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第一部分:原发性和继发性雷诺现象的流行病学、病理生理学和临床注意事项。

Part I: Epidemiology, pathophysiology, and clinical considerations of primary and secondary Raynaud's phenomenon.

机构信息

The Ronald O. Perelman Department of Dermatology, New York University Grossman School of Medicine, New York, New York.

Department of Dermatology, Lahey Hospital and Medical Center, Burlington, Massachusetts.

出版信息

J Am Acad Dermatol. 2024 Feb;90(2):223-234. doi: 10.1016/j.jaad.2022.06.1199. Epub 2022 Jul 7.

DOI:10.1016/j.jaad.2022.06.1199
PMID:35809798
Abstract

Raynaud's phenomenon (RP) is a relatively common disease with both primary and secondary forms. It is well understood as a vasospastic condition affecting the acral and digital arteries, resulting in characteristic, well-demarcated color changes typically in the hands and feet in response to cold or stress. Secondary RP (SRP) has been described in association with a variety of rheumatologic and nonrheumatologic diseases, environmental exposures, and/or medications. While both primary RP and SRP may impact the quality of life, SRP may lead to permanent and potentially devastating tissue destruction when undiagnosed and untreated. It is therefore crucial for dermatologists to distinguish between primary and secondary disease forms early in clinical evaluation, investigate potential underlying causes, and risk stratify SRP patients for the development of associated autoimmune connective tissue disease. The epidemiology, pathogenesis, and clinical presentation and diagnosis of both forms of RP are described in detail in this review article.

摘要

雷诺现象(RP)是一种相对常见的疾病,具有原发性和继发性两种形式。它被很好地理解为一种影响指(趾)动脉的血管痉挛性疾病,导致特征性的、界限分明的颜色变化,通常在手和脚上,以应对寒冷或压力。继发性 RP(SRP)已被描述与多种风湿性和非风湿性疾病、环境暴露和/或药物有关。虽然原发性 RP 和 SRP 都会影响生活质量,但当未被诊断和治疗时,SRP 可能导致永久性和潜在破坏性的组织破坏。因此,皮肤科医生在临床评估早期区分原发性和继发性疾病形式、调查潜在的根本原因以及对 SRP 患者进行风险分层以预测相关自身免疫性结缔组织病的发展至关重要。本文详细描述了这两种 RP 的流行病学、发病机制、临床表现和诊断。

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