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混合性结缔组织病患者的甲襞毛细血管镜检查

Naifold capillaroscopy in mixed connective tissue disease patients.

作者信息

Ornowska Sylwia, Wudarski Mariusz, Dziewięcka Ewa, Olesińska Marzena

机构信息

Department of Connective Tissue Diseases, National Institute of Geriatrics, Rheumatology and Rehabilitation, ul. Spartańska 1, 02-637, Warsaw, Poland.

Department of Cardiac and Vascular Diseases, Jagiellonian University Collegium Medicum, Cardiac Institute, John Paul II Hospital, Kraków, Poland.

出版信息

Clin Rheumatol. 2024 May;43(5):1703-1709. doi: 10.1007/s10067-024-06879-7. Epub 2024 Mar 21.

Abstract

INTRODUCTION

Mixed connective tissue disease (MCTD) is a rare systemic disease characterized by overlapping features of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermato-/polymyositis (DM/PM), and rheumatoid arthritis (RA). Naifold capillaroscopy (NFC) is a non-invasive test for evaluating the capillaries of the nail shaft used in the diagnosis of rheumatic diseases.

OBJECTIVES

To determine whether there are characteristic abnormalities in NFC in MCTD patients, and whether the type of NFC lesions correlates with organ involvement in these patients.

METHODS

Clinical picture and NFC patterns were analyzed in 43 patients with MCTD. Capillaroscopic images were divided into scleroderma-like pattern (SD-like pattern) according to the Cutolo classification, non-specific lesions, and normal images. Relationships between the clinical aspects considered in the MCTD classification criteria and the changes in the capillaroscopic images were evaluated.

RESULTS

SD-like pattern was present in 20 MCTD patients (46.51%) with a predominance of the "early" pattern. Giant, branched, dilated capillaries and reduced capillary density were found more frequently in MCTD patients compared to the control group (p-values 0.0005, 0.005, 0.02, < 0.0001 respectively). There were associations found between the presence of a reduced number of vessels, avascular areas, and SD-like pattern with the presence of sclerodactyly in MCTD patients (p = 0.002, p = 0.006, p = 0.02, respectively), alongside an association between the presence of branched vessels and the subpapillary plexus with pulmonary arterial hypertension (PAH) (p = 0.04 and p = 0.005, respectively).

CONCLUSIONS

MCTD patients are significantly more likely to have abnormalities upon NFC. It is worthwhile to perform capillaroscopic examination in MCTD patients. Key Points • Scleroderma-like pattern was found in more than half of the MCTD patients. • Reduced capillary density was found to be a significant predictor of the diagnosis of MCTD. • There were relationships between the presence of reduced capillary density, avascular areas, and SD-like with the presence of sclerodactyly in the MCTD patients. • There was an association between the presence of branched vessels and the visibility of the subpapillary plexus and pulmonary arterial hypertension (PAH).

摘要

引言

混合性结缔组织病(MCTD)是一种罕见的全身性疾病,其特征为具有系统性红斑狼疮(SLE)、系统性硬化症(SSc)、皮肌炎/多肌炎(DM/PM)和类风湿关节炎(RA)的重叠特征。甲襞毛细血管镜检查(NFC)是一种用于评估甲襞毛细血管的非侵入性检查,可用于风湿性疾病的诊断。

目的

确定MCTD患者的NFC是否存在特征性异常,以及NFC病变类型是否与这些患者的器官受累相关。

方法

分析43例MCTD患者的临床表现和NFC模式。根据Cutolo分类,将毛细血管镜图像分为硬皮病样模式(SD样模式)、非特异性病变和正常图像。评估MCTD分类标准中考虑的临床方面与毛细血管镜图像变化之间的关系。

结果

20例MCTD患者(46.51%)出现SD样模式,以“早期”模式为主。与对照组相比,MCTD患者中发现巨大、分支、扩张的毛细血管和毛细血管密度降低更为频繁(p值分别为0.0005、0.005、0.02、<0.0001)。在MCTD患者中,血管数量减少、无血管区域和SD样模式的存在与指(趾)硬皮病的存在之间存在关联(p分别为0.002、0.006、0.02),同时分支血管和乳头下血管丛的存在与肺动脉高压(PAH)之间存在关联(p分别为0.04和0.005)。

结论

MCTD患者NFC出现异常的可能性显著更高。对MCTD患者进行毛细血管镜检查是值得的。要点:•超过一半的MCTD患者发现有硬皮病样模式。•发现毛细血管密度降低是MCTD诊断的重要预测指标。•MCTD患者中,毛细血管密度降低、无血管区域和SD样模式的存在与指(趾)硬皮病的存在之间存在关系。•分支血管的存在与乳头下血管丛的可见性和肺动脉高压(PAH)之间存在关联。

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