany L, Kuchinad Kamini, Jeong Seeun Judy, Stiles Lauren E, Shah Ami A, Chung Tae, McMahan Zsuzsanna H
Division of Rheumatology, Johns Hopkins University, Baltimore, MD, USA.
Department of Physical Medicine and Rehabilitation, Johns Hopkins University, Baltimore, MD, USA.
Rheumatology (Oxford). 2025 May 1;64(5):3080-3084. doi: 10.1093/rheumatology/keae516.
Patients with autonomic dysfunction, or dysautonomia, often report discolouration of their dependent extremities, which is thought to be from venous pooling or acrocyanosis. A subset of patients with SSc are affected by dysautonomia but may be challenging to identify. We sought to determine whether patients with SSc who report discolouration in their feet have a higher burden of autonomic symptoms, including orthostatic, gastrointestinal (GI), urinary, secretomotor and pupillomotor.
167 patients with SSc completed the Composite Autonomic Symptom Score (COMPASS)-31 survey, which queries whether the patient experiences discolouration of the feet or hands. We compared the COMPASS-31 subdomain scores between SSc patients with and without foot discolouration. Seventy-nine patients with postural orthostatic tachycardia syndrome (POTS) also completed the COMPASS-31 questionnaire for comparison.
We found that extremity discolouration is common in POTS and more often affects the feet, whereas in SSc, the hands are more frequently involved; 62% of SSc patients report colour changes in their feet. These patients are more likely to have other autonomic symptoms, including orthostatic (11.7 ± 10.6 vs 8.6 ± 9.9, P = 0.06), GI (11.3 ± 4.3 vs 8.8 ± 4.3, P = 0.0003), urinary (1.4 ± 1.5 vs 0.8 ± 1.3, P = 0.002) and secretomotor (7.0 ± 3.8 vs 5.9 ± 3.8, P = 0.06) symptoms. These associations persist in a multivariable model after adjusting for potential confounders.
Dependent extremity discolouration is common in dysautonomia. Patients with SSc who report colour changes in their feet are more likely to report other symptoms of autonomic dysfunction.
自主神经功能障碍(或自主神经失调)患者常报告其下垂肢体变色,这被认为是由于静脉淤血或手足发绀所致。系统性硬化症(SSc)患者中有一部分受自主神经失调影响,但可能难以识别。我们试图确定报告足部变色的SSc患者是否有更高的自主神经症状负担,包括直立性、胃肠道(GI)、泌尿、分泌运动和瞳孔运动方面的症状。
167例SSc患者完成了综合自主神经症状评分(COMPASS)-31调查,该调查询问患者是否经历足部或手部变色。我们比较了有和没有足部变色的SSc患者之间的COMPASS-31子领域评分。79例体位性直立性心动过速综合征(POTS)患者也完成了COMPASS-31问卷以作比较。
我们发现肢体变色在POTS中很常见,且更常影响足部,而在SSc中,手部更常受累;62%的SSc患者报告足部有颜色变化。这些患者更有可能有其他自主神经症状,包括直立性(11.7±10.6对8.6±9.9,P = 0.06)、胃肠道(11.3±4.3对8.8±4.3,P = 0.0003)、泌尿(1.4±1.5对0.8±1.3,P = 0.002)和分泌运动(7.0±3.8对5.9±3.8,P = 0.06)症状。在调整潜在混杂因素后,这些关联在多变量模型中仍然存在。
下垂肢体变色在自主神经失调中很常见。报告足部颜色变化的SSc患者更有可能报告其他自主神经功能障碍症状。