Nailfold microvascular abnormalities are associated with a higher prevalence of pulmonary arterial hypertension in patients with MCTD
Nailfold microvascular abnormalities are associated with a higher prevalence of pulmonary arterial hypertension in patients with MCTD
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甲襞微血管异常与 MCTD 患者肺动脉高压患病率较高相关
DOI：
10.1093/rheumatology/keac165
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发表时间：
2022
期刊：
影响因子：
5.5
通讯作者：
Tanaka Yoshiya
中科院分区：
文献类型：
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作者：
Todoroki Yasuyuki;Kubo Satoshi;Nakano Kazuhisa;Miyazaki Yusuke;Ueno Masanobu;Satoh-Kanda Yurie;Kanda Ryuichiro;Miyagawa Ippei;Hanami Kentaro;Nakatsuka Keisuke;Saito Kazuyoshi;Nakayamada Shingo;Tanaka Yoshiya
目的emctd以微血管病变为临床表现，具有SLE、SSc和特发性炎性肌病（IIM）的重叠临床特征。本研究的目的是利用甲襞视频毛细血管镜（NVC）探讨MCTD患者微血管病变的临床意义。方法采用多中心、前瞻性和观察性研究方法，对50例新诊断和未经治疗的MCTD患者进行研究。在基线和干预后1年后评估临床特征和NVC结果，以及疾病对照[SLE (n= 40), SSc （n= 70）和IIM (n= 50)]。结果MCTD患者均出现雷诺现象，抗u1 RNP抗体阳性，22.0%（11/50）患者有肺动脉高压（PAH）。MCTD中NVC硬皮病的患病率为38.0%，低于SSc(88.6%)，但高于SLE（10.0%）。此外，当我们根据是否存在NVC硬皮病模式将MCTD患者分为两组时，我们发现NVC硬皮病模式患者中PAH的患病率更高。也就是说，在所有合并PAH的MCTD患者中观察到NVC硬皮病模式，而在未合并PAH的患者中观察到21.0%的NVC硬皮病模式。在强化免疫抑制治疗后，半数MCTD患者的NVC硬皮病模式消失，而SSc患者的NVC硬皮病模式没有改变。结论smctd与SLE、SSc和IIM在NVC硬皮病的患病率和对免疫抑制治疗的反应性方面存在差异。检测MCTD的甲襞微血管异常有助于预测PAH，并有助于我们进一步了解MCTD的发病机制。
ObjectiveMCTD manifests with microvasculopathy and overlapping clinical features of SLE, SSc and idiopathic inflammatory myopathies (IIM). The aim of this study was to investigate the clinical significance of microvasculopathy in patients with MCTD using nailfold videocapillaroscopy (NVC).MethodsFifty patients with newly diagnosed and untreated MCTD were enrolled in this multicentre, prospective and observational study. Clinical features and NVC findings were assessed at baseline and after 1 year post-intervention, along with disease controls [SLE (n= 40), SSc (n= 70) and IIM (n= 50)].ResultsAll MCTD patients presented Raynaud’s phenomenon and were positive for anti-U1 RNP antibodies, and 22.0% (11/50) had pulmonary arterial hypertension (PAH). The prevalence of NVC scleroderma patterns in MCTD was 38.0%, which was lower than SSc (88.6%) but higher than SLE (10.0%). In addition, when we divided MCTD patients into two groups by presence or absence of NVC scleroderma patterns, we found a higher prevalence of PAH in patients with NVC scleroderma patterns. Namely, NVC scleroderma patterns were observed in all MCTD patients with PAH, and in 21.0% of those without PAH. After intensive immunosuppressive therapy, NVC scleroderma patterns disappeared in half of the MCTD patients but were not changed in SSc patients.ConclusionsMCTD differed from SLE, SSc and IIM in terms of the prevalence and responsiveness of NVC scleroderma patterns to immunosuppressive therapy. Detection of nailfold microvascular abnormalities in MCTD could contribute to predicting PAH and help us to understand further aspects of the pathogenesis of MCTD.