PATHOGENESIS OF SYSTEMIC SCLEROSIS A VASCULAR HYPOTHESIS
PATHOGENESIS OF SYSTEMIC SCLEROSIS A VASCULAR HYPOTHESIS
复制标题
DOI：
10.1016/0049-0172(75)90017-7
复制
发表时间：
1975-01-01
影响因子：
5
通讯作者：
CARWILE LE ROY E
中科院分区：
文献类型：
--
作者：
CAMPBELL P M;CARWILE LE ROY E
系统性硬化症，或硬皮病，是一种全身性多系统疾病，其中皮肤特征主导患者的外观，但内脏受累决定患者的生存。在历史上，硬皮病被认为是皮肤的一种折磨，但内脏受累的累积证据导致Goetzl提出了术语“进行性系统性硬化症”（许多患者缺乏进展导致最近的观察者使用术语“系统性硬化症”作为硬皮病的同义词）。在硬皮病的鉴别和早期研究中，重点集中在晚期纤维化;最近的兴趣是广泛的血管受累及其作为统一的发病机制概念的潜力。对哥伦比亚长老会医学中心在过去20年中观察到的261例系统性硬化症的研究和回顾导致了一种概念性方案，部分是推测性的，强调血管树是系统性硬化症的靶器官。这一假说，连接炎症，增殖和硬结阶段的血管病变，提出的背景下，系统性硬化症的一般特征，并扩展到突出的具体点，在管理器官的参与。由于最近的几篇综述提供了对该疾病的极好的、详细的报道，因此这里只包括有助于我们理解这一假设的那些方面。2* 4* 5J'
YSTEMIC SCLEROSIS, or scleroderma, is a generalized multisystem S disease in which the cutaneous features dominate the patient’s appearance, but visceral involvement determines the patient’s survival. Historically, scleroderma was thought to be an affliction of the skin, but cumulative evidence of visceral involvement led Goetzl to propose the term “progressive systemic sclerosis”(the lack of progression in many patients has led recent observers to use the term “systemic sclerosis” as essentially synonymous with scleroderma). In both the identification and early investigation of scleroderma, emphasis centered on the late-stage fibrosis; more recent interest has been in the widespread vascular involvement and its potential as a unifying pathogenetic concept.The study and review of the 261 cases of systemic sclerosis seen at Columbia-Presbyterian Medical Center over the past 20 yr has led to a conceptual scheme, speculative in part, of etiology and pathophysiology which emphasizes the vascular tree as a target organ in systemic sclerosis. This hypothesis, linking inflammatory, proliferative, and indurative phases of the vascular lesion, is presented on a background of the general features of systemic sclerosis and is extended to highlight specific points in the management of organ involvement. Since several recent reviews provide excellent, detailed coverage of the disease, only those aspects which contribute to our understanding of this hypothesis will be included here. 2* 4* 5J’