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自身免疫性皮肤状况:系统性硬化症。

Autoimmune Skin Conditions: Systemic Sclerosis.

机构信息

Department of Family and Community Medicine - Penn State College of Medicine, 700 HMC Crescent Road, Hershey, PA 17033.

Department of Dermatology - Penn State College of Medicine, 700 HMC Crescent Road, Hershey, PA 17033.

出版信息

FP Essent. 2023 Mar;526:18-24.

Abstract

Systemic sclerosis (formerly scleroderma) is a relatively rare autoimmune connective tissue disease which symmetrically affects the skin and affects the internal organs. There are two types: limited cutaneous and diffuse cutaneous. Each type is categorized by different clinical, systemic, and serologic findings. Autoantibodies can be used to predict phenotype and internal organ involvement. Systemic sclerosis can affect the lungs, gastrointestinal system, kidneys, and heart. Pulmonary and cardiac disease are the leading causes of death, so screening for these conditions is important. Early management of systemic sclerosis is paramount to prevent progression. Numerous therapeutic interventions for systemic sclerosis exist, but there is no cure. The goal of therapy is to improve quality of life by minimizing specific organ-threatening involvement and life-threatening disease.

摘要

系统性硬化症(原称硬皮病)是一种相对罕见的自身免疫性结缔组织疾病,它会对称地影响皮肤并影响内部器官。有两种类型:局限性和弥漫性。每种类型根据不同的临床、系统和血清学发现进行分类。自身抗体可用于预测表型和内部器官受累。系统性硬化症可影响肺部、胃肠道系统、肾脏和心脏。肺部和心脏疾病是导致死亡的主要原因,因此筛查这些疾病很重要。早期管理系统性硬化症对于防止疾病进展至关重要。存在许多针对系统性硬化症的治疗干预措施,但目前尚无治愈方法。治疗的目标是通过最大限度地减少特定器官威胁性受累和危及生命的疾病来提高生活质量。

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