University Research Institute of Maternal and Child Health and Precision Medicine and UNESCO Chair in Adolescent Health Care, Medical School, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital.
Joint Academic Rheumatology Program, National and Kapodistrian University of Athens Medical School.
Curr Opin Rheumatol. 2023 Nov 1;35(6):324-333. doi: 10.1097/BOR.0000000000000965. Epub 2023 Aug 13.
To describe the clinical significance of and the diagnostic approach to Raynaud phenomenon (RP) in the peripheral extremities and the heart.
Nailfold capillaroscopy has recently been standardized in an expert consensus paper. Abnormal capillaroscopy in combination with specific autoantibody profiles and clinical signs are highly predictive of progression of RP to systemic sclerosis (SSc). Magnetic resonance imaging (MRI) can also perform tissue characterization of both the extremities and the heart. Microvascular wall abnormalities detected using nailfold capillaroscopy in patients with SSc may lead to deposition of erythrocyte-derived iron, due to microhemorrhages, which may predispose to fibrosis. MRI can assess the presence of iron using T2∗ measurements.
RP is a hallmark of the microvasculopathy in SSc and can affect both the peripheral extremities and the heart. Nailfold capillaroscopy is the current gold standard for the evaluation of the peripheral microvasculature. Other imaging modalities include thermography, laser Doppler-derived methods, 99m Tc-pertechnetate hand perfusion scintigraphy, power Doppler ultrasonography, dynamic optical coherence tomography, MRI, and photoacoustic imaging, but these are currently not widely used. Cardiac RP can be investigated with positron emission tomography or cardiovascular magnetic resonance, with the latter offering the additional possibility of tissue characterization and iron content quantification secondary to microhemorrhages.
描述外周肢端和心脏雷诺现象（RP）的临床意义和诊断方法。
甲襞毛细血管显微镜检查最近在专家共识文件中得到了标准化。异常的毛细血管显微镜检查结合特定的自身抗体谱和临床体征，对 RP 向系统性硬化症（SSc）的进展具有高度预测性。磁共振成像（MRI）也可以对四肢和心脏进行组织特征分析。使用甲襞毛细血管显微镜检查发现 SSc 患者的微血管壁异常可能导致红细胞衍生铁的沉积，这可能导致纤维化。MRI 可以使用 T2∗测量来评估铁的存在。
RP 是 SSc 微血管病的标志，可影响外周肢端和心脏。甲襞毛细血管显微镜检查是评估外周微血管的当前金标准。其他成像方式包括热成像、激光多普勒衍生方法、99mTc-高锝酸盐手部灌注闪烁扫描、功率多普勒超声、动态光学相干断层扫描、MRI 和光声成像，但目前尚未广泛应用。心脏 RP 可以用正电子发射断层扫描或心血管磁共振进行检查，后者提供了组织特征分析和铁含量定量的额外可能性，这是由于微出血所致。
