Hoitsma Elske, Faber Carin G, van Kroonenburgh Marinus J P G, Gorgels Anton P M, Halders Serve G E A, Heidendal Guido A K, Kessels Alfons G H, Reulen Jos P H, Drent Marjolein
Dept of Neurology, Management Center, University Hospital Maastricht, the Netherlands.
Sarcoidosis Vasc Diffuse Lung Dis. 2005 Mar;22(1):43-50.
Recently we found that small fiber neuropathy (SFN) occurs frequently in sarcoidosis. Autonomic dysfunction may be a feature of SFN. Since cardiac autonomic dysfunction has been identified as a strong predictor of morbidity and mortality, recognition of cardiac autonomic involvement is of clinical relevance. It was hypothesised that SFN might be related to cardiac sympathetic denervation in sarcoidosis.
In the present study 45 consecutive sarcoidosis patients (13 without SFN assessed by thermal threshold testing (TTT), 32 with SFN (abnormal TTT) were enrolled. To rule out bias due to myocardial ischemia, cases with abnormal Thallium (201Tl) perfusion scintigraphy were excluded (n = 2). Cardiovascular autonomic function testing (Ewing tests) and 123I-MIBG (metaiodobenzylguanidine) scintigraphy were used to assess cardiac autonomic function. Further cardiac diagnostic work-up included ECG, Holter recording and echo Doppler cardiography.
Mild to moderate heterogeneity of 123I-MIBG uptake regional in the myocardium was demonstrated in a substantial number of the studied sarcoidosis population, especially in those with SFN (abnormal TTT). Mean inferior-anterior ratios were 0.85+/-0.17 (SFN) and 1.0+/-0.17 (no SFN; p = 0.003), respectively. Four out of the 14 cases with abnormal MIBG scintigraphy and SFN showed an abnormal Ewing test.
Cardiac sympathetic dysfunction assessed by use of 123I-MIBG myocardial scanning appeared to be heterogeneous in sarcoidosis patients and dependent on the presence or absence of SFN. MIBG scintigraphy may be of additional value in the management and follow-up of sarcoidosis patients. Future study is warranted to explore possible prognostic and therapeutic implications of these findings in sarcoidosis.
最近我们发现结节病患者中常出现小纤维神经病变(SFN)。自主神经功能障碍可能是SFN的一个特征。由于心脏自主神经功能障碍已被确定为发病率和死亡率的强预测指标,因此认识到心脏自主神经受累具有临床意义。据推测,SFN可能与结节病中的心脏交感神经去神经支配有关。
在本研究中,连续纳入了45例结节病患者(13例经热阈值测试(TTT)评估无SFN,32例有SFN(TTT异常))。为排除心肌缺血导致的偏差,排除了铊(201Tl)灌注闪烁显像异常的病例(n = 2)。采用心血管自主神经功能测试(尤因测试)和123I -间碘苄胍(MIBG)闪烁显像来评估心脏自主神经功能。进一步的心脏诊断检查包括心电图、动态心电图记录和超声心动图检查。
在大量研究的结节病患者中,尤其是那些有SFN(TTT异常)的患者,心肌中123I - MIBG摄取区域存在轻度至中度异质性。下壁 - 前壁平均比值分别为0.85±0.17(SFN组)和1.0±0.17(无SFN组;p = 0.003)。14例MIBG闪烁显像异常且有SFN的病例中有4例尤因测试异常。
通过123I - MIBG心肌扫描评估的心脏交感神经功能障碍在结节病患者中表现为异质性,且取决于是否存在SFN。MIBG闪烁显像在结节病患者的管理和随访中可能具有额外价值。有必要进行进一步研究以探索这些发现对结节病可能的预后和治疗意义。