背景:“硬皮病”型毛细血管镜检查模式是风湿病学中的一种参考模式,在适当的临床背景下是系统性硬化症(SSc)的诊断标志,在90%以上的硬皮病患者中观察到。类似的微血管变化,所谓的“硬皮病样”尽管在其他风湿性疾病患者中比例较低,如皮肌炎(DM),未分化结缔组织病(UCTD),系统性红斑狼疮(SLE),等。Cutolo等人提出了“硬皮病”模式的三个不同阶段。,即,\"早\",\"活动\",和“迟到”。然而,疾病持续时间只是导致微血管变化进展的因素之一,在这方面,在病程较短的患者中可以观察到“活跃”甚至“晚期”模式。此外,在其他病例中可以发现长期稳定的微血管变化。
目的:本研究的目的是评估SSc中的“硬皮病”模式和其他风湿性疾病中的“硬皮病样”模式之间的区别特征。
方法:在当前的回顾性横断面研究中分析了684张表现为“硬皮病”和“硬皮病样”模式的毛细血管镜图像。从50例SSc患者获得479张毛细血管镜照片,来自7名DM患者的105名,来自10名类风湿性关节炎(RA)患者的38名,来自5例SLE患者的36张图像,和9例UCTD患者的26张图像。当前分析中使用的所有毛细血管镜图像均符合“硬皮病/硬皮病样”模式的标准,由于毛细血管参数的病理变化也已通过毛细血管直径的定量测量得到证实,毛细管密度,和毛细管间距离。所有图像都被归类为以下一组,即,\"早\",“活动”和“后期”阶段(根据Cutolo等人的定义。),或“其他”发现,后者被具体描述,因为它们不能归因于其他三个类别之一。
结果:从50例硬皮病患者中获得了479张毛细管镜检查照片。其中31个显示“早期”,391个“活动”阶段,和57个“晚期”期“硬皮病”型微血管病。在69张被评估为“活跃”模式的图像中,发现了新血管生成。在来自DM患者的105张毛细血管镜照片中,有43张,检测到“活动”阶段;在其中2张图像中,发现了一个“迟到”模式,在60张毛细血管镜照片中,观察到新血管生成与巨大的毛细血管环相结合。本组未发现早期微血管病变。在SLE患者的毛细管镜图像中,未发现“晚期”微血管病。“早期”阶段出现在3张图像中,“活跃”阶段29,“活跃”阶段新血管生成4张图片。在11例RA患者(9例患者中有8例)的毛细血管镜检查图像中检测到早期微血管病变,4张图像中的“活动”阶段(3名患者),在23张毛细血管镜图像中,观察到新血管生成伴轻度毛细血管紊乱和毛细血管丢失以及单个巨大毛细血管(“类风湿新血管生成模式”)。在RA患者和UCTD患者中未发现经典的“晚期”型微血管病变。UCTD患者的主要毛细管镜检查模式是早期微血管病(n=23)。UCTD的其余图像表现出“活动”阶段的特征。
结论:结论:在RA中观察到早期微血管病变,SLE,和UCTD患者,但不是DM患者。在除SSc以外的所有患者组中检测到“活动”阶段“硬皮病”型毛细管镜检查模式,即,DM,SLE,RA,UCTD硬皮病和DM患者出现“晚期”“硬皮病”型微血管病,在SLE中未观察到,RA,UCTD尽管在某些情况下,硬皮病和其他风湿性疾病的微血管病可能无法区分,目前的研究结果表明,“硬皮病”和“硬皮病样”微血管病之间存在一些区别特征,这可能是一种形态学现象,与各种风湿性疾病的发病机制和微血管病理程度的差异有关。
BACKGROUND: The \"scleroderma\" type capillaroscopic pattern is a reference pattern in rheumatology that is a diagnostic sign for systemic sclerosis (SSc) in an appropriate clinical context and is observed in more than 90% of scleroderma patients. Similar microvascular changes, the so-called \"scleroderma-like\", have been described albeit in a lower proportion of patients with other rheumatic diseases, such as dermatomyositis (DM), undifferentiated connective tissue diseases (UCTD), systemic lupus erythematosus (SLE), etc. Three distinct stages of \"scleroderma\" pattern have been suggested by Cutolo et al., i.e., \"early\", \"active\", and \"late\". However, disease duration is just one of the factors that contributes to the progression of microvascular changes, and in this regard, \"active\" or even \"late\" pattern could be observed in patients with shorter disease duration. In addition, stable microvascular changes could be found for long periods in other cases.
OBJECTIVE: The aim of the study was to assess the presence of differentiating features between \"scleroderma\" pattern in SSc and \"scleroderma-like\" pattern in other rheumatic diseases.
METHODS: 684 capillaroscopic images demonstrating a \"scleroderma\" and \"scleroderma-like\" pattern have been analysed in the current retrospective cross-sectional study. 479 capillaroscopic pictures were obtained from 50 SSc patients, 105 from 7 DM patients, 38 from 10 rheumatoid arthritis (RA) patients, 36 images from 5 patients with SLE, and 26 images from 9 patients with UCTD. All capillaroscopic images used in the current analysis have fulfilled the criteria for \"sclerderma/scleroderma-like\" pattern, as the pathological changes in the capillaroscopic parameters have also been confirmed by quantitative measurement of capillary diameters, capillary density, and intercapillary distance. All the images have been categorized into one of the following groups, i.e., \"early\", \"active\" and \"late\" phases (according to the definition of Cutolo et al.), or \"other\" findings, the latter being specifically described as they could not be attributed to one of the other three categories.
RESULTS: 479 capillaroscopic pictures were obtained from 50 scleroderma patients. 31 of them showed an \"early\", 391 an \"active\" phase, and 57 a \"late\" phase \"scleroderma\" type microangiopathy. In 69 images assessed as an \"active\" pattern, neoangiogenesis was found. In 43 out of 105 capillaroscopic pictures from DM patients, an \"active\" phase was detected; in 2 of the images, a \"late\" pattern was found, and in 60 capillaroscopic pictures, neoangiogenesis in combination with giant capillary loops was observed. Early microangiopathy was not found in this group. Among capillaroscopic images from SLE patients, \"late\" phase microangiopathy was not found. \"Early\" phase was present in 3 images, \"active\" phase in 29, neoangiogenesis in \"active\" phase in 4 pictures. Early microangiopathy was detected in 11 capillaroscopic pictures from RA patients (8 out of 9 patients), an \"active\" phase in 4 images (3 patients), and in 23 capillaroscopic images, neoangiogenesis with mild capillary derangement and capillary loss and single giant capillaries (\"rheumatoid neoangiogenic pattern\") were observed. Classic \"late\" type microangiopathy was not found in RA patients as well as among patients with UCTD. The predominant capillaroscopic pattern in UCTD patients was early microangiopathy (n = 23). The rest images from UCTD exhibited features of the \"active\" phase.
CONCLUSIONS: In conclusion, early microangiopathy was observed in RA, SLE, and UCTD patients, but not in patients with DM. An \"active\" phase \"scleroderma\" type capillaroscopic pattern was detected in all patient groups other than SSc, i.e., DM, SLE, RA, and UCTD. \"Late\" phase \"scleroderma\" type microangiopathy was present in patients with scleroderma and DM and was not observed in SLE, RA, and UCTD. Despite the fact that in some cases, microangiopathy in scleroderma and other rheumatic diseases may be indistinguishable, the results of the current research have shown the presence of some differentiating features between \"scleroderma\" and \"scleroderma-like\" microangiopathy that might be a morphological phenomenon associated with differences in the pathogenesis and the degree of microvascular pathology in various rheumatic diseases.