Kim Laura, Kedor Claudia, Buttgereit Frank, Heidecke Harald, Schaumburg Desiree, Scheibenbogen Carmen
Institute of Medical Immunology, Charité-Universitätsmedizin Berlin, 13353 Berlin, Germany.
Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, 10117 Berlin, Germany.
J Clin Med. 2023 Jul 29;12(15):4994. doi: 10.3390/jcm12154994.
Fatigue is the most commonly reported and debilitating extraglandular symptom of primary Sjögren's syndrome (pSS). Fatigue and exertional intolerance are hallmark symptoms of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). We aimed to characterize fatigue and further symptoms among pSS patients and to determine whether there is a symptom overlap in pSS and ME/CFS. In 19 patients with pSS, we assessed pSS symptom severity and disease activity via questionnaires as well as the Canadian Consensus Criteria (CCC) for ME/CFS. Hand grip strength (HGS) and levels of α1-, α2-, β1-, β2-, M3- and M4-receptor-autoantibodies were measured. A subgroup of pSS patients exhibited severe fatigue and had higher severity of pain ( = 0.045), depression ( = 0.021) and sleep disturbances ( = 0.020) compared to those with less fatigue. Four of eighteen pSS patients fulfilled the CCC. HGS parameters strongly correlated with fatigue severity ( < 0.05), but strength fully recovered one hour after exertion in contrast to ME/CFS. Levels of β1-, β2- and M4-receptor-autoantibodies were elevated and correlated significantly with disease activity assessed by the ESSDAI ( < 0.05), but not fatigue severity. Only a minor subgroup of pSS patients fulfills the CCC, and post exertional malaise (PEM) is atypical, as it is primarily triggered by mental/emotional but not physical exertion. HGS assessment is an objective measure to assess overall fatigue severity.
疲劳是原发性干燥综合征(pSS)最常报告且使人衰弱的腺体外症状。疲劳和运动不耐受是肌痛性脑脊髓炎/慢性疲劳综合征(ME/CFS)的标志性症状。我们旨在描述pSS患者的疲劳及其他症状特征,并确定pSS和ME/CFS之间是否存在症状重叠。我们对19例pSS患者进行了研究,通过问卷以及ME/CFS的加拿大共识标准(CCC)评估pSS症状严重程度和疾病活动度。测量了握力(HGS)以及α1-、α2-、β1-、β2-、M3-和M4-受体自身抗体水平。与疲劳程度较轻的患者相比,pSS患者亚组表现出严重疲劳,且疼痛(P = 0.045)、抑郁(P = 0.021)和睡眠障碍(P = 0.020)的严重程度更高。18例pSS患者中有4例符合CCC标准。HGS参数与疲劳严重程度密切相关(P < 0.05),但与ME/CFS不同的是,运动后1小时力量可完全恢复。β1-、β2-和M4-受体自身抗体水平升高,且与ESSDAI评估的疾病活动度显著相关(P < 0.05),但与疲劳严重程度无关。只有一小部分pSS患者符合CCC标准,且运动后不适(PEM)不典型,因为它主要由精神/情绪而非体力活动引发。HGS评估是评估总体疲劳严重程度的客观指标。