Department of Internal Medicine AP-HM, Hopital Timone, 264 Rue Saint Pierre, 13385, Marseille cedex, France.
Aix-Marseille University, Marseille, France.
Clin Rheumatol. 2019 Dec;38(12):3451-3458. doi: 10.1007/s10067-019-04710-2. Epub 2019 Aug 22.
INTRODUCTION/OBJECTIVES: The aim of our study was to investigate possible differences in nailfold videocapillaroscopy (NVC) features between patients with dermatomyositis (DM), overlap myositis (OM), antisynthetase syndrome (ASS), and immune-mediated necrotizing myopathy (IMNM).
We performed a cross-sectional monocentric study. All patients with inflammatory myopathies (IMs) over a 6-month period were analyzed by NVC for giant and ramified capillaries, tortuosities, capillary density, disorganization, and scleroderma pattern. Clinical, biological, and pathological characteristics were retrospectively recorded. Patients were classified as having DM, OM, ASS, or IMNM for comparison. Patients were also compared with a group of patients with systemic sclerosis (SSc).
NVC was analyzed in DM (n = 17), OM (n = 8), ASS (n = 12), and IMNM (n = 6). Vascular disorganization and avascular zones were observed only in DM (11.8%) and OM (62.5%). The percentage of patients with giant capillaries was higher in OM (n = 4/8) than in DM (n = 3/17) and absent in ASS and IMNM. Frequency of ramified capillaries, tortuosities, hemorrhages, or thrombosis was not different between subgroups. A scleroderma pattern was only observed in OM patients.
In this limited series of patients, we observed that DM and OM NVC abnormalities are different from ASS and IMNM. We could not determine NVC specific patterns associated with myositis-specific antibody subtypes of DM because of the small number of patients.Key Points• Nailfold videocapillaroscopy abnormalities are different in subgroups of inflammatory myopathies.• Giant capillaries, disorganization, and major capillary loss are observed in overlap myositis and dermatomyositis but not in antisynthetase syndrome (ASS) or immune-mediated necrotizing myopathy.• Nailfold videocapillaroscopy abnormalities in overlap myositis (with the exclusion of ASS) are close to systemic sclerosis.
简介/目的：我们研究的目的是探讨皮肌炎（DM）、重叠性肌炎（OM）、抗合成酶综合征（ASS）和免疫介导的坏死性肌病（IMNM）患者的甲襞毛细血管镜（NVC）特征是否存在差异。
我们进行了一项横断面单中心研究。在 6 个月的时间内，对所有炎症性肌病（IM）患者进行 NVC 检查，以分析巨毛细血管和分支毛细血管、扭曲、毛细血管密度、组织紊乱和硬皮病样模式。回顾性记录临床、生物学和病理学特征。将患者分为 DM、OM、ASS 和 IMNM 进行比较。还将患者与一组系统性硬化症（SSc）患者进行比较。
分析了 17 例 DM、8 例 OM、12 例 ASS 和 6 例 IMNM 患者的 NVC。血管组织紊乱和无血管区仅见于 DM（11.8%）和 OM（62.5%）。巨毛细血管患者百分比在 OM（4/8）中高于 DM（3/17），ASS 和 IMNM 中不存在。分支毛细血管、扭曲、出血或血栓形成的频率在亚组之间无差异。OM 患者仅观察到硬皮病样模式。
在这组有限的患者中，我们观察到 DM 和 OM 的 NVC 异常与 ASS 和 IMNM 不同。由于 DM 肌炎特异性抗体亚型患者数量较少，我们无法确定与肌炎特异性抗体亚型相关的 NVC 特定模式。
炎症性肌病亚组的甲襞毛细血管镜异常不同。
重叠性肌炎（ASS 除外）和皮肌炎可见巨毛细血管、组织紊乱和主要毛细血管丧失，但抗合成酶综合征或免疫介导的坏死性肌病无此现象。
重叠性肌炎（ASS 除外）的甲襞毛细血管镜异常与系统性硬化症相似。
