■重症肌无力(MG),一种罕见的自身免疫性疾病,提出了诊断和管理挑战,随着欧洲发病率的增加和对患者生活质量的显著影响。尽管普遍存在自主神经症状,缺乏综合主观和客观措施的综合评估。我们旨在调查MG患者和健康对照(HCs)自主神经功能障碍的患病率和严重程度。
■我们在标准化自主神经功能测试(AFTs)和综合自主神经症状评分31(COMPASS-31)问卷中使用了逐次搏动的血液动力学反应。研究参与者包括,53例MG和30例年龄和性别匹配的HCs患者接受了标准化的心血管AFTs,并完成了COMPASS-31问卷。患者根据其心血管自主神经病变(CAN)状态分为非CAN和CAN组,使用综合自主评分量表(CASS)进行评估。在AFTs期间,心血管参数,包括心率,收缩压(BP),舒张压,平均血压,每搏输出量(SV),心输出量(CO),测量总外周阻力(TPR)。
■20例MG患者(38%)表现为轻度CAN(CASS≥2),中位CASS总评分为1.00,CASS为0.00。在27例患者中观察到了肾上腺素能损害(52%),有13例患者(24.5%)在Valsalva动作(VM)后表现出更长的压力恢复时间。71%的患者出现明显的心血管功能损害,深呼吸试验的异常结果为39.6%,VM的异常结果为56.6%。CANMG的总COMPASS-31得分比HC差(p<0.001),直立位(OI)(p<0.001),分泌运动(p=0.004),和瞳孔运动结构域(p=0.004)。COMPASS-31和OI总评分与较差的疾病预后相关(疾病持续时间,severity),血流动力学参数变化(SV,CO,TPR)在VM的第二阶段晚期,随着Δ收缩压血压的变化(Δ倾斜-仰卧),Δ舒张压BP,Δ平均BP,抬头倾斜试验期间的ΔTPR,但不是CASS得分。
■我们的研究结果表明,MG患者的肾上腺素能和心迷走神经域存在轻度心血管自主神经损伤。此外,患者报告的自主神经症状与AFTs期间的血流动力学变化以及更差的疾病结局相关,而与自主神经异常的分级无关.在AFTs期间纳入逐次搏动的血液动力学可能为表征MG组的直立不耐受症状提供进一步的见解。
UNASSIGNED: Myasthenia gravis (MG), a rare autoimmune disorder, poses diagnostic and management challenges, with increasing incidence in Europe and significant impact on patient quality of life. Despite prevalent autonomic symptoms, comprehensive assessments integrating subjective and objective measures are lacking. We aimed to investigate the prevalence and severity of autonomic dysfunction in patients with MG and healthy controls (HCs).
UNASSIGNED: We used beat-to-beat hemodynamic responses during standardized autonomic function tests (AFTs) and the Composite Autonomic Symptom Score 31 (COMPASS-31) questionnaire. Study participants including, 53 patients with MG and 30 age- and sex matched HCs underwent standardized cardiovascular AFTs and completed the COMPASS-31 questionnaire. Patients were categorized into Non-CAN and CAN groups based on their Cardiovascular Autonomic Neuropathy (CAN) status, as evaluated using the Composite Autonomic Scoring Scale (CASS). During the AFTs, cardiovascular parameters including heart rate, systolic blood pressure (BP), diastolic BP, mean BP, stroke volume (SV), cardiac output (CO), and total peripheral resistance (TPR) were measured.
UNASSIGNED: Twenty patients with MG (38%) exhibited mild CAN (CASS ≥2) with a median total CASS score of 1.00 and CASS 0.00 in HCs. Adrenergic impairment was observed in 27 patients (52%), with 13 patients (24.5%) exhibiting longer pressure recovery time after Valsalva maneuver (VM). Cardiovagal impairment was evident in 71% of patients, with abnormal results observed in 39.6% for the deep breathing test and 56.6% for the VM. CAN MG showed worse scores than HCs for the total COMPASS-31 (p < 0.001), orthostatic (OI) (p < 0.001), secretomotor (p = 0.004), and pupillomotor domains (p = 0.004). Total COMPASS-31 and OI scores were correlated with worse disease outcomes (disease duration, severity), hemodynamic parameter changes (SV, CO, TPR) during phase II late of VM, and with changes (Δtilt-supine) in Δsystolic BP, Δdiastolic BP, Δmean BP, ΔTPR during head-up-tilt test, but not with CASS score.
UNASSIGNED: Our findings demonstrate mild cardiovascular autonomic impairment in adrenergic and cardiovagal domains in patients with MG. Additionally, patient-reported autonomic symptoms correlated with hemodynamic changes during AFTs and worse disease outcomes and not with the grade of autonomic abnormalities. Incorporating beat-to-beat hemodynamics during AFTs may offer further insights for characterizing orthostatic intolerance symptoms in MG group.