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以系统性硬化症为重点的雷诺现象

Raynaud's Phenomenon with Focus on Systemic Sclerosis.

作者信息

Maciejewska Magdalena, Sikora Mariusz, Maciejewski Cezary, Alda-Malicka Rosanna, Czuwara Joanna, Rudnicka Lidia

机构信息

Department of Dermatology, Medical University of Warsaw, Koszykowa 82A, 02-008 Warsaw, Poland.

National Institute of Geriatrics, Rheumatology and Rehabilitation, Spartańska 1, 02-637 Warsaw, Poland.

出版信息

J Clin Med. 2022 Apr 28;11(9):2490. doi: 10.3390/jcm11092490.

Abstract

Raynaud's phenomenon is a painful vascular condition in which abnormal vasoconstriction of the digital arteries causes blanching of the skin. The treatment approach can vary depending on the underlying cause of disease. Raynaud's phenomenon can present as a primary symptom, in which there is no evidence of underlying disease, or secondary to a range of medical conditions or therapies. Systemic sclerosis is one of the most frequent causes of secondary Raynaud's phenomenon; its appearance may occur long before other signs and symptoms. Timely, accurate identification of secondary Raynaud's phenomenon may accelerate a final diagnosis and positively alter prognosis. Capillaroscopy is fundamental in the diagnosis and differentiation of primary and secondary Raynaud's phenomenon. It is helpful in the very early stages of systemic sclerosis, along with its role in disease monitoring. An extensive range of pharmacotherapies with various routes of administration are available for Raynaud's phenomenon but a standardized therapeutic plan is still lacking. This review provides insight into recent advances in the understanding of Raynaud's phenomenon pathophysiology, diagnostic methods, and treatment approaches.

摘要

雷诺现象是一种疼痛性血管疾病,其中手指动脉的异常血管收缩导致皮肤变白。治疗方法可能因疾病的潜在病因而异。雷诺现象可表现为原发性症状,即无潜在疾病的证据,或继发于一系列医疗状况或治疗。系统性硬化症是继发性雷诺现象最常见的病因之一;其出现可能早在其他体征和症状之前。及时、准确地识别继发性雷诺现象可能会加快最终诊断并积极改变预后。毛细血管镜检查是原发性和继发性雷诺现象诊断和鉴别的基础。它在系统性硬化症的极早期阶段很有帮助,同时也在疾病监测中发挥作用。针对雷诺现象有多种给药途径的广泛药物疗法,但仍缺乏标准化的治疗方案。本综述深入探讨了雷诺现象病理生理学、诊断方法和治疗方法的最新进展。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a692/9105786/959be443207c/jcm-11-02490-g001.jpg

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