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系统性硬化症中的镶嵌式毛细血管镜检查结果

Mosaic capillaroscopic findings in systemic sclerosis.

作者信息

Lambova Sevdalina Nikolova, Müller-Ladner Ulf

机构信息

Faculty of Medicine, Department of Propaedeutics of Internal Diseases, Medical University Plovdiv, 15A "Vasil Aprilov" Blvd, 4002, Plovdiv, Bulgaria.

Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Giessen, Germany.

出版信息

Wien Med Wochenschr. 2018 Jun;168(9-10):248-249. doi: 10.1007/s10354-018-0617-3. Epub 2018 Feb 1.

Abstract

A 67-year-old woman with triphasic Raynaud's phenomenon since 2 years was diagnosed as having limited cutaneous systemic sclerosis (SSc) 1 year ago based on puffy fingers, positive antinuclear and anti-Scl-70 autoantibodies, and initial pulmonary fibrosis. Capillaroscopic examination revealed "mosaic" capillaroscopic findings that included normal pattern; "scleroderma"-type capillaroscopic pattern "early" phase, with single giant capillaries and haemorrhages, preserved capillary distribution; and "scleroderma"-type capillaroscopic pattern "active" phase, with high number of giant capillary loops and haemorrhages, moderately disturbed distribution. Nailfold capillaroscopy is an established non-invasive imaging technique for morphologic evaluation of the capillaries in the nail-fold area that reveals diagnostic changes in the majority of SSc patients. It has been included in the recently published EULAR/ACR (European League Against Rheumatism/American College of Rheumatology) classification criteria for SSc (2013). The generalized microangiopathy is a cardinal feature of SSc, but the underlying endothelial damage of the capillaries varies in the different compartments of the body, sometimes even within the same organ. As typical capillaroscopic findings are diagnostic in clinical context, this case of a "mosaic" pattern should remind the clinicians that the capillaroscopic examination should be performed for the eight fingers bilaterally (excluding the thumbs because of poor visibility), in order to detect the presence of all types and grades of pathological microvascular changes to facilitate the highest diagnostic accuracy.

摘要

一名67岁女性,出现三相雷诺现象已有2年,1年前因手指肿胀、抗核抗体和抗Scl - 70自身抗体阳性及早期肺纤维化被诊断为局限性皮肤系统性硬化症(SSc)。毛细血管镜检查发现“马赛克”样毛细血管镜表现,包括正常形态;“硬皮病”型毛细血管镜表现“早期”,有单个巨大毛细血管和出血,毛细血管分布保留;以及“硬皮病”型毛细血管镜表现“活动期”,有大量巨大毛细血管袢和出血,分布中度紊乱。甲襞毛细血管镜检查是一种成熟的非侵入性成像技术,用于评估甲襞区域毛细血管的形态,可在大多数SSc患者中发现诊断性改变。它已被纳入最近发表的欧洲抗风湿病联盟/美国风湿病学会(EULAR/ACR)SSc分类标准(2013年)。全身性微血管病是SSc的主要特征,但毛细血管潜在的内皮损伤在身体的不同部位有所不同,有时甚至在同一器官内也不同。由于典型的毛细血管镜检查结果在临床背景下具有诊断意义,这例“马赛克”样表现的病例应提醒临床医生,应双侧对八根手指进行毛细血管镜检查(因拇指观察不清而除外),以便检测出所有类型和程度的病理性微血管变化,以提高诊断准确性。

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